Systemic Lupus Erythematosus in Children - Clinicals, Diagnosis, and Management

Rheumatology

Clinicals - History

Fact Explanation
Introduction Systemic Lupus Erythematosus is an autoimmune connective tissue disorder. This is rarecondition among children. Incidence is around 0.5-0.6: 100 000 and prevelence is around 4-9: 100 000 among children. Majority of patients are adolescent girls. So this condition is more common among girls (4:1). In this condition Various antigentc stimulation causes wide spectrum of antibody production following B and T cell activation. The exact triggering factors for autoantibody production is unknown. Following this abnormal immune response. Antibodies or antigen-antibody complexes deposition in various organs causes influx of neutrophils and lymphocytes inducing inflammatory reaction. Ongoing same reactions finally leads to permanent organ damage. Introduction
Systemic Lupus Erythematosus is an autoimmune connective tissue disorder. This is rarecondition among children. Incidence is around 0.5-0.6: 100 000 and prevelence is around 4-9: 100 000 among children. Majority of patients are adolescent girls. So this condition is more common among girls (4:1). In this condition Various antigentc stimulation causes wide spectrum of antibody production following B and T cell activation. The exact triggering factors for autoantibody production is unknown. Following this abnormal immune response. Antibodies or antigen-antibody complexes deposition in various organs causes influx of neutrophils and lymphocytes inducing inflammatory reaction. Ongoing same reactions finally leads to permanent organ damage.
Prolonged fever, weight loss, anorexia and other constitutional symptoms like malaise, lethargy, fatiguability These symptoms are the most common presentations. But these features are not related to the severity of disease activity or complications. Prolonged fever, weight loss, anorexia and other constitutional symptoms like malaise, lethargy, fatiguability
These symptoms are the most common presentations. But these features are not related to the severity of disease activity or complications.
Joint pain Joint involvement is the main complain and this can be seen in more then 90% of patients. children will complain of bilateral symmetrical small joint pain with mild morning stiffness. It can be a migratory arthralgia. Some times there will be swelling as well. Rarely there will be pain in major joints. These patients are at risk of developing aseptic necrosis of the hip/ knee as a rare complication of the disease or following the treatment with corticosteroids. Joint pain
Joint involvement is the main complain and this can be seen in more then 90% of patients. children will complain of bilateral symmetrical small joint pain with mild morning stiffness. It can be a migratory arthralgia. Some times there will be swelling as well. Rarely there will be pain in major joints. These patients are at risk of developing aseptic necrosis of the hip/ knee as a rare complication of the disease or following the treatment with corticosteroids.
development of severe pain and bluish discoloration over tip of fingers after exposure to cool water This called Raynaud’s phenomenon. These symptoms occur following vasculitis. These symptoms commonly associated with arthralgia or arthritis. development of severe pain and bluish discoloration over tip of fingers after exposure to cool water
This called Raynaud’s phenomenon. These symptoms occur following vasculitis. These symptoms commonly associated with arthralgia or arthritis.
Muscle pain and weakness Patients can develop myalgia but only few patients develop myositis. Muscle pain and weakness
Patients can develop myalgia but only few patients develop myositis.
Skin rash ( may be painful/ pruritic) There will be eruthematous rash on the cheeks aross the nasal bridge (butterfly rash). purpura and urticarial rash over the tip of the fingers and nail folds. These are caused by vasculitis (vasculitic rash). There will be other skin changes like Livedo reticularis and periungual erythema. Patient may also develop palmar and plantar rashes. Patients may develop a erythematous rash in face with well defined margins. These plques can ultimately lead to scarring and pigmentations (Discoid rash). In Subacute cutaneous lupus erythematosus, there is a migratory, non-scarring, papulosquamous/ annular rash. Skin rash ( may be painful/ pruritic)
There will be eruthematous rash on the cheeks aross the nasal bridge (butterfly rash). purpura and urticarial rash over the tip of the fingers and nail folds. These are caused by vasculitis (vasculitic rash). There will be other skin changes like Livedo reticularis and periungual erythema. Patient may also develop palmar and plantar rashes. Patients may develop a erythematous rash in face with well defined margins. These plques can ultimately lead to scarring and pigmentations (Discoid rash). In Subacute cutaneous lupus erythematosus, there is a migratory, non-scarring, papulosquamous/ annular rash.
Alopecia Patients with disoid lupus, commonly developed scarred alopecia causing permanent skin loss (following discoid rash over the scalp). Alopecia
Patients with disoid lupus, commonly developed scarred alopecia causing permanent skin loss (following discoid rash over the scalp).
Generalized body swelling, reduced urine output, haematuria/ frothy urine Patients with renal involvement can develop these features of renal impairment. Thought histological changes of glomerulonephritis are occur in majority of patients with SLE only few will present with clinical symptoms. Children can be present with features of pure nephritic syndrome or nephrotic syndrome or as a mixed picture. Generalized body swelling, reduced urine output, haematuria/ frothy urine
Patients with renal involvement can develop these features of renal impairment. Thought histological changes of glomerulonephritis are occur in majority of patients with SLE only few will present with clinical symptoms. Children can be present with features of pure nephritic syndrome or nephrotic syndrome or as a mixed picture.
Difficulty in breathing, pleuritic type chest pain, haemoptysis Patient will develop chest pain and difficulty in breathing following Pneumonitis, atelectasis. Patients also commonly develop bilateral exudative pleural effusions following pleuritis. With these lung manifestations there will reduction of lung volumes (shrinking lung syndrome). Rarely lung fibrosis can occur with Systemic Lupus Erythematosus. Intrapulmonary haemorrhages (due to vasculitis) can cause life threatening conditions. Difficulty in breathing, pleuritic type chest pain, haemoptysis
Patient will develop chest pain and difficulty in breathing following Pneumonitis, atelectasis. Patients also commonly develop bilateral exudative pleural effusions following pleuritis. With these lung manifestations there will reduction of lung volumes (shrinking lung syndrome). Rarely lung fibrosis can occur with Systemic Lupus Erythematosus. Intrapulmonary haemorrhages (due to vasculitis) can cause life threatening conditions.
Chest pain, palpitation, dizziness cardiac involvement also can be seen in paediatric population. Pericarditis with pericardial effusion, myocaditis causing arrhythmias, valve lesions with cardiomyopathy, arterial and venous thrombosis following vasculitis can be seen. These patients are at risk of developing strokes and ischemic heart diseases. Chest pain, palpitation, dizziness
cardiac involvement also can be seen in paediatric population. Pericarditis with pericardial effusion, myocaditis causing arrhythmias, valve lesions with cardiomyopathy, arterial and venous thrombosis following vasculitis can be seen. These patients are at risk of developing strokes and ischemic heart diseases.
Features of hypoperfusion/ ischemia following vasculitis Due to the cardiac involvement and with the possibility of arterial and venous thrombus formation with vasculitis, patients are at risk of developing organ hypoperfusion.
Eg: Brain strokes can occur giving features of paralysis, paresthesia and cranial nerve palsy.
In spinal cord, features of infarction like limb paralysis, paresthesia, bladder/ bowel dysfunction.
In bones, ischemic pain at site of the joint/back pain and fractures following long bone infarction can be seen. Myocardial infarctions can occur following hypoperfusion of the myocardium( chest pain, difficulty in breathing, dizziness).
In lungs there will be shortness of breath and pleuritic type chest pain.
In mesentry, acute abdominal pain will be the presentation.
In digits, painful fingers and toes with small bone infarction.
In kidneys infarction of medulla with papillary necrosis may lead to fail in concentrating urine causing high urine out put, dehydration and nocturnal enuresis. Chronic liver failure with micro infarction causing loss of appetite, yellowish discoloration of eyes.
Splenic infarction leads to recurrent infections like upper/ lower respiratory tract infections and diarrheal illnessess.
Features of hypoperfusion/ ischemia following vasculitis
Due to the cardiac involvement and with the possibility of arterial and venous thrombus formation with vasculitis, patients are at risk of developing organ hypoperfusion.
Eg: Brain strokes can occur giving features of paralysis, paresthesia and cranial nerve palsy.
In spinal cord, features of infarction like limb paralysis, paresthesia, bladder/ bowel dysfunction.
In bones, ischemic pain at site of the joint/back pain and fractures following long bone infarction can be seen. Myocardial infarctions can occur following hypoperfusion of the myocardium( chest pain, difficulty in breathing, dizziness).
In lungs there will be shortness of breath and pleuritic type chest pain.
In mesentry, acute abdominal pain will be the presentation.
In digits, painful fingers and toes with small bone infarction.
In kidneys infarction of medulla with papillary necrosis may lead to fail in concentrating urine causing high urine out put, dehydration and nocturnal enuresis. Chronic liver failure with micro infarction causing loss of appetite, yellowish discoloration of eyes.
Splenic infarction leads to recurrent infections like upper/ lower respiratory tract infections and diarrheal illnessess.
Seizures, alteration in mental status, headache, poor concentration 60% of patient will develop cerebral lupus. theses patients are at risk of developing epilepsy, migraines, poor concentration, cerebellar ataxia, aseptic meningitis, cranial nerve lesions, cerebrovascular disease and polyneuropathy. Also there can be visual hallucinations, chorea, organic psychosis. Seizures, alteration in mental status, headache, poor concentration
60% of patient will develop cerebral lupus. theses patients are at risk of developing epilepsy, migraines, poor concentration, cerebellar ataxia, aseptic meningitis, cranial nerve lesions, cerebrovascular disease and polyneuropathy. Also there can be visual hallucinations, chorea, organic psychosis.
Eye pain, redness of the eyes and visual impairment With vasculitis there will be retinal infarctions leading to hard exudates, and haemorrhages. Also patients can develop episcleritis, conjunctivitis, optic neuritis and Sjögren’s syndrome (dry eyes and mucous menmbranes). Though several eye complications can occur, blindness is very rare. Eye pain, redness of the eyes and visual impairment
With vasculitis there will be retinal infarctions leading to hard exudates, and haemorrhages. Also patients can develop episcleritis, conjunctivitis, optic neuritis and Sjögren’s syndrome (dry eyes and mucous menmbranes). Though several eye complications can occur, blindness is very rare.
Yellowish discoloration of the eyes, oral ulcers, sudden onset abdominal pain patient with Systemic Lupus Erythematosus can develop oral ulcers, mesenteric ischemia (causes abdominal pain), bowel perforation (causes acute abdomen with sudden onset severe abdominal pain, and features of septicaemia), liver failure( right hypochondrial pain, icterus, loss of weight, loss of appetitie) and pancreatitis ( causing diabetes mellitus and features of food indigestion). In an acute flareup patient can develop nausea, vomiting and diarrhoea. Yellowish discoloration of the eyes, oral ulcers, sudden onset abdominal pain
patient with Systemic Lupus Erythematosus can develop oral ulcers, mesenteric ischemia (causes abdominal pain), bowel perforation (causes acute abdomen with sudden onset severe abdominal pain, and features of septicaemia), liver failure( right hypochondrial pain, icterus, loss of weight, loss of appetitie) and pancreatitis ( causing diabetes mellitus and features of food indigestion). In an acute flareup patient can develop nausea, vomiting and diarrhoea.
Recurrent infections, easy fatiguabilty, increased bleeding tendency patients can develop neutropenia, lymphopenia, thrombocytopenia and haemolytic anaemia. Recurrent infections, easy fatiguabilty, increased bleeding tendency
patients can develop neutropenia, lymphopenia, thrombocytopenia and haemolytic anaemia.
Drug history Some drugs like hydralazine, isoniazid, procainamide, penicillamine can induce this condition. Drug history
Some drugs like hydralazine, isoniazid, procainamide, penicillamine can induce this condition.
History of exposure to sun light Ultraviolet light is a known triggering factor. History of exposure to sun light
Ultraviolet light is a known triggering factor.
History of recent Epstein–Barr virus infection Exposure to Epstein–Barr virus also a known predisposing factor. History of recent Epstein–Barr virus infection
Exposure to Epstein–Barr virus also a known predisposing factor.
Family history of diagnosed SLE or feature suggestive of SLE Systemic Lupus Erythematosus goes as a hereditory disease. Deficiencies of the complement genes C1q, C2 or C4 are at risk of developing Systemic Lupus Erythematosus. Family history of diagnosed SLE or feature suggestive of SLE
Systemic Lupus Erythematosus goes as a hereditory disease. Deficiencies of the complement genes C1q, C2 or C4 are at risk of developing Systemic Lupus Erythematosus.
Past medical history In a diagnosed child, complete past medical history will be useful in further management. About previous episodes, complications, tratments given, medications which patient is currently on. Past medical history
In a diagnosed child, complete past medical history will be useful in further management. About previous episodes, complications, tratments given, medications which patient is currently on.

Clinicals - Examination

Fact Explanation
General examination- febrile, ill looking patient with mild lumphadenopathy and evidence of loss of weight These symptoms are common during exacerbations due to increased metabolic activity. General examination- febrile, ill looking patient with mild lumphadenopathy and evidence of loss of weight
These symptoms are common during exacerbations due to increased metabolic activity.
Joint examination characteristically joints are normal despite of pain. Mildly swollen, tender small joints can be identified. These symptoms are usually symmetrical. Very rarely joint deformities can be seen due to bony erosions (Jaccoud's arthropathy) and contractions of the joint capsule and tendon following long term inflammatory process. Major joints also can involved. Avascular necrosis and fractures also seen among this patients. It can be either due to the disease condition it self or can be secondary to corticosteroid treatment.. Joint examination
characteristically joints are normal despite of pain. Mildly swollen, tender small joints can be identified. These symptoms are usually symmetrical. Very rarely joint deformities can be seen due to bony erosions (Jaccoud's arthropathy) and contractions of the joint capsule and tendon following long term inflammatory process. Major joints also can involved. Avascular necrosis and fractures also seen among this patients. It can be either due to the disease condition it self or can be secondary to corticosteroid treatment..
capillary nail-fold loops examination and observation of Raynaud’s phenomenon This can be done with an ophthalmoscope. It is helpful in distinguishing primary from secondary Raynaud's (loss of the normal loop pattern and capillary 'fallout' with haemorrhage and dots indicate underlying disease). Raynaud’s phenomenon can be observed while patient handling the cool water. capillary nail-fold loops examination and observation of Raynaud’s phenomenon
This can be done with an ophthalmoscope. It is helpful in distinguishing primary from secondary Raynaud's (loss of the normal loop pattern and capillary 'fallout' with haemorrhage and dots indicate underlying disease). Raynaud’s phenomenon can be observed while patient handling the cool water.
Muscle examination If the patient is having myositis there will be tenderness on examination. Muscle examination
If the patient is having myositis there will be tenderness on examination.
examination of the skin On examination there will be various types of rashes (60-85%).
eg: eruthematous rash on the cheeks aross the nasal bridge (butterfly rash). Purpura and urticarial rash over the tip of the fingers and nail folds. Skin changes like Livedo reticularis and periungual erythema. Erythematous rash in face with well defined margins. With time these plques may lead to scarring and pigmentations (Discoid rash). In Subacute cutaneous lupus erythematosus, there will be a migratory, non-scarring, papulosquamous/ annular rash
examination of the skin
On examination there will be various types of rashes (60-85%).
eg: eruthematous rash on the cheeks aross the nasal bridge (butterfly rash). Purpura and urticarial rash over the tip of the fingers and nail folds. Skin changes like Livedo reticularis and periungual erythema. Erythematous rash in face with well defined margins. With time these plques may lead to scarring and pigmentations (Discoid rash). In Subacute cutaneous lupus erythematosus, there will be a migratory, non-scarring, papulosquamous/ annular rash
scalp examination This will reveals alopacia common in temporal areas. Non scarring alopacia will be common but there can be scarring alopacia seconary to discoid rash of the scalp . scalp examination
This will reveals alopacia common in temporal areas. Non scarring alopacia will be common but there can be scarring alopacia seconary to discoid rash of the scalp .
Examination of mucus membranes Oral and nasal musus membranes are commonly involved in here. Mucus membrane changes can be ranging from mild erythema to ulcers. So oral ulcers are common. Some times nasal septum, urinal tract and vaginal involvement can be seen. deep infiltration can be leads to nasal septal destruction as well. Examination of mucus membranes
Oral and nasal musus membranes are commonly involved in here. Mucus membrane changes can be ranging from mild erythema to ulcers. So oral ulcers are common. Some times nasal septum, urinal tract and vaginal involvement can be seen. deep infiltration can be leads to nasal septal destruction as well.
Central nervous System examination On examination there will be seizures, cerebral ataxia, features of meningits (headache, photophobia, neck stiffness), cranial nerve palsy, and evidence of polyneuropathy ( muscle weakness, altered sensation, autonomic symptoms). Central nervous System examination
On examination there will be seizures, cerebral ataxia, features of meningits (headache, photophobia, neck stiffness), cranial nerve palsy, and evidence of polyneuropathy ( muscle weakness, altered sensation, autonomic symptoms).
Examination of the eye General examination of the eye will reveals orbital infections like episcleritis, conjunctivitis and dry mucous membranes (in presence of Sjögren’s syndrome). Visual acuity, colour vision and visual field will be affected in presence of optic neuritis. Fundoscopic examination will reveals retinal infarctions with hard exudates and retinal haemorrhages. Examination of the eye
General examination of the eye will reveals orbital infections like episcleritis, conjunctivitis and dry mucous membranes (in presence of Sjögren’s syndrome). Visual acuity, colour vision and visual field will be affected in presence of optic neuritis. Fundoscopic examination will reveals retinal infarctions with hard exudates and retinal haemorrhages.
abdominal examination General examination will reveals icterus. In abdominal examination, mesenteric ischemia will reveals abdominal tenderness, gueding, rigidity will present in peritonitis, hypotension, tachicardia, tachypnoea will present in septicaemia, In liver failure there will be ankle oedema, ascitis.
Hepatosplenomegaly also seen in patients at initial stage as well as during flare up.
abdominal examination
General examination will reveals icterus. In abdominal examination, mesenteric ischemia will reveals abdominal tenderness, gueding, rigidity will present in peritonitis, hypotension, tachicardia, tachypnoea will present in septicaemia, In liver failure there will be ankle oedema, ascitis.
Hepatosplenomegaly also seen in patients at initial stage as well as during flare up.
Cardiovascular examination In pericarditis with pericardial effusion will associated with pericardial rub. In myocarditis patient will hve arrhythmias( irregular pulse, tacycardia), valve lesions with cardiomyopathy will have murmurs. Cardiovascular examination
In pericarditis with pericardial effusion will associated with pericardial rub. In myocarditis patient will hve arrhythmias( irregular pulse, tacycardia), valve lesions with cardiomyopathy will have murmurs.
look for features of renal impairment and examine urine in presence of haematuria/ proteinuria. Generalized body swelling, pallor, haematuria will suggest the renal involvement(50-75%). look for features of renal impairment and examine urine in presence of haematuria/ proteinuria.
Generalized body swelling, pallor, haematuria will suggest the renal involvement(50-75%).
evidence of neutropenia, lymphopenia, thrombocytopenia and haemolytic anaemia evidence of recurrent infection, easy bruising, pallor will present. evidence of neutropenia, lymphopenia, thrombocytopenia and haemolytic anaemia
evidence of recurrent infection, easy bruising, pallor will present.
Respiratory system examination This will reveals the signs of bilateral pleural effusion( reduced lung expansion, stony dullness on percussion), signs of fibrosis (reduced air entry, presence of brochial breathing, dullness on percussion, increased vocal resonance) Respiratory system examination
This will reveals the signs of bilateral pleural effusion( reduced lung expansion, stony dullness on percussion), signs of fibrosis (reduced air entry, presence of brochial breathing, dullness on percussion, increased vocal resonance)
Mental State Examination This will reveals the presence of visual hallucinations, organic psychosis in elder children. Mental State Examination
This will reveals the presence of visual hallucinations, organic psychosis in elder children.

Investigations - Diagnosis

Fact Explanation
full blood count patients can develop neutropenia, lymphopenia, thrombocytopenia and anaemia (Anaemia of chronic disease or autoimmune haemolytic anaemia) full blood count
patients can develop neutropenia, lymphopenia, thrombocytopenia and anaemia (Anaemia of chronic disease or autoimmune haemolytic anaemia)
ESR, CRP In a controlled disease ESR will be high but CRP will be normal. But if there is any active inflammatory condition is present, CRP will be high. ESR, CRP
In a controlled disease ESR will be high but CRP will be normal. But if there is any active inflammatory condition is present, CRP will be high.
Autoantibodies like ANA, anti-dsDNA, anti-Ro, anti-Sm and anti-La There are several antibodies present in Systemic Lupus Erythematosus. ANA are positive in more than 95% of patients. Autoantibodies like ANA, anti-dsDNA, anti-Ro, anti-Sm and anti-La
There are several antibodies present in Systemic Lupus Erythematosus. ANA are positive in more than 95% of patients.
Serum complement C3 and C4 levels These are normal during the remission but levels are low during flareups. Serum complement C3 and C4 levels
These are normal during the remission but levels are low during flareups.
Renal function tests like UFR, serum creatinine, blood ureas These tests help in diagnosing the renal involvement. Renal function tests like UFR, serum creatinine, blood ureas
These tests help in diagnosing the renal involvement.
Liver function tests like AST, ALT, serum billirubin, serum albumin level and Liver function tests helpful in diagnosing the liver involvement . Liver function tests like AST, ALT, serum billirubin, serum albumin level and
Liver function tests helpful in diagnosing the liver involvement .
ECG, Echocardiogram These will be helpful in diagnosing the cardiovascular complications like pericardial effusions, arrhythmias, and valvular abnormalities. ECG, Echocardiogram
These will be helpful in diagnosing the cardiovascular complications like pericardial effusions, arrhythmias, and valvular abnormalities.
Ultrasound scan of the abdomen Ultrasound scan of the abdomen will help to assess the presence of ascitis in chronic liver failure. This also useful In assessing the hepatosplenomegaly present in SLE. In renal involmetn Ultrasound scan can use to assess the urinary system. Ultrasound scan of the abdomen
Ultrasound scan of the abdomen will help to assess the presence of ascitis in chronic liver failure. This also useful In assessing the hepatosplenomegaly present in SLE. In renal involmetn Ultrasound scan can use to assess the urinary system.
Chest X ray As Systemic Lupus Erythematosus can cause pulmonary involvement, Chest X ray is useful. Chest X ray
As Systemic Lupus Erythematosus can cause pulmonary involvement, Chest X ray is useful.
CT/ MRI scan of brain uncomplicated cases ct/ MRI findings will be norma. These tests will be useful in assessing cerebral complications. Brain arophy, brain infarction following vasculitis can be identified.High-resolution computer tomography (CT) scanning will be useful in investigating the pulmonary complications like fibrosis . CT/ MRI scan of brain
uncomplicated cases ct/ MRI findings will be norma. These tests will be useful in assessing cerebral complications. Brain arophy, brain infarction following vasculitis can be identified.High-resolution computer tomography (CT) scanning will be useful in investigating the pulmonary complications like fibrosis .
pleural aspirates for cytology, culture and ABST In the presence of pleural effusion these tests useful in identifyint the exudate anfd excluding pulmonary infections. pleural aspirates for cytology, culture and ABST
In the presence of pleural effusion these tests useful in identifyint the exudate anfd excluding pulmonary infections.
CSF studies like CSF full report, culture and ABST These will be helpful in excluding the septic meningitis in patients with clinical symptoms and signd suggestive of meningitis. CSF studies like CSF full report, culture and ABST
These will be helpful in excluding the septic meningitis in patients with clinical symptoms and signd suggestive of meningitis.
Histological and immunofluorescent study of biopsies from the kidney and the skin In these studies deposition of IgG and complement will be identified. This will be useful in classifying the lupus nephritis as well. Histological and immunofluorescent study of biopsies from the kidney and the skin
In these studies deposition of IgG and complement will be identified. This will be useful in classifying the lupus nephritis as well.

Investigations - Management

Fact Explanation
FBC, blood picture and reticulocyte count patients can develop neutropenia, lymphopenia, thrombocytopenia and anaemia (Anaemia of chronic disease or autoimmune haemolytic anaemia). blood picture will show normocytic normochromic anaemia with low counts of WBC and platelets. Reticulocyte count will be high. FBC, blood picture and reticulocyte count
patients can develop neutropenia, lymphopenia, thrombocytopenia and anaemia (Anaemia of chronic disease or autoimmune haemolytic anaemia). blood picture will show normocytic normochromic anaemia with low counts of WBC and platelets. Reticulocyte count will be high.
ESR, CRP In a controlled disease ESR will be high but CRP will be normal. But if there is any active inflammatory condition is present, CRP will be high. ESR, CRP
In a controlled disease ESR will be high but CRP will be normal. But if there is any active inflammatory condition is present, CRP will be high.
Serum complement C3 and C4 levels These are normal during the remission but levels are low during flareups. Serum complement C3 and C4 levels
These are normal during the remission but levels are low during flareups.
Histological and immunofluorescent study of biopsies from the kidney and the skin In these studies deposition of IgG and complement will be identified. This will be useful in classifying the lupus nephritis as well. Histological and immunofluorescent study of biopsies from the kidney and the skin
In these studies deposition of IgG and complement will be identified. This will be useful in classifying the lupus nephritis as well.
Muscle biopsy There will be necrosis and inflammation on muscle biopsy in the presence of myositis. Muscle biopsy
There will be necrosis and inflammation on muscle biopsy in the presence of myositis.
Antiphospholipid antibodies This will give an idea about the prognosis of the patient as 25-40% of patients with positive Antiphospholipid antibodies will develop Antiphospholipid syndrome in future. Antiphospholipid antibodies
This will give an idea about the prognosis of the patient as 25-40% of patients with positive Antiphospholipid antibodies will develop Antiphospholipid syndrome in future.
Fasting lipid profile, fasting glucose level Patient who are getting corticosteroid treatments should under screen wit there tests to assess the drug induced complications like diabetes mellitus and dyslipidaemia. Fasting lipid profile, fasting glucose level
Patient who are getting corticosteroid treatments should under screen wit there tests to assess the drug induced complications like diabetes mellitus and dyslipidaemia.
FBC This will be useful in looking for platelet count, haemoglobin level and WBC count when preparing the patient for invasive procedures. FBC
This will be useful in looking for platelet count, haemoglobin level and WBC count when preparing the patient for invasive procedures.
Clotting profile test with PT/INR, APTT These test also useful to assess the clotting status of the patient. Clotting profile test with PT/INR, APTT
These test also useful to assess the clotting status of the patient.
Renal function tests like UFR, serum creatinine, blood urea, Assessment of the renal function of the patient is useful during imaging and invasive procedures. Renal function tests like UFR, serum creatinine, blood urea,
Assessment of the renal function of the patient is useful during imaging and invasive procedures.
Liver function tests like AST, ALT, serum billirubin, serum albumin level and Ultrasound scan of the abdomen Liver function tests helpful in management to diagnosing the liver involvement . Liver function tests like AST, ALT, serum billirubin, serum albumin level and Ultrasound scan of the abdomen
Liver function tests helpful in management to diagnosing the liver involvement .
ECG, Echocardiogram These will be helpful in diagnosing the cardiovascular complications during the management. ECG, Echocardiogram
These will be helpful in diagnosing the cardiovascular complications during the management.
Chest X ray Chest X ray is also useful in assessing the fitness of the patient. Chest X ray
Chest X ray is also useful in assessing the fitness of the patient.
American Rheumatism Association Criteria for Systemic Lupus Erythematosus diagnosis (four or more of these features need to be present serially or simultaneously, on two separate occasions) Malar rash- Fixed erythema, flat or raised, sparing the nasolabial folds
Discoid rash- Erythematous raised patches with adherent keratotic scarring and follicular plugging
Photosensitivity- Rash as a result of unusual reaction to sunlight
Oral ulcers- Oral or nasopharyngeal ulceration, which may be painless Arthritis- Non-erosive, involving two or more peripheral joints
Serositis- Pleuritis (convincing history of pleuritic pain or rub, or pleural effusion) or Pericarditis (rub, ECG evidence oreffusion) Renal disorder- Persistent proteinuria > 0.5 g/day or Cellular casts (red cell, granular or tubular)
Neurological disorder- Seizures or psychosis, in the absence of offending drugs or metabolic derangement Haematological disorder- Haemolytic anaemia or Leucopenia2 (< 4 ××109/l), or Lymphopenia2 (< 1 ××109/l), or Thrombocytopenia2 (< 100 ××109/l) in the absence of offending drugs
Immunological disorder- Anti-DNA antibodies in abnormal titre or Presence of antibody to Sm antigen or Positive antiphospholipid antibodies
Antinuclear antibody (ANA) disorder- Abnormal titre of ANA by immunofluorescence
American Rheumatism Association Criteria for Systemic Lupus Erythematosus diagnosis (four or more of these features need to be present serially or simultaneously, on two separate occasions)
Malar rash- Fixed erythema, flat or raised, sparing the nasolabial folds
Discoid rash- Erythematous raised patches with adherent keratotic scarring and follicular plugging
Photosensitivity- Rash as a result of unusual reaction to sunlight
Oral ulcers- Oral or nasopharyngeal ulceration, which may be painless Arthritis- Non-erosive, involving two or more peripheral joints
Serositis- Pleuritis (convincing history of pleuritic pain or rub, or pleural effusion) or Pericarditis (rub, ECG evidence oreffusion) Renal disorder- Persistent proteinuria > 0.5 g/day or Cellular casts (red cell, granular or tubular)
Neurological disorder- Seizures or psychosis, in the absence of offending drugs or metabolic derangement Haematological disorder- Haemolytic anaemia or Leucopenia2 (< 4 ××109/l), or Lymphopenia2 (< 1 ××109/l), or Thrombocytopenia2 (< 100 ××109/l) in the absence of offending drugs
Immunological disorder- Anti-DNA antibodies in abnormal titre or Presence of antibody to Sm antigen or Positive antiphospholipid antibodies
Antinuclear antibody (ANA) disorder- Abnormal titre of ANA by immunofluorescence
Classification of Lupus Nephritis by international Society Of Nephrology and Renal pathology Society Class I – Minimal mesangial lupus nephritis: with immune deposits but normal on light microscopy. Asymptomatic. Class II – Mesangial proliferative lupus nephritis: with mesangial hypercellularity and matrix expansion. Clinically, mild renal disease.
Class III – Focal lupus nephritis: (involving < 50% of glomeruli) with subdivisions for active or chronic lesions. Subepithelial deposits seen. Clinically have haematuria and proteinuria. 10–20% of all lupus nephritis.
Class IV – Diffuse lupus nephritis: (involving ≥ 50% of glomeruli) classified by the presence of segmental and global lesions as well as active and chronic lesions. Subendothelial deposits are present. Clinically there is progression to the nephrotic syndrome, hypertension and renal insufficiency. Most common and most severe form of lupus nephritis.
Class V – Membranous lupus nephritis: affects 10–20% of patients. Can occur in combination with III or IV. Good prognosis. Class VI – Advanced sclerosing lupus nephritis: (≥ 90% globally sclerosed glomeruli without residual activity). This represents the advanced stages of the above, as well as healing. Immunosuppressive therapy is unlikely to help as it is ‘inactive’. Progressive renal failure.
Classification of Lupus Nephritis by international Society Of Nephrology and Renal pathology Society
Class I – Minimal mesangial lupus nephritis: with immune deposits but normal on light microscopy. Asymptomatic. Class II – Mesangial proliferative lupus nephritis: with mesangial hypercellularity and matrix expansion. Clinically, mild renal disease.
Class III – Focal lupus nephritis: (involving < 50% of glomeruli) with subdivisions for active or chronic lesions. Subepithelial deposits seen. Clinically have haematuria and proteinuria. 10–20% of all lupus nephritis.
Class IV – Diffuse lupus nephritis: (involving ≥ 50% of glomeruli) classified by the presence of segmental and global lesions as well as active and chronic lesions. Subendothelial deposits are present. Clinically there is progression to the nephrotic syndrome, hypertension and renal insufficiency. Most common and most severe form of lupus nephritis.
Class V – Membranous lupus nephritis: affects 10–20% of patients. Can occur in combination with III or IV. Good prognosis. Class VI – Advanced sclerosing lupus nephritis: (≥ 90% globally sclerosed glomeruli without residual activity). This represents the advanced stages of the above, as well as healing. Immunosuppressive therapy is unlikely to help as it is ‘inactive’. Progressive renal failure.
Urine for protein and red blood cells As these patients are at risk of developing renal failure all patients are regularly screened and look for presence of protein and red blood cells in the urine. From these tests lupus nephritis can be diagnosed in early stages before going into renal impairment. Urine for protein and red blood cells
As these patients are at risk of developing renal failure all patients are regularly screened and look for presence of protein and red blood cells in the urine. From these tests lupus nephritis can be diagnosed in early stages before going into renal impairment.

Management - Supportive

Fact Explanation
Health education Patient and parents/ caregiver should be educated regarding the disease, symptoms associated with, possible complications, investigations needed, available treatment options, predisposing factors for exacerbations and prognosis. As this is a life long genetically transmitting disease that fact also need to be address. Health education
Patient and parents/ caregiver should be educated regarding the disease, symptoms associated with, possible complications, investigations needed, available treatment options, predisposing factors for exacerbations and prognosis. As this is a life long genetically transmitting disease that fact also need to be address.
Life style modification Due to the symptoms associated with the disease patient's normal day to day life can be affected. eg: malaise, lethargy, fatiguability will limit the patient to bed. Joint pain and deformities will interfere with fine works. This should be discussed with the patient and physiotherapy, occupational therapy and help from social services can be afforded to improve the quality of life. Life style modification
Due to the symptoms associated with the disease patient's normal day to day life can be affected. eg: malaise, lethargy, fatiguability will limit the patient to bed. Joint pain and deformities will interfere with fine works. This should be discussed with the patient and physiotherapy, occupational therapy and help from social services can be afforded to improve the quality of life.
Avoid precipitating factors Patient and parents/ caregivers should be provide informations regarding precipitating factors like excessive exposure to sun light, drugs causing exacerbations and hormone replacement therapy. These factors should be avoided (eg: use high factor sun blocks to minimize UV light exposure, hats and protective cloths will be useful) as much as possible and patient should be educated to early identification of exacerbations. Avoid precipitating factors
Patient and parents/ caregivers should be provide informations regarding precipitating factors like excessive exposure to sun light, drugs causing exacerbations and hormone replacement therapy. These factors should be avoided (eg: use high factor sun blocks to minimize UV light exposure, hats and protective cloths will be useful) as much as possible and patient should be educated to early identification of exacerbations.
Psychological help to cope with difficulties occurring with disease Especially during late childhood and adolescent period the disease will be a big problem to the patient due to complications and episodic exacerbations. The disease will affect their learning, may limit them from doing sports and limit them from socialization. So psychological help will be useful to maintain a successful normal life. Psychological help to cope with difficulties occurring with disease
Especially during late childhood and adolescent period the disease will be a big problem to the patient due to complications and episodic exacerbations. The disease will affect their learning, may limit them from doing sports and limit them from socialization. So psychological help will be useful to maintain a successful normal life.
Nutritional supplementation Children should be given a nutritious food with all micro and macronutrients. If the patient is anaemic patient should supply more food especially containing iron, folic acid, vitamin B12. As patients with SLE and on corticosteroid treatments are at high risk of developing osteopenia, so calcium and vitamin D supplements should be a major component in nutritional supplementation. Also patients on steroids need to be given foods containing less amounts of lipids and salt( added salt should be avoided). Nutritional supplementation
Children should be given a nutritious food with all micro and macronutrients. If the patient is anaemic patient should supply more food especially containing iron, folic acid, vitamin B12. As patients with SLE and on corticosteroid treatments are at high risk of developing osteopenia, so calcium and vitamin D supplements should be a major component in nutritional supplementation. Also patients on steroids need to be given foods containing less amounts of lipids and salt( added salt should be avoided).

Management - Specific

Fact Explanation
Multidisciplinary team management This is very important in managing patients with Systemic Lupus Erythematosus as it affect multi systems of the patients. This multidisciplinary team will need various specialist of the doctors according to the condition of the patient. eg: Physician- In managing the general condition of the patient Cardiologist/ cardiothoracic surgeon- in managing the cardiac complications like pericardial effusion, arrhythmias, valve problems Neurologist- In managing the cerebral lupus Rheumatologist- to manage arthritis and joint deformities Dermatologist- in managing the skin conditions Immunologist- In assessing the immunological status of the patient Nephrologist- In the presence of lupus nephritis Pulmonarologist- In presence of pulmonary complication Haematologist- In managing haematological complications (neurtropenia, leucopenia, thrombocytopenia and anaemia) , Pathologist- In assessing the histology ogf the patient Gynaecologist- in recurrent miscarriages Radiologist- In investigating the patient Gastroenterologist/ surgeon- in presence of gastrointestinal complication like mesenteric ischemia, bowel perforation Psychiatrist- in psychological counseling Other than doctors nursing care by nurses, physiotherapists, occupational therapists, social workers, relatives need to be included. Genetic specialist in genetic counseling. Multidisciplinary team management
This is very important in managing patients with Systemic Lupus Erythematosus as it affect multi systems of the patients. This multidisciplinary team will need various specialist of the doctors according to the condition of the patient. eg: Physician- In managing the general condition of the patient Cardiologist/ cardiothoracic surgeon- in managing the cardiac complications like pericardial effusion, arrhythmias, valve problems Neurologist- In managing the cerebral lupus Rheumatologist- to manage arthritis and joint deformities Dermatologist- in managing the skin conditions Immunologist- In assessing the immunological status of the patient Nephrologist- In the presence of lupus nephritis Pulmonarologist- In presence of pulmonary complication Haematologist- In managing haematological complications (neurtropenia, leucopenia, thrombocytopenia and anaemia) , Pathologist- In assessing the histology ogf the patient Gynaecologist- in recurrent miscarriages Radiologist- In investigating the patient Gastroenterologist/ surgeon- in presence of gastrointestinal complication like mesenteric ischemia, bowel perforation Psychiatrist- in psychological counseling Other than doctors nursing care by nurses, physiotherapists, occupational therapists, social workers, relatives need to be included. Genetic specialist in genetic counseling.
Non Steroid Anti Inflammatory Drugs In the presence of arthralgia, arthritis, serositis, fever and other constitutional symptoms standard doses of Non Steroid Anti Inflammatory Drugs will be useful. Non Steroid Anti Inflammatory Drugs
In the presence of arthralgia, arthritis, serositis, fever and other constitutional symptoms standard doses of Non Steroid Anti Inflammatory Drugs will be useful.
Topical corticosteroids Topical corticosteroids are helpful in managing the skin conditions in cutaneous lupus. Topical corticosteroids
Topical corticosteroids are helpful in managing the skin conditions in cutaneous lupus.
Antimalarial drugs Antimalarial drugs like chloroquine, hydroxychloroquine can be used in mild skin disease, fatigue and arthralgias where those symptoms cannot be controlled with NSAIDs. Antimalarial drugs
Antimalarial drugs like chloroquine, hydroxychloroquine can be used in mild skin disease, fatigue and arthralgias where those symptoms cannot be controlled with NSAIDs.
Corticosteroids Various types of corticosteroids can be use to treat this condition. eg: Short course of oral corticosteroids in mild to moderate disease condition like presence of rash, serositis. Single intramuscular injections of long-acting corticosteroids or short courses of oral corticosteroids are useful in treating severe flares of arthritis, pleuritis or pericarditis. High dose of oral corticosteroids in lupus nephritis, cerebral lupus, severe haemolytic anaemia or thrombocytopenia. Corticosteroids
Various types of corticosteroids can be use to treat this condition. eg: Short course of oral corticosteroids in mild to moderate disease condition like presence of rash, serositis. Single intramuscular injections of long-acting corticosteroids or short courses of oral corticosteroids are useful in treating severe flares of arthritis, pleuritis or pericarditis. High dose of oral corticosteroids in lupus nephritis, cerebral lupus, severe haemolytic anaemia or thrombocytopenia.
immunosuppressive drugs In addition to high dose of oral corticosteroids in lupus nephritis and cerebral lupus patients should be treated with immunosuppressive drugs like Cyclophosphamide, mycophenolate mofetil. Azathioprine like immunosuppressive drugs are used in maintaining remissions. immunosuppressive drugs
In addition to high dose of oral corticosteroids in lupus nephritis and cerebral lupus patients should be treated with immunosuppressive drugs like Cyclophosphamide, mycophenolate mofetil. Azathioprine like immunosuppressive drugs are used in maintaining remissions.
Newer drugs like rituximab These are used in refractory cases of Systemic Lupus Erythematosus. It reduces the auto antibody levels by suppressing levels of CD20 positive B lymphocytes.
8) warfarin Patients with past history of previous thrombotic complications should be given life-long warfarin.
Newer drugs like rituximab
These are used in refractory cases of Systemic Lupus Erythematosus. It reduces the auto antibody levels by suppressing levels of CD20 positive B lymphocytes.
8) warfarin Patients with past history of previous thrombotic complications should be given life-long warfarin.
Prevention and Treatment of recurrent infections SLE patients are at risk of developing recurrent infections.
eg:
Bacterial- Pneumococcus, meningococcus, hemophilus influenza type B and salmonella
viruses- influenza, varicella zoster

So vaccination against those bacteria and epidemic viruses will helpful in preventing disease. Early identification and prompt treatment (with antibiotics/ antiviral drugs) will also important.
Prevention and Treatment of recurrent infections
SLE patients are at risk of developing recurrent infections.
eg:
Bacterial- Pneumococcus, meningococcus, hemophilus influenza type B and salmonella
viruses- influenza, varicella zoster

So vaccination against those bacteria and epidemic viruses will helpful in preventing disease. Early identification and prompt treatment (with antibiotics/ antiviral drugs) will also important.

Concise, fact-based medical articles to refresh your knowledge

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