Granulomatous hepatitis

Gastroenterology

Clinicals - History

Fact Explanation
Pyrexia of unknown origin (PUO) Granulomatous hepatitis is a pathological entity rather than a disease. Etiological factors are Tuberculosis. mycobacteria, histoplasmosis, fungi, brucellosis, Q- fever, syphilis, parasites, viruses, sarcoidosis & other systemic granulomatous diseases, Neoplasia- Hogdkin's disease, vasculitis,berylliosis ,drug reactions (quinine, quinidine, sulphanamide, penecilline, allopurinol, phenylbutazone, methyldopa, hydralazine, carbamezapine, isoniazid, nitrofurantoin & diazepam) & liver diseases- primary billiary cirrhosis. From these tuberculosis & sarcoidosis are the commonest(50%- 65%). 20%- 26% unknown etiology. Most patient present with fever of unknown origin, though the diagnosis of granulomatous hepatitis is rare, its important to bare in mind.Pyrexia of unknown origin is defined as fever of 38.3 Celsius or greater for at least 3 weeks with out an identifiable cause, after 3 days of hospital evaluation or 3 out patient visits. Usually Fever is relapsing in character, but continuous & remittent fever patterns have been described. Depending on the etiology they can have high spiking fever, drenching night sweats, shaking
chills.
Pyrexia of unknown origin (PUO)
Granulomatous hepatitis is a pathological entity rather than a disease. Etiological factors are Tuberculosis. mycobacteria, histoplasmosis, fungi, brucellosis, Q- fever, syphilis, parasites, viruses, sarcoidosis & other systemic granulomatous diseases, Neoplasia- Hogdkin's disease, vasculitis,berylliosis ,drug reactions (quinine, quinidine, sulphanamide, penecilline, allopurinol, phenylbutazone, methyldopa, hydralazine, carbamezapine, isoniazid, nitrofurantoin & diazepam) & liver diseases- primary billiary cirrhosis. From these tuberculosis & sarcoidosis are the commonest(50%- 65%). 20%- 26% unknown etiology. Most patient present with fever of unknown origin, though the diagnosis of granulomatous hepatitis is rare, its important to bare in mind.Pyrexia of unknown origin is defined as fever of 38.3 Celsius or greater for at least 3 weeks with out an identifiable cause, after 3 days of hospital evaluation or 3 out patient visits. Usually Fever is relapsing in character, but continuous & remittent fever patterns have been described. Depending on the etiology they can have high spiking fever, drenching night sweats, shaking
chills.
Abdominal pain These patients can have right sided abdominal pain. Mainly in right hypochondrium. Food is an aggravating factor & vomiting is a common association. Abdominal pain
These patients can have right sided abdominal pain. Mainly in right hypochondrium. Food is an aggravating factor & vomiting is a common association.
Arthralgia, myalgia Minority of the patients have these complaints. Arthralgia, myalgia
Minority of the patients have these complaints.
Asymptomatic patients They are incidental, histological or ultra sound scan findings.
The histological analysis helps to identify presence of granulomas in granulomatous hepatitis, The appearance of granulomas differ according to the etiology. -In idiopathic granulomatous hepatitis granuloma will show focal nodular aggregations of lymphocytes, mononuclear cells, epitheloid cells. Caseation is absent, granulomas are distributed in hepatic parenchyma. USS- hepatic granulomas- nodular lesions. -E.g. in hodgkin lymphoma- diffuse, hypoechoic, small or large nodular lesions in the liver. This will also show the hepatomegaly.
Asymptomatic patients
They are incidental, histological or ultra sound scan findings.
The histological analysis helps to identify presence of granulomas in granulomatous hepatitis, The appearance of granulomas differ according to the etiology. -In idiopathic granulomatous hepatitis granuloma will show focal nodular aggregations of lymphocytes, mononuclear cells, epitheloid cells. Caseation is absent, granulomas are distributed in hepatic parenchyma. USS- hepatic granulomas- nodular lesions. -E.g. in hodgkin lymphoma- diffuse, hypoechoic, small or large nodular lesions in the liver. This will also show the hepatomegaly.

Clinicals - Examination

Fact Explanation
Febrile Most patient present with fever of unknown origin, though the diagnosis of granulomatous hepatitis is rare, its important to bare in mind. Depending on the etiology they can have high spiking fever, drenching night sweats, shaking
chills. Usually Fever is relapsing in character, but continuous & remittent fever patterns have been described.
Febrile
Most patient present with fever of unknown origin, though the diagnosis of granulomatous hepatitis is rare, its important to bare in mind. Depending on the etiology they can have high spiking fever, drenching night sweats, shaking
chills. Usually Fever is relapsing in character, but continuous & remittent fever patterns have been described.
Jaundice Usually they have is the intermittent jaundice, but few has had deep jaundice with hepatic failure. Jaundice
Usually they have is the intermittent jaundice, but few has had deep jaundice with hepatic failure.
Skin lesions Commonly encountered skin lesions are spider angiomata, Erythema nodosum, palmer erythema. These are signs of chronic liver disease. Skin lesions
Commonly encountered skin lesions are spider angiomata, Erythema nodosum, palmer erythema. These are signs of chronic liver disease.
Edema This is a sign of chronic liver disease. Edema
This is a sign of chronic liver disease.
Hepatic tenderness Occurs due to the underlying inflammation. Hepatic tenderness
Occurs due to the underlying inflammation.
Hepatomegaly Patients have some degree of hepatomegaly at least as a scan finding. Hepatomegaly
Patients have some degree of hepatomegaly at least as a scan finding.
Splenomegaly Patients can have some degree of splenomegaly at least as a scan finding. Splenomegaly
Patients can have some degree of splenomegaly at least as a scan finding.
Ascitis This is a sign of chronic liver disease. Ascitis
This is a sign of chronic liver disease.

Investigations - Diagnosis

Fact Explanation
Liver biopsy The histological analysis helps to identify presence of granulomas in granulomatous hepatitis. The appearance of granulomas differ according to the etiology. e.g. -In tuberculosis- paucity of granulomas, scantiness of giant cells, mild surrounding inflammatory cell infiltrate, absence of reticulin. -Sarcoidosis- there will be numerous giant cells confined to portal areas, well marked surrounding non specific chronic inflammatory cell infiltrate. - Hepatic bartonellosis -rare cause of granulomatous hepatitis, causative organism- Bartonella henselae,In this granulomas are necrotizing granulomas,there will be accumulations of activated macrophages, surrounded by lymphocytes & fibroblasts. In idiopathic granulomatous hepatitis granuloma will show focal nodular aggregations of lymphocytes, mononuclear cells, epitheloid cells. Caseation is absent. distributed in hepatic parenchyma. Liver biopsy
The histological analysis helps to identify presence of granulomas in granulomatous hepatitis. The appearance of granulomas differ according to the etiology. e.g. -In tuberculosis- paucity of granulomas, scantiness of giant cells, mild surrounding inflammatory cell infiltrate, absence of reticulin. -Sarcoidosis- there will be numerous giant cells confined to portal areas, well marked surrounding non specific chronic inflammatory cell infiltrate. - Hepatic bartonellosis -rare cause of granulomatous hepatitis, causative organism- Bartonella henselae,In this granulomas are necrotizing granulomas,there will be accumulations of activated macrophages, surrounded by lymphocytes & fibroblasts. In idiopathic granulomatous hepatitis granuloma will show focal nodular aggregations of lymphocytes, mononuclear cells, epitheloid cells. Caseation is absent. distributed in hepatic parenchyma.
Abnormalities in liver function tests Alkaline phosphatase is elevated, Transaminases are also elevated. Serum LDH is high, bilirubin retention is also seen. Abnormalities in liver function tests
Alkaline phosphatase is elevated, Transaminases are also elevated. Serum LDH is high, bilirubin retention is also seen.
ESR elevated. This response to therapy & is a non specific but useful indicator of this patients. ESR
elevated. This response to therapy & is a non specific but useful indicator of this patients.

Investigations - Management

Fact Explanation
Full blood count- Hb Patients can have anemia, prior to treatment correction is necessary. Full blood count- Hb
Patients can have anemia, prior to treatment correction is necessary.

Management - Specific

Fact Explanation
Corticosteroid therapy In idiopathic granulomatous hepatitis prednisilone like steroids are administered. Corticosteroid therapy
In idiopathic granulomatous hepatitis prednisilone like steroids are administered.
Immuno suppressant therapy Use in idiopathic granulomatous hepatitis who is resistant to corticosteroid. Drugs used are methotrexate, cyclophosphamide. Infliximab has been used with success. Immuno suppressant therapy
Use in idiopathic granulomatous hepatitis who is resistant to corticosteroid. Drugs used are methotrexate, cyclophosphamide. Infliximab has been used with success.

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