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Idiopathic granulomatous hepatitis is a rare medical condition characterized by granulomas in the liver, recurrent fever, myalgia, and fatigue. The condition is not a true hepatitis, and some experts believe it is a variant of sarcoidosis. [1] [2]
Sarcoidosis is a disease of unknown cause characterized by non-necrotizing ("non-caseating") granulomas in multiple organs and body sites, [12] most commonly the lungs and lymph nodes within the chest cavity. Other common sites of involvement include the liver, spleen, skin, and eyes.
These granulomas are the main reason for the name granulomatosis with polyangiitis, although it is not an essential feature. Nevertheless, necrotizing granulomas are a hallmark of this disease. However, many biopsies can be nonspecific and 50% provide too little information for the diagnosis of GPA. [12]
Around 70% of people have granulomas in their livers, although only in about 20–30% of cases, liver function test anomalies reflecting this fact are seen. [20] [28] About 5–15% of patients exhibit hepatomegaly. [22] Only 5–30% of cases of liver involvement are symptomatic. [68]
Other diagnoses to consider include conditions that may cause acute hepatitis or chronic liver inflammation that may be accompanied by cirrhosis: Other autoimmune diseases that involve the liver: Primary biliary cholangitis - the presence of isolated elevated AMA antibodies usually signifies primary biliary cholangitis rather than autoimmune ...
Other causes include myeloproliferative disorders (especially late-stage or spent polycythemia vera), granulomatous diseases, and (lipid) storage diseases. Myelofibrosis can occur in all of these. Factors that may contribute to decreased RBC production include a decreased quantity of functioning hematopoietic tissue, disordered metabolism ...
Lymphomatoid means lymphoma-like and granulomatosis denotes the microscopic characteristic of the presence of granulomas with polymorphic lymphoid infiltrates and focal necrosis within it. LG most commonly affects middle aged people, [2] but has occasionally been observed in young people. [3] Males are found to be affected twice as often as ...
Nodular regenerative hyperplasia (NRH) is a rare liver disease, characterised by the growth of nodules within the liver, resulting in liver hyperplasia.While in many cases it is asymptomatic and thus goes undetected – or is only discovered incidentally while investigating some other medical condition – in some people it results in non-cirrhotic portal hypertension (NCPH).