Hepatic involvement in granulomatosis with polyangiitis - diagnostic difficulties

Area of Science:

• Nephrology
• Rheumatology
• Hepatology

Background:

• Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis.
• Liver involvement in GPA is uncommon, posing diagnostic challenges.
• This case presents a unique manifestation of GPA with significant hepatic involvement.

Purpose of the Study:

• To report a rare case of granulomatosis with polyangiitis (GPA) presenting with significant liver involvement.
• To discuss the diagnostic considerations and management of GPA with hepatic manifestations.
• To emphasize the importance of recognizing GPA as a potential cause of acute hepatitis.

Main Methods:

• A 57-year-old male presented with symptoms including dry cough, hemoptysis, fever, lung nodules, erythrocyturia, and acute hepatitis.
• Diagnosis of GPA was confirmed via lung biopsy and positive anti-proteinase-3 anti-neutrophil cytoplasmic antibodies (ANCA).
• Exclusion of other common causes of liver damage was performed.

Main Results:

• Initial treatment with prednisone and cyclophosphamide led to partial remission of pulmonary findings and improved liver function.
• A subsequent flare showed new lung infiltrations and elevated liver enzymes.
• Treatment with rituximab achieved complete clinical and radiological remission, with normalization of liver function tests.

Conclusions:

• Rare hepatic involvement can occur in granulomatosis with polyangiitis.
• Prompt diagnosis and immunosuppressive therapy, including rituximab, are crucial for managing GPA with liver manifestations.
• This case underscores the importance of considering GPA in the differential diagnosis of unexplained hepatitis, especially in the presence of other systemic symptoms.