Uszkodzenie wątroby w przebiegu ziarniniakowatości z zapaleniem naczyń - trudności diagnostyczne.
Data nadesłania: 24-08-2017
Data ostatniej rewizji: 19-10-2017
Data akceptacji: 24-11-2017
Data publikacji online: 30-12-2017
Data publikacji: 31-12-2017
Reumatologia 2017;55(6):318-322
We report the case of a 57-year-old Caucasian man who presented with dry cough, haemoptysis, fever, lung nodules, erythrocyturia, and acute hepatitis. After a lung biopsy, the patient was diagnosed with granulomatosis with polyangiitis. The diagnosis was supported by the presence of antiproteinase-3 anti-neutrophil cytoplasmic antibodies. The most common causes of liver damage are excluded.
The patient was treated with prednisone and cyclophosphamide, which resulted in remission of chest CT findings and improvement in liver function tests. During the flare, new lung infiltrations as well as elevation of liver enzymes were present. Treatment with rituximab resulted in complete clinical and radiological remission and normalisation of liver function tests. What makes this case worth reporting is the rare liver involvement in the GPA. After exclusion of coexistence of autoimmune liver disease and drug-induced liver lesion, organ involvement was supported by the reversion of abnormal LFT after the immunosuppression of GPA.
Jennette JC. Nomenclature and classification of vasculitis: lessons learned from granulomatosis with polyangiitis. Clin Exp Immunol 2011; 164 (Suppl. 1): 7-10.
Goritsas C, Paissios NP, Trigidou R, Delladetsima J. Hepatic involvement in Wegener’s granulomatosis: a case report. J Med Case Rep 2010; 4: 9.
Yates M, Watts RA, Bajemna IM, et al. EULAR-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis 2016; 75: 1583-1594.
Soultati A, Dourakis S. Hepatic manifestations of autoimmune rheumatic diseases. Ann Gastroenterol 2005; 18: 309-324.
De Santis M, Crotti C, Selmi C. Liver abnormalities in connective tissue diseases. Best Pract Res Clin Gastroenterol 2013; 27: 543-551.
Tovoli F, Vannini A, Fusconi M, et al. Autoimmune liver diseases and small-vessel vasculitis: four case reports and review of the literature. Ann Hepatol 2014; 13: 136-141.
Shah IA, Holstege A, Riede UN. Bioptic diagnosis of Wegener’s granulomatosis in the absence of vasculitis and granulomas. Pathol Res Pract 1984; 178: 407-415.
Den Bakker MA, Tangkau PL, Steffens TW, et al. Rupture of a hepatic artery aneurysm caused by Wegener’s granulomatosis. Pathol Res Pract 1997; 193: 61-66.
Seo P, Miny YI, Holbrook JK, et al. Damage caused by Wegener’s granulomatosis and its treatment: prospective data from the Wegener’s Granulomatosis Etanercept Trial (WGET). Arthritis Rheum 2005; 52: 2168-2178.
Pavone L, Grasselli C, Chierici E, et al. Outcome and prognostic factors during the course of primary small-vessel vasculitides. J Rheumatol 2006; 33: 1299-1306.
Willeke P, Schlüter B, Limani A, et al. Liver involvement in ANCA-associated vasculitis. Clin Rheumatol 2016; 35: 387-394.
Patel A, Patel S, Patel S, et al. Cyclophosphamide Therapy in Granulomatous Hepatitis: Cure or Culpit? Am J Therapeut 2009; 16: 367-370.
Selmi C, De Santis M, Gershwin ME. Liver involvement in subjects with rheumatic disease. Arthritis Res Ther 2011; 13: 226.
Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD). Mod Rheumatol 2012; 22: 21.
Graham R, Smyrk T, Chari S, et al. Isolated IgG4-related sclerosing cholangitis: a report of 9 cases. Human Pathol 2014; 45: 1722-1729.
Perez AR, Martínez C, Espinoza LR. IgG4-associated vasculitis. Curr Rheumatol Rep 2013; 15: 348.
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