Splenic infarction may be the first sign of granulomatosis with polyangiitis: case reports

Abstract

Granulamatous polyangiitis (GPA) is a rare systemic vasculitis that makes up most of the antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV). The disease is more likely to affect the upper and lower respiratory tract, kidney and skin. The gastrointestinal tract involvement is more rare. In the general population, spleen infarction is a rare condition. Most spleen infarcts are asymptomatic, and most patients receive a diagnosis by chance during the evaluation. The most common causes of non-traumatic spleen infarction in the general population include cardiac causes such as atrial fibrillation, infections, thrombophilic conditions, and malignancies. In rheumatic diseases, various spleen involvement has been identified, such as splenomegaly, spleen rupture, spleen abscess, asplenia and spleen infarction. The development of spleen infarct in antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE) is well known. It is not well known whether GPA is a risk factor for spleen infarction. However, publications including cases of developing spleen infarction due to GPA are increasing. This review highlights the occurrence of spleen infarction in patients with GPA, by presenting two GPA cases with splenic infarction. It also emphasizes that spleen infarction can occur in GPA and may frequently be without symptoms.