We describe a rare case of concurrent eosinophilic granulomatosis with polyangiitis and mixed connective tissue disease in a 27-year-old man who presented with pulmonary, renal, cardiac, and skin manifestations. We confirmed the diagnosis based on clinical, histopathological, and serological criteria. We treated the patient with corticosteroids, methotrexate, cyclophosphamide, and hydroxychloroquine, achieving early remission. The coexistence of both conditions in the same patient is extremely rare and has only been reported in a few cases worldwide. We also review the literature on these two rare autoimmune diseases' coexistence, pathogenesis, diagnosis, and management. Our case emphasizes recognizing overlapping autoimmune conditions in patients with complex clinical features and employing a comprehensive diagnostic approach and tailored treatment strategies. Further research is needed to understand these patients' epidemiology, prognosis, and optimal therapy. Early diagnosis and aggressive immunosuppression are crucial for achieving remission and preventing organ damage. We also identified the knowledge gaps and research needs in this field.
Keywords: antineutrophil cytoplasmic antibody (anca) associated vasculitis (aav); churg-strauss syndrome; dermatopathology; eosinophilic granulomatosis with polyangiitis (egpa); granulomatosis with polyangiitis (gpa); panca/mpo (myeloperoxidase)-positive microscopic polyangiitis (mpa); polymyositis; sle pathogenesis; systemic lupus erythematosus; vasculitis.
Copyright © 2023, Shafique Ur Rehman et al.