Granulomatosis with Polyangiitis Restricted to the Back Muscle: The First Case Report

Abstract

Granulomatosis with polyangiitis (GPA) is an autoimmune disease which is a type of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis that frequently affects the lungs and kidneys. However, GPA limited to a single organ has also been reported. A 71-year-old man was admitted for back pain and fever. We detected elevated levels of inflammatory markers and myeloperoxidase-ANCA. Magnetic resonance imaging indicated diffuse inflammation of the back and psoas muscles. Histology showed degenerated muscle fibers and granulomatosis vasculitis with mixed lymphoplasma cell infiltration. High-dose methylprednisolone therapy improved his symptoms. A final diagnosis of GPA limited to the muscles was made.

Vasculitis asociadas a ANCA: análisis de 26 pacientes

“…Se ha demostrado cierta predisposición genética de HLA-DP para PR3 y HLA-DQ para MPO, lo que avala que son síndromes autoinmunes distintos y posiblemente gatillados por factores externos 27,28 . Las manifestaciones clínicas son variadas, pudiendo limitarse solo al riñón o comprometer la vía aérea superior, los pulmones, la piel entre otros órganos [29][30][31][32][33][34][35][36][37] . El compromiso renal ocurre en el 70% de los pacientes y se suele manifestar como una glomerulonefritis rápidamente progresiva, pudiendo evolucionar en el 50-66% de los casos a insuficiencia renal crónica con un grado variable de ingreso a hemodiálisis crónica.…”

“…Nuestro grupo ha publicado pacientes con formas de presentación infrecuentes 33,34 . Las formas clínicas de inicio más frecuentes fueron las caracterizadas por compromiso renal, pulmonar y otorrinolaringológico, hecho que coincide con lo comunicado en la literatura 8,[25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][41] . Si bien el compromiso renal en forma global fue cercano al 70%, similar al de otras series, en el análisis de subgrupos evidenciamos menor compromiso a lo reportado en la GPA (54.54%) y mayor compromiso en la MPA (100%).…”

Granulomatous myopathy: Sarcoidosis and beyond

“…However, inflammatory infiltration of vessel walls with or without fibrinoid necrosis remains a more frequent finding on muscle biopsy in both groups 69 . Most patients were found to have weakness and myalgia of the lower limbs, 69–71 but isolated paraspinal muscle myositis and granulomatous vasculitis in GPA have been reported 72 …”

“…69 Most patients were found to have weakness and myalgia of the lower limbs, [69][70][71] but isolated paraspinal muscle myositis and granulomatous vasculitis in GPA have been reported. 72 Crohn disease is an inflammatory bowel disease characterized by transmural inflammation of any portion of the luminal gastrointestinal tract in which granulomas can be present. Extraintestinal manifestations are generally related to inflammatory disease activity, and granulomatous involvement of bone, synovium, and vulva have been described.…”