1/3. Fibrosing alveolitis predating microscopic polyangiitis.A 65 year old male was diagnosed with "cryptogenic fibrosing alveolitis (CFA)" and treated successfully with prednisone. In the year following prednisone-tapering he presented with livedo reticularis, segmental pauci-immune glomerulonephritis and necrotizing vasculitis of the peripheral nerves, increased pulmonary fibrosis, and the presence of p-ANCA antibodies. Aggressive immunosuppressive treatment of this microscopic polyangiitis (MPA) was successful and also resulted in stabilization of the pulmonary fibrosis. This case illustrates that MPA may present itself monosymptomatic as CFA.- - - - - - - - - - ranking = 1keywords = microscopic polyangiitis, polyangiitis (Clic here for more details about this article) |
2/3. microscopic polyangiitis presenting as idiopathic pulmonary fibrosis: is anti-neutrophilic cytoplasmic antibody testing indicated?We report a 55-year old woman with microscopic polyangiitis who presented with idiopathic pulmonary fibrosis and 1 year later developed hematuria and proteinuria. She had a high serum level of perinuclear anti-neutrophilic cytoplasmic antibodies. Renal angiogram was normal. The diagnosis of microscopic polyangiitis was confirmed by renal biopsy, which showed pauci-immune crescentic glomerulonephritis. The patient received immunosuppressive therapy and improved markedly. Consideration of small vessel vasculitis is important in the differential diagnosis of idiopathic pulmonary fibrosis.- - - - - - - - - - ranking = 0.30000135267458keywords = microscopic polyangiitis, polyangiitis (Clic here for more details about this article) |
3/3. Pulmonary interstitial fibrosis as a presenting manifestation in perinuclear antineutrophilic cytoplasmic antibody microscopic polyangiitis.microscopic polyangiitis (MPA) is one of the vasculitides that is included in the pulmonary renal syndromes. Pathologically, MPA has been defined as necrotizing vasculitis with few or no immune deposits, primarily affecting small vessels including arterioles, venules, or capillaries. Pulmonary interstitial fibrosis (PIF) as an accompanying manifestation in MPA has not been widely appreciated. In the present study, we report six cases of MPA at our institution with radiographic evidence of PIF that was apparent before any treatment was administered. All had biopsy evidence of renal disease that was consistent with MPA as well as positive serum perinuclear antineutrophilic cytoplasmic antibody titers. hemoptysis was observed in approximately one half of the patients. As determined by CT of the chest, PIF was detected in all of the patients and was often present years before a diagnosis of MPA was made. We conclude that PIF may occur as a pulmonary manifestation of MPA. Further appreciation of this finding may lead to more data with respect to the incidence of PIF in MPA, and to a better understanding of the mechanisms that are involved in the development of this finding.- - - - - - - - - - ranking = 0.82000027053492keywords = microscopic polyangiitis, polyangiitis (Clic here for more details about this article) |