Microscopic Polyangiitis Associated with Diffuse Panbronchiolitis

Abstract

There are several case reports of systemic vasculitis associated with chronic suppurative lung diseases. We describe a 46-year-old female, previously diagnosed as having diffuse panbronchiolitis (DPB), presenting with hemosputum and dyspnea. Her serum titer of MPO-ANCA was positive together with a high titer of BPI-ANCA. Chest X-ray and chest CT scan showed pulmonary hemorrhage, and the renal biopsy specimen revealed necrotizing, crescentic glomerulonephritis. She was diagnosed as having ANCA-associated vasculitis, and more specifically, microscopic polyangiitis accompanied by DPB. She was treated with methylprednisolone pulse therapy, followed by intravenous cyclophosphamide. This case suggested a possible association with chronic bacterial infection, which may play a role in the pathogenesis of ANCA-associated vasculitis.

A Case of Sinobronchial Syndrome Complicated with Myeloperoxidase Antineutrophil Cytoplasmic Antibody Associated Vasculitis: Review of the Literature

“…An association between chronic airway diseases (CAD) such as bronchiectasis (BE), diffuse panbronchiolitis (DPB) and sinobronchial syndrome (SBS), and vasculitis has been reported. To our knowledge, there have been 22 reported cases of CAD complicating systemic vasculitis (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14). In these reported cases, CAD preceded the onset of vasculitis.…”

Systemic Vasculitis Associated with Anti-Neutrophil Cytoplasmic Antibodies against Bactericidal/Permeability Increasing Protein

“…One of the patients was positive for anti-BPI antibodies and another was positive for anti-lactoferrin antibodies (8). In Japan, there have only been three reports written in English describing patients with BPI-ANCA to date (12)(13)(14). To the best of our knowledge, this is the first report describing BPI-ANCA-related systemic vasculitis with interstitial pneumonia.…”

Antineutrophil Cytoplasmic Antibody-associated Vasculitis Superimposed on Infection-related Glomerulonephritis Secondary to Pulmonary <i>Mycobacterium avium</i> Complex Infection

“…Particularly, a significant number of articles reported the cases of PR-3ANCA and Wegener's granulomatosis in relation to staphylococcal infection (11). Although further investigation is still required, certain infections are considered to induce autoimmune responses through antigenic mimicry and enhanced immunogenicity of host antigens as a result of activation of the innate immune system (8,(12)(13)(14).…”