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Abstract
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are a collection of diseases, characterised by destruction and inflammation of small and medium vessels. Microscopic Polyangiitis (MPA) is part of an ANCA-associated vasculitis (AAV).The clinical signs diverge and disturba number of organs such as the kidneys, lungs, stomach and intestine. Skin manifestations such as purpuric, urticarial, nodular, ulcerative, livedoid and necrotic skin lesions were common as in other vaso-occlusive disorder. Morphology and added features aid the diagnostic approach. Here, we report a diagnostically challenging case of microscopic polyangiitis with progressive glomerulonephritis.
Highlights
Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) are categorised by systemic inflammation of small to medium sized blood vessels [1]
We here by discuss a rare case of ANCA associated vasculitis microscopic polyangiitis, patient presented with palpable purpuric lesions on both legs for 10 days
It is important to differentiate from other ANCA associated vasculitis
Summary
Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) are categorised by systemic inflammation of small to medium sized blood vessels [1]. The AAV encompass granulomatosis with polyangiitis (GPA, formerly known as Wegener’s granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, earlier known as Churg-Strauss syndrome) They are united by their link with antibodies focused against proteinase 3 (PR3) and myeloperoxidase (MPO). PR3 and MPO are proteins that function as antigens inside the azurophilic granules in the cytoplasm of a neutrophil Both MPA and GPA are analogous in presentation. It turned dusky red giving rise to a blister which broke open into an ulcer on left dorsum of foot Following these skin lesions, she developed bilateral leg swelling. She had fever in due course which was intermittent and high grade, subsided with medication and without any associated systemic complaints. Endocapillary focal proliferation with mild C3 deposits observed on immunofluorescence and these features suggestive of progressive glomerulonephritis
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