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Revista Cubana de Reumatología

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2020, Number S1

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Rev Cub de Reu 2020; 22 (S1)

Antineutrophil cytoplasmic antibody associated vasculitis: microscopic polyangiitis

Gálvez VJF, Martínez OJF, Vásquez BSP, Ortega AME
Full text How to cite this article

Language: Spanish
References: 15
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PDF size: 405.39 Kb.


Key words:

ANCA-associated vasculitis, microscopic polyangiitis, p-ANCA, nephritis.

ABSTRACT

Microscopic Polyangiitis is defined as an autoimmune, multisystemic, low prevalence disease, associated to antineutrophil cytoplasmic antibody. Characterized by necrotizing vasculitis of small vessels, with no granulomatous inflammation and absence of immune complex deposits, predominantly affecting renal, pulmonary and cutaneous tissue. 49-year old male patient with no past medical history. Refers symptomatology that started thirty days ago, characterized by dry cough, that evolves to productive cough, hemoptysis, exertional dyspnea, asthenia and pitting edema. Laboratory tests show severe anemia, glomerular filtration rate of 6.9 ml/min/1.73m2, hematuria, proteinuria, positive presence of antineutrophil cytoplasmic antibodies and anti-myeloperoxidase antibodies, additional thoracic imaging shows ground glass opacities consolidations. Immunosuppressive therapy is started with Methylprednisolone and Cyclophosphamide along with antibiotic prophylaxis, resulting in a decrease in the vasculitis activity score with subsequent remission; however due to renal tissue damage, there is no improvement in the glomerular filtration rate and the patient continues receiving renal replacement therapy. Due to its striking morbidity and mortality, we consider of great importance the knowledge of its clinical presentation, diagnostic procedures and treatment, in order to obtain a positive impact on the patient’s quality of life and survival rate.


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