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An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis
Published on: February 8, 2019
[MICROSCOPIC POLYANGIITIS WITH VARIABLE PRESENTATION]
Maria Karlinskaya1, Lyudmila Vaysman1, Emma Lerner1
1Internal Medicine Department B, Wolfson Medical Center, Israel.
Introduction:
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a group of rare diseases of systemic necrotizing vasculitis affecting small and medium-sized vessels and may be associated with the presence of anti- neutrophil cytoplasmic antibody. Vessels in different organs and systems are involved, leading to various clinical manifestations of the disease. We present 3 cases of microscopic polyangiitis which have been diagnosed and treated in one medical department for over 4 years. The first patient presented with a clinical picture resembling idiopathic pulmonary fibrosis (IPF) and the diagnosis of microscopic polyangiitis (MPA) was established only when acute renal failure appeared. With appropriate therapy, the renal function normalized but her respiratory status deteriorated and she died due to pulmonary infection. The second case presented with constitutional symptoms such as general weakness, weight loss, leg edema and elevated CRP. During the investigation, mononeuritis multiplex and then MPA were diagnosed. She was successfully treated. The third patient diagnosed with MPA presented as end stage renal failure and was treated by cyclophosphamide and rituximab. He did not receive cotrimoxazole that was recommended and was hospitalized for pneumocystis jirovecii pneumonia. Despite intensive therapy in the ICU by various antibiotics and mechanical ventilation, his condition deteriorated and the patient died.
Insights
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, including microscopic polyangiitis (MPA), presents diverse clinical symptoms. This case series highlights varied patient outcomes and treatment challenges in managing MPA, emphasizing the need for timely diagnosis and appropriate care.
Area of Science:
- Nephrology
- Rheumatology
- Pulmonology
- Immunology
Background:
- Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis encompasses rare systemic necrotizing diseases affecting small to medium-sized vessels.
- Microscopic polyangiitis (MPA), a form of ANCA-associated vasculitis, involves multiple organ systems, leading to varied clinical presentations.
- Early diagnosis and management are crucial for improving outcomes in ANCA-associated vasculitis.
Purpose of the Study:
- To present three distinct cases of microscopic polyangiitis (MPA) managed within a single department over four years.
- To illustrate the diverse clinical manifestations and diagnostic challenges associated with MPA.
- To highlight the impact of treatment adherence and potential complications in MPA patients.
Main Methods:
- Retrospective case series analysis of three patients diagnosed with microscopic polyangiitis (MPA).
- Review of clinical presentations, diagnostic workups, treatment regimens, and patient outcomes.
- Analysis of factors contributing to treatment success and therapeutic failure.
Main Results:
- Case 1: Presented with idiopathic pulmonary fibrosis-like symptoms, diagnosed with MPA upon renal failure onset; patient succumbed to pulmonary infection despite initial renal recovery.
- Case 2: Exhibited constitutional symptoms, diagnosed with MPA after developing mononeuritis multiplex; patient responded well to treatment.
- Case 3: Presented with end-stage renal failure, treated with cyclophosphamide and rituximab; patient died from *Pneumocystis jirovecii* pneumonia due to non-adherence to cotrimoxazole prophylaxis.
Conclusions:
- Microscopic polyangiitis (MPA) can mimic other conditions, necessitating a high index of suspicion for timely diagnosis.
- Patient outcomes in MPA are influenced by factors including the extent of organ involvement, promptness of diagnosis, and adherence to prophylactic treatments.
- Effective management of MPA requires a multidisciplinary approach and vigilant monitoring for complications such as infections.
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