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Published on: March 8, 2019
Pulmonary involvement in microscopic polyangiitis
Christopher E Collins1, Francisco P Quismorio
1Department of Internal Medicine, Division of Rheumatology and Immunology, University of Southern California Keck School of Medicine, Los Angeles, California, USA.
Purpose Of Review:
Microscopic polyangiitis is a systemic necrotizing vasculitis that affects small vessels, resulting in a wide spectrum of organ involvement including the kidneys and the lungs. This paper reviews recent insights and observations into the pathogenesis, clinical manifestations, and treatment of pulmonary involvement in microscopic polyangiitis.
Recent Findings:
The spectrum of clinical presentations ranges from antecedent interstitial fibrosis to frank hemoptysis secondary to capillaritis. Computerized tomography imaging reveals a variety of pulmonary findings, including ground-glass attenuation, consolidation, thickening of bronchovascular bundles, and honeycombing. Antineutrophil cytoplasmic antibodies are important in diagnosis as well as in the pathogenesis and prognosis of microscopic polyangiitis. There is more evidence to support the various therapeutic modalities currently used in pulmonary manifestations of microscopic polyangiitis, including induction therapy with cyclophosphamide, the use of other novel pharmacologic agents such as the tumor necrosis factor-alpha blockers and rituximab, and nonpharmacologic modalities such as plasmapheresis and ventilatory management.
Summary:
The pulmonary manifestations of microscopic polyangiitis are diverse and often difficult to manage; however, as our understanding and experience grows so does our ability to successfully diagnose and treat these patients.
Insights
Microscopic polyangiitis can affect the lungs, causing diverse symptoms and imaging findings. Early diagnosis and tailored treatments, including novel agents, improve patient outcomes.
Area of Science:
- Pulmonary Medicine
- Rheumatology
- Immunology
Background:
- Microscopic polyangiitis is a systemic necrotizing vasculitis impacting small vessels.
- It frequently involves organs such as the kidneys and lungs, presenting a broad clinical spectrum.
- Pulmonary involvement presents unique diagnostic and therapeutic challenges.
Purpose of the Study:
- To review recent advancements in understanding the pathogenesis of pulmonary microscopic polyangiitis.
- To summarize current clinical manifestations and diagnostic approaches for lung involvement.
- To discuss evolving treatment strategies for pulmonary manifestations.
Main Methods:
- Literature review of recent insights and observations.
- Analysis of clinical presentations and diagnostic imaging findings.
- Evaluation of current and emerging therapeutic modalities.
Main Results:
- Pulmonary involvement ranges from interstitial fibrosis to hemoptysis due to capillaritis.
- CT imaging shows diverse findings like ground-glass opacities, consolidation, and honeycombing.
- Antineutrophil cytoplasmic antibodies (ANCA) are crucial for diagnosis, pathogenesis, and prognosis.
- Therapeutic options include cyclophosphamide, rituximab, TNF-alpha blockers, plasmapheresis, and ventilatory support.
Conclusions:
- Pulmonary manifestations of microscopic polyangiitis are varied and challenging.
- Improved understanding enhances diagnostic accuracy and treatment efficacy.
- Multidisciplinary management is key for successful patient outcomes.
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