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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

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Microscopic polyangiitis in systemic sclerosis.

Hiroshi Hashimoto¹

¹Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyoku, Tokyo 113-0033, Japan.

International Journal of Rheumatology

|October 29, 2010

Summary

This summary is machine-generated.

Scleroderma patients with positive ANCA may have microscopic polyangiitis (MPA), a serious condition. Early recognition of MPA

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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

Area of Science:

Rheumatology
Immunology
Nephrology

Background:

Scleroderma (SSc) is a complex autoimmune disease.
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) can occur in SSc patients, though infrequently.
Microscopic polyangiitis (MPA) is a specific type of AAV characterized by multisystemic inflammation.

Purpose of the Study:

To describe the presentation of AAV from the perspective of MPA in Scleroderma patients.
To highlight the importance of considering MPA in SSc patients with ANCA positivity.
To outline diagnostic and therapeutic considerations for MPA in the context of SSc.

Main Methods:

Review of clinical manifestations of MPA.
Correlation of MPA symptoms with SSc patient profiles.
Discussion of diagnostic markers including ANCA, MPO-ANCA, and IIF patterns.
Consideration of inflammatory markers like ESR and CRP.

Main Results:

SSc patients with AAV may exhibit MPA's characteristic clinical features.
Key MPA manifestations include rapidly progressive glomerulonephritis (RPGN), pulmonary hemorrhage, mononeuritis, and skin involvement.
High inflammatory markers (ESR, CRP) and MPO-ANCA positivity are common in MPA.

Conclusions:

MPA should be suspected in SSc patients presenting with ANCA positivity, especially with RPGN, pulmonary hemorrhage, or systemic inflammation.
Prompt diagnosis and induction therapy (e.g., cyclophosphamide, corticosteroids, plasmapheresis) are crucial for severe MPA in SSc.
Vigilance for AAV manifestations is essential for early MPA diagnosis in SSc patients.