Related Experiment Video

Updated: Aug 19, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
04:44

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

Fibrosing alveolitis predating microscopic polyangiitis

A Becker-Merok1, J C Nossent, N Ritland

  • 1Department of Rheumatology, University Hospital Tromsø, Norway.

Scandinavian Journal of Rheumatology
|September 30, 1999
PubMed

Insights

Microscopic polyangiitis (MPA) can initially present as cryptogenic fibrosing alveolitis (CFA). Prompt immunosuppressive treatment is crucial for managing MPA, even when it mimics lung fibrosis.

Area of Science:

  • Pulmonary Medicine
  • Nephrology
  • Rheumatology

Background:

  • Cryptogenic fibrosing alveolitis (CFA) is a progressive lung disease.
  • Microscopic polyangiitis (MPA) is a systemic vasculitis often associated with anti-neutrophil cytoplasmic antibodies (ANCA).

Observation:

  • A 65-year-old male initially diagnosed with CFA responded well to prednisone.
  • Following prednisone tapering, the patient developed livedo reticularis, glomerulonephritis, and peripheral nerve vasculitis.
  • Pulmonary fibrosis worsened, and p-ANCA antibodies were detected.

Findings:

  • The patient was diagnosed with microscopic polyangiitis (MPA).
  • Aggressive immunosuppressive therapy led to successful treatment of MPA.
  • Pulmonary fibrosis stabilized with MPA treatment.

Implications:

  • MPA can present initially with isolated pulmonary fibrosis (CFA).
  • Early recognition and immunosuppression are vital for managing MPA.
  • This case highlights the importance of considering systemic vasculitis in unexplained fibrotic lung disease.

More Related Videos

Oropharyngeal Administration of Bleomycin in the Murine Model of Pulmonary Fibrosis06:03

Oropharyngeal Administration of Bleomycin in the Murine Model of Pulmonary Fibrosis

Published on: May 9, 2025

Refined Murine Model of Idiopathic Pulmonary Fibrosis07:51

Refined Murine Model of Idiopathic Pulmonary Fibrosis

Published on: June 17, 2025

Related Concept Videos

Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...
Atypical Pneumonia01:14

Atypical Pneumonia

Atypical pneumonia, often caused by Mycoplasma pneumoniae, is a form of pulmonary infection that differs from the classical presentation of bacterial pneumonia in both its cause and clinical symptoms. Mycoplasma pneumoniae is a pleomorphic bacterium notable for its lack of a rigid cell wall. This structural characteristic imparts resistance to beta-lactam antibiotics and significantly influences the bacterium’s behavior within the human host.Other pathogens responsible for the disease include...
Chronic Obstructive Pulmonary Disease II: Emphysema01:23

Chronic Obstructive Pulmonary Disease II: Emphysema

Emphysema, a major phenotype of chronic obstructive pulmonary disease (COPD), is characterized by irreversible destruction of alveolar walls and permanent enlargement of distal airspaces. Unlike chronic bronchitis, which primarily affects the airways, emphysema predominantly involves the lung parenchyma, where structural damage leads to airflow limitation.PathophysiologyIt most commonly results from prolonged exposure to cigarette smoke and other toxic gases, particularly cigarette smoke.