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Updated: Mar 10, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Early interstitial lung disease in microscopic polyangiitis: Case report and literature review
Marcos García-Nava1, Heidegger Mateos-Toledo2, Ana Patricia Georgina Guevara-Canseco1
1Servicio de Medicina Interna, Centro Médico ISSEMyM Toluca, Metepec, Estado de México, México.
Abstract:
Microscopic polyangiitis (MPA) is a systemic disease included in the Chapel Hill 2012 Classification as necrotizing vasculitis affecting capillaries, venules and arterioles. It usually expresses antineutrophil cytoplasmic antibodies (ANCA) and has a perinuclear immunofluorescence pattern and correlation with anti-myeloperoxidase (MPO) antibodies. Capillaritis with alveolar hemorrhage is the most common manifestation of lung disease. Interstitial lung disease (ILD) is uncommon, with usual interstitial pneumonia being the predominant pattern. However, other patterns such as organizing pneumonia have been described. No guidelines exist for treating patients with ILD and, currently, ANCA-associated vasculitis (AAV) is managed along the lines of small vessel vasculitis. The prognosis with this association is uncertain, with possibilities of relapse and a fatal outcome. We present a case in which ILD was the first manifestation of MPA, without alveolar hemorrhage, with subsequent renal involvement and, in which, the established treatment produced a significant clinical improvement.
Insights
Microscopic polyangiitis can first appear as interstitial lung disease, a rare presentation. Early diagnosis and treatment of this ANCA-associated vasculitis led to significant clinical improvement in a patient.
Area of Science:
- Rheumatology
- Pulmonology
- Nephrology
Background:
- Microscopic polyangiitis (MPA) is a systemic necrotizing vasculitis characterized by antineutrophil cytoplasmic antibodies (ANCA), typically anti-myeloperoxidase (MPO).
- Lung involvement in MPA commonly presents as capillaritis with alveolar hemorrhage; interstitial lung disease (ILD) is an uncommon manifestation.
- Current management guidelines for ANCA-associated vasculitis (AAV) do not specifically address ILD, and its prognosis in MPA is uncertain.
Observation:
- This case highlights an unusual initial presentation of MPA where interstitial lung disease (ILD) preceded other systemic manifestations.
- The patient did not exhibit alveolar hemorrhage, a more typical pulmonary finding in MPA.
- Renal involvement subsequently developed, confirming the systemic nature of the vasculitis.
Findings:
- The patient's interstitial lung disease was the first clinical manifestation of microscopic polyangiitis.
- Treatment for ANCA-associated vasculitis resulted in significant clinical improvement, despite the atypical presentation.
- This case underscores the importance of considering MPA in patients with unexplained ILD.
Implications:
- This case suggests that ILD can be a primary presenting feature of MPA, necessitating a high index of suspicion.
- Effective management of AAV, even with unusual ILD manifestations, can lead to favorable outcomes.
- Further research is warranted to establish specific treatment guidelines for MPA-associated ILD.
