Two patients with microscopic polyangiitis and unusual pulmonary manifestation
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Published on: March 8, 2019
Jae Joong Baik1, Yong Duk Jeon, Yeontae Chung
1The Department of Internal Medicine, National Medical Center, Seoul, Korea. jjbaik@lycos.co.kr
Abstract:
We encountered two patients with microscopic polyangiitis (MPA) associated with unusual pulmonary manifestations. The first patient was a 45-year-old man who had worked in amine for 3 years when he was young. On admission, chest X-rays showed long-standing silicosis and a new patchy infiltration. The second patient was a 52-year-old female. On admission, chest X-rays showed bilateral patchy infiltrations. Since then, variable patterns of patchy infiltration have waxed and waned repeatedly. The renal biopsy revealed that both patients had glomerulonephritis associated with small vessel vasculitis but with few or no immune deposits. There was neither granulomatous inflammation nor eosinophilic infiltration. Myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA) was positive in both patients. After treatment with glucocorticoids and cyclophosphamide, radiological findings were minimal and stable. These two cases show that patients with MPA have a wide spectrum of radiological findings.
Insights
Microscopic polyangiitis (MPA) can present with varied lung issues, including silicosis and patchy infiltrations. This study highlights the diverse radiological findings in MPA patients, emphasizing the need for comprehensive diagnosis.
Area of Science:
- Nephrology
- Pulmonology
- Rheumatology
Background:
- Microscopic polyangiitis (MPA) is a small vessel vasculitis often associated with glomerulonephritis and pulmonary involvement.
- Pulmonary manifestations in MPA typically include diffuse alveolar hemorrhage or nodules, but atypical presentations can occur.
Observation:
- Two patients with MPA presented with unusual pulmonary findings: one with long-standing silicosis and new patchy infiltrations, the other with recurrent bilateral patchy infiltrations.
- Renal biopsies confirmed glomerulonephritis with pauci-immune small vessel vasculitis in both cases, negative for granulomatous inflammation or eosinophilic infiltration.
- Both patients tested positive for myeloperoxidase-antineutrophil cytoplasmic antibodies (ANCA).
Findings:
- The radiological findings in these MPA patients demonstrated a broad spectrum beyond typical presentations.
- Treatment with glucocorticoids and cyclophosphamide led to minimal and stable radiological improvements.
- The cases underscore the variability of pulmonary manifestations in microscopic polyangiitis.
Implications:
- Clinicians should consider MPA in patients with unexplained pulmonary infiltrates, even in the presence of occupational lung diseases like silicosis.
- Recognition of diverse radiological patterns is crucial for timely diagnosis and management of MPA.
- This study expands the understanding of the clinical spectrum of microscopic polyangiitis, particularly its pulmonary manifestations.
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