Isolated pulmonary capillaritis
ORPHA:264691· ICD-10 J84.8
Definition
Isolated pauciimmune pulmonary capillaritis is a small vessel vasculitis restricted to the lungs that may induce diffuse alveolar hemorrhage with dyspnea, anemia, chest pain, hemoptysis, bilateral and diffuse alveolar infiltrates at chest X-rays, without any underlying systemic disease. ANCA are frequently positive but could be negative.
- Prevalence
- Unknown
- Inheritance
- Not applicable
- Age of onset
- Childhood