ANCA negative pulmonary vasculitis: a challenging diagnosis

Area of Science:

• Nephrology
• Pulmonology
• Rheumatology

Background:

• A 40s male with end-stage kidney disease (IgA nephropathy) on peritoneal dialysis presented with respiratory symptoms.
• Initial presentation included breathlessness, cough, and nosebleeds, with chest CT showing ground-glass changes.

Observation:

• Elevated inflammatory markers were noted, but vasculitis screening (ANCA, anti-GBM) was negative.
• Symptoms worsened despite antibiotics for suspected atypical pneumonia, necessitating oxygen support and causing hemoptysis.

Findings:

• Clinical deterioration and radiological findings suggested ANCA-negative pulmonary vasculitis or IgA-associated pulmonary capillaritis.
• Treatment with methylprednisolone and rituximab resulted in rapid clinical improvement and oxygen withdrawal.

Implications:

• This case underscores the importance of considering ANCA-negative vasculitis in patients with kidney disease and unexplained pulmonary symptoms.
• Early diagnosis and aggressive immunosuppressive therapy are crucial for managing this rare condition.