Adults
ANCA-associated vasculitis with organ threat
Rising creatinine with active urine sediment, lung hemorrhage or mononeuritis multiplex. Same-day contact; often admission.
What to look for
- Rapidly progressive glomerulonephritis: rising creatinine, blood and protein on urinalysis, RBC casts
- Diffuse alveolar hemorrhage: hemoptysis, a falling hemoglobin, new infiltrates or hypoxia
- Mononeuritis multiplex (foot or wrist drop), scleritis, or stridor from subglottic stenosis
- GPA: chronic sinusitis, crusting, epistaxis, hearing loss, lung nodules or cavities
- EGPA: adult-onset asthma, eosinophilia, neuropathy, cardiac involvement
What to do
- Pulmonary hemorrhage or a rapidly rising creatinine needs the emergency department or admission. Contact rheumatology and nephrology directly.
- Order PR3- and MPO-ANCA by antigen-specific immunoassay, the preferred first test. Add urinalysis with microscopy, urine protein-to-creatinine ratio, creatinine, CBC, CRP and chest imaging.
- Don't wait for ANCA results when the picture is compelling. About 1 in 10 people with GPA or MPA, and most people with EGPA, are ANCA-negative, and biopsy may be needed.
Good to know
- Treatment now commonly combines rituximab or cyclophosphamide with lower-dose glucocorticoid regimens and, in selected patients, avacopan.
About this urgency level
Call rheumatology directly. Many of these need the emergency department or admission, and treatment often starts before the specialist visit.
Timeframes are typical targets. Local pathways, capacity and clinical judgment come first.