IgG4-related aortitis
ORPHA: 449400
Overview
human disease
Available Treatments (0)
No treatments linked yet
Clinical trials or compassionate use may be available — consult a specialist.
Clinical Presentation
Signs and symptoms associated with IgG4-related aortitis, sourced from HPO and Orphanet clinical annotations.
Increased circulating immunoglobulin concentrationIncreased circulating IgG4 levelWeight lossFeverAbdominal painAsthmaAutoimmunityIncreased circulating IgE concentrationLow back painAntinuclear antibody positivityElevated erythrocyte sedimentation rateElevated circulating C-reactive protein concentrationAllergyIncreased inflammatory responseThoracic aortic aneurysmSeverely increased total eosinophil countHydronephrosisAortic dissectionReduced circulating complement concentrationAscending tubular aorta aneurysmIntestinal obstructionAbnormal aortic arch morphologyDilated left subclavian arteryCytoplasmic antineutrophil antibody positivityAbnormal common carotid artery morphology
Classification & Codes
Orphanet Code
ORPHA:449400IgG4-related aortitis
| Orphanet | ORPHA:449400 |
| Treatments | 0 drug(s) |
| Symptoms on record | 25 signs |
| Status | published |
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO