IgG4-related kidney disease
ORPHA: 449395
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 kidney disease, sourced from HPO and Orphanet clinical annotations.
Tubulointerstitial nephritisIncreased circulating IgG concentrationIncreased circulating IgG4 levelRenal insufficiencyProteinuriaHematuriaAcute kidney injuryRheumatoid factor positiveElevated circulating creatinine concentrationAntinuclear antibody positivityReduced circulating complement concentrationDecreased circulating complement C3 concentrationChronic kidney diseaseIncreased circulating IgG1 levelRenal interstitial immunoglobulin depositsDecreased circulating complement C4 concentrationProstatitisEnlarged kidneyDacryocystitisCholecystitisMeningitisDecreased liver functionAbnormal aortic morphologyPericarditisPancreatitisWeight lossIncreased total eosinophil countAbdominal painAbnormal lung morphologyPleuritisLymphadenopathyLymphadenitisIncreased circulating IgE concentrationRetroperitoneal fibrosisUreteral obstructionInterstitial pneumonitisChronic sinusitisInflammatory abnormality of the skinAbnormality of the anterior pituitaryArteritisFatigueMembranous nephropathyAlbuminuriaNephrotic range proteinuriaAbnormal ureter morphologySclerosing cholangitisDecreased circulating retinol-binding protein concentrationAbnormal breast morphologySialadenitisLymphocytoma cutisAbnormal mediastinum morphologyAbnormal mesentery morphologyUrinary bladder inflammationThyroiditisUrethritisHydronephrosisPedal edemaElevated circulating C-reactive protein concentration
Classification & Codes
Orphanet Code
ORPHA:449395IgG4-related kidney disease
| Orphanet | ORPHA:449395 |
| Treatments | 0 drug(s) |
| Symptoms on record | 58 signs |
| Status | published |
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO