systemic scleroderma

MeSH: D012595ORPHA: 90291

Overview

scleroderma that is characterized by fibrosis (or hardening) of the skin and major organs, as well as vascular alterations, and autoantibodies

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 systemic scleroderma, sourced from HPO and Orphanet clinical annotations.

Thickened skinArthralgiaMyalgiaAntinuclear antibody positivityAbnormality of the gastrointestinal tractRaynaud phenomenonCutaneous sclerotic plaqueAbnormality of the kidneyNarrow mouthTelangiectasiaNail bed telangiectasiaMuscle weaknessJoint swellingAbnormal esophagus morphologyPulmonary fibrosisElevated circulating creatine kinase concentrationSpotty hypopigmentationAcral ulcerationAbnormal phalangeal joint morphology of the handAbnormal pulmonary interstitial morphologyIrregular hyperpigmentationSclerodactylyPainFinger swellingCalcinosis cutisAnti-topoisomerase I antibody positivityAnti-centromere antibody positivityDigital pitting scarDigital ulcerAnti-RNA-polymerase-III-autoantibody positivityAnti-carbonic anhydrase II antibody positivityAnti-PM-Scl antibody positivityAnti-centromere protein A antibody positivityAnti-centromere protein B antibody positivityAnti-U3 RNP antibody positivityAnti-Th/To antibody positivityAnti-bicaudal D2 antibody positivityAnti-nucleolus-organizing region antibody positivityAnti-PM-Scl100 antibody positivityAnti-PM-Scl75 antibody positivityAnti-U11/U12 RNP antibody positivityAnti-Ku antibody positivityAnti-B23 antibody positivityAnti-RuvBL1/2 antibody positivityAnti-platelet derived growth factor receptorAnti-Ro52/TRIM21 antibody positivityAnti-angiotensin receptor type-1 antibody positivityAnti-endothelin-1 type A receptor antibody positivityAnti-angiotensin-converting enzyme 2 antibody positivityAnti-platelet antigen antibody positivityAnti-voltage-gated potassium channel antibody positivityRenal insufficiencyProteinuriaGlomerulonephritisHypohidrosisPruritusSyncopeArthritisFlexion contractureRecurrent skin infectionsAlopeciaPericarditisRight ventricular failureAcute kidney injuryDysphagiaGastroesophageal refluxPulmonary arterial hypertensionDyspneaAbnormal small intestine morphologyAbnormal large intestine morphologyAbnormal stomach morphologyGastroparesisIntestinal bleedingGastrointestinal telangiectasiaBowel incontinenceVascular dilatationOsteomyelitisOsteolytic defects of the phalanges of the handAbnormality of facial soft tissueConstrictive median neuropathyAlbuminuriaChronic kidney diseaseMyocarditisInterstitial cardiac fibrosisBarrett esophagusGangrene

Classification & Codes

MeSH Code

D012595

Orphanet Code

ORPHA:90291
systemic scleroderma
MeSHD012595
OrphanetORPHA:90291
Treatments0 drug(s)
Symptoms on record86 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO
systemic scleroderma | OrphanDrug