Systemic lupus erythematosus

ICD-10: 4A40.0MeSH: D008180ORPHA: 536

Overview

Systemic lupus erythematosus is a chronic autoimmune disease with immune-complex mediated inflammation and multisystem involvement, driven by genetic susceptibility and environmental triggers. Common manifestations include fatigue, arthritis, rash, nephritis, cytopenias, and neurologic disease, with a relapsing-remitting course. It is not ultra-rare, but severe refractory forms qualify for specialty orphan-drug development in some settings, and biologic therapies are documented for selected patients.

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 lupus erythematosus, sourced from HPO and Orphanet clinical annotations.

Lupus nephritisAnti-ribosome Po antibody positivityAnti-complement component C1q antibody positivityDecreased circulating complement C4 concentrationAnti-titin antibody positivityOral ulcerProteinuriaWeight lossFeverAnorexiaAntinuclear antibody positivityFatigueMalaiseHematuriaHypertensionArthritisAlopeciaDecreased total leukocyte countAntineutrophil antibody positivityDecreased circulating complement C3 concentrationPolyarticular arthritisPyuriaMalar rashRaynaud phenomenonDepressionCutaneous photosensitivitySeizureThrombocytopeniaHemolytic anemiaLymphadenopathyDiscoid lupus rashAnti-dsDNA antibody positivityAnti-La/SS-B antibody positivityAnti-U1 ribonucleoprotein antibody positivityAnti-Sm antibody positivityAnti-nucleoporin 62 antibody positivityAnti-Ro52/TRIM21 antibody positivityAnti-phosphatidic acid antibody positivitySerositisAbnormal pigmentation of the oral mucosaAbnormality of salivationCheilitisRetinopathyChorea

Classification & Codes

ICD-10 Code

4A40.0

MeSH Code

D008180

Orphanet Code

ORPHA:536
Systemic lupus erythematosus
ICD-104A40.0
MeSHD008180
OrphanetORPHA:536
Treatments0 drug(s)
Symptoms on record44 signs
Statuspublished
Factual Authority
Last Updated7/24/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO