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Systemic connective tissue disorders & Vasculitis: Specific syndromes

Autosomal RecessiveImmune/AntibodyOMIM 615688
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Overview

From WUSTL Neuromuscular

Rheumatoid Arthritis: Neuropathy syndromes Vasculitic Neuropathy 43 Epidemiology 10% of all RA 35% to 63% of RA with systemic vasculitis Longstanding disease: ~ 15 years after onset of RA Clinical Onset: Acute or Subacute Common pattern: Mononeuritis multiplex Modalities: Sensory-Motor or Sensory Multifocal or Asymmetric in 2/3 Nerves involved Peroneal (90%) Popliteal (50%) Ulnar (35%) Median (15%) Prognosis Worse with Cutaneous vasculitis Peripheral neuropathy Low serum CH50 & C4 complement RA + Vasculitis: Course No treatment: 5 yr survival 10% Immunosuppression: 5 yr survival 80% Systemic a

OMIM Entries

OMIM #615688OMIM #607575OMIM #182410

Related Conditions

Shared genes
POLYMYALGIA RHEUMATICA
DRB1TNF
Sarcoidosis
TNFTNF-

External Resources

WUSTL Neuromuscular
Washington University Disease Center
OMIM
Online Mendelian Inheritance in Man
PubMed
Biomedical Literature
GeneReviews
NCBI Expert-Authored Reviews
Orphanet
Portal for Rare Diseases
NORD
National Organization for Rare Disorders

Data sourced from the Washington University Neuromuscular Disease Center. For clinical use, always refer to primary sources.