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Penicillamine induced polymyositis and dermatomyositis
G J Carroll1, R K Will, J B Peter
1Department of Clinical Immunology, Royal Perth Hospital, Western Australia.
Abstract:
Eight Australian cases of D-penicillamine induced polymyositis/dermatomyositis (PM/DM) are reported. In terms of clinical, pathological and electromyographic features, D-penicillamine PM/DM is similar to idiopathic PM/DM but is generally less severe. Recovery is usually rapid when D-penicillamine is withdrawn. Sera were available for study in 6 of the 8 reported cases. Two of the 6 had elevated titers of acetylcholine receptor autoantibodies. Neither of these patients had clinical signs of myasthenia gravis. In 3 of 6 patients typed for C2, no bands were detected suggesting homozygous C2 deficiency. D-penicillamine PM/DM is associated with HLA-B18, B35 and DR4 and is immunogenetically different from idiopathic PM/DM, rheumatoid arthritis and D-penicillamine myasthenia gravis.
Insights
D-penicillamine can induce polymyositis/dermatomyositis (PM/DM), which is typically less severe than the idiopathic form. Withdrawal of the drug usually leads to rapid recovery, highlighting its role in drug-induced autoimmune conditions.
Area of Science:
- Rheumatology
- Immunology
- Neurology
Background:
- D-penicillamine is a chelating agent used to treat various conditions, including rheumatoid arthritis.
- Drug-induced autoimmune syndromes are increasingly recognized.
- Polymyositis/dermatomyositis (PM/DM) are inflammatory myopathies with distinct clinical and pathological features.
Observation:
- Eight Australian cases of D-penicillamine-induced PM/DM were identified.
- Clinical, pathological, and electromyographic findings were similar to idiopathic PM/DM but generally less severe.
- Rapid recovery was observed upon discontinuation of D-penicillamine.
Findings:
- Two of six patients had elevated acetylcholine receptor autoantibodies without clinical signs of myasthenia gravis.
- Homozygous C2 deficiency was suggested in three of six patients.
- D-penicillamine PM/DM is immunogenetically associated with HLA-B18, B35, and DR4.
Implications:
- D-penicillamine-induced PM/DM represents a distinct entity from idiopathic PM/DM and other D-penicillamine-related conditions.
- Understanding the immunogenetic associations may aid in diagnosis and management.
- Early recognition and drug withdrawal are crucial for favorable outcomes in D-penicillamine-induced PM/DM.