The pathophysiology of penicillamine-induced myasthenia gravis.
The temporal course and pathophysiology of penicillamine-induced myasthenia gravis were studied in detail in a typical case. Our results suggest that this disorder and idiopathic autoimmune myasthenia gravis share the same essential pathophysiological features, including the presence of anti-acetylcholine receptor (AChR) antibody, serum-induced blockade of AChRs, antibody-mediated accelerated degradation of AChRs, and a resultant quantitative reduction in available junctional AChRs. An initial severe reduction in junctional AChRs was reversed and the patient recovered, both within 8 months of stopping penicillamine. Our data suggest that penicillamine probably produced myasthenia gravis by initiating a new autoimmune response rather than by enhancing ongoing autoimmunity.
D000818:Animals; D000906:Antibodies / Q000032:analysis; D006801:Humans; D008297:Male; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000139:chemically induced* / Q000276:immunology / Q000503:physiopathology; D009469:Neuromuscular Junction / Q000276:immunology / Q000378:metabolism; D010396:Penicillamine / Q000009:adverse effects*; D051381:Rats; D011950:Receptors, Cholinergic / Q000276:immunology / Q000378:metabolism
D000906:Antibodies; D011950:Receptors, Cholinergic; D010396:Penicillamine