A 49 year old woman with polyneuropathy, glomerulonephritis, leucocytoclastic vasculitis, restrictive lung disease, scleroderma-like skin changes, and myalgia, developed progressive myopathy, autoantibodies against voltage gated calcium channels (VGCCs) and a typical electromyographic pattern in accordance with a Lambert-Eaton myasthenic syndrome (LEMS). HLA typing showed that the patient carried the 8.1 ancestral haplotype (HLA-A1, B8, DR3), associated with multiple immunological diseases.¹ To our knowledge, this is the first presentation of LEMS occurring in a patient with undifferentiated connective tissue disease.

CASE REPORT

Our patient initially presented at the age of 32 (in 1987) with sicca syndrome and Raynaud’s phenomenon followed by arthralgia, malaise, and paraesthesia. She subsequently developed leucocytoclastic vasculitis, restrictive lung disease, scleroderma-like skin changes, and proteinuria responsive to steroids. Rheumatoid factor was occasionally positive, while C4 was persistently low to undetectable. SSB and anti-Sm antibodies were once weakly positive (but never thereafter), while low titres of antinuclear antibodies, IgM and IgG immune complexes were increased on several visits. SSA, dsDNA, U1RNP, phospholipid, cANCA, pANCA, Jo-1, and centromere antibodies were negative on several occasions. Thus, her clinical and laboratory investigations did not allow …