[Eosinophilia-myalgia syndrome with fasciitis and interstitial myositis after L-tryptophan administration]. / Eosinophilie-Myalgie-Syndrom mit Fasziitis und interstitieller Myositis nach L-Tryptophan-Einnahme.

We describe a 53-year-old women with eosinophilia-myalgia syndrome who suddenly developed severe persistent myalgias of her arms, legs, back, and shoulder after a 5-month period of daily L-tryptophan ingestion, associated with fever, progressive stenocardia and left-sided congestive heart failure. Laboratory tests showed a leukocytosis of 11.2/nl with 3.14/nl eosinophils and an elevated erythrocyte sedimentation rate. There was a marked, predominantly proximal sclerosis of her arms, legs and trunk with a brownish discoloration. The skin of her arms and legs appeared dimpled (peau d'orange). Findings of the electrophysiological examinations were consistent with sensory neuropathy and myositis. Remarkable fasciitis and interstitial myositis were present in a biopsy specimen (from skin to muscle) taken from her thigh. However, eosinophilic infiltrates were rare. Angiography revealed an apical obstructive cardiomyopathy. In this paper, we describe the clinical findings, the course over 2 years, as well as the therapeutic management. Furthermore, the most important differential diagnoses are discussed and the literature is reviewed with special attention given to more recent pathogenic insights into this newly recognized multisystem disease.

Immunogenetic associations of scleroderma-related antinuclear antibodies.

Patients selected for the presence of scleroderma-related antibodies (anti-DNA-topoisomerase I [anti-topo I; n = 43], anticentromere antibody [ACA; n = 63], or anti-Pm-Scl [n = 12]) were studied for class I and class II major histocompatibility complex antigens, as well as for Gm and Km allotypes. Anti-topo I was associated with HLA-DR5 (70% of patients versus 30.6% of controls; Pcorr = 0.0018, relative risk [RR] = 5.3). All patients with anti-Pm-Scl were positive for HLA-DR3 (versus 23.5% of controls; Pcorr less than 0.001); 6 of these patients were DR3/4 heterozygous (50% versus 3.5% of controls; Pcorr less than 0.001, RR = 27.3). Patients with ACA were frequently positive for HLA-DR1, DR4, or DRw8, with 73.7% demonstrating at least 1 of these alleles (versus 41.2% of controls; Pcorr = 0.0152, RR = 4.0). This group of ACA-positive patients who had DR1, DR4, and/or DRw8 consisted mainly of a subgroup of patients with rheumatoid arthritis. We conclude that different class II major histocompatibility complex antigens influence the formation of anti-topo I and anti-Pm-Scl. Important clinical differences between these patient groups and the immunogenetic heterogeneity support the notion of different antibody-defined scleroderma subsets.