The 3-week sulphasalazine syndrome strikes again

A 34-year-old lady developed a constellation of dermatitis, fever, lymphade nopathy and hepatitis, beginning on the 17th day of a course of oral sulpha salazine for sero-negative rheumatoid arthritis. Cervical and inguinal lymp h node biopsies showed the features of severe necrotising lymphadenitis, as sociated with erythrophagocytosis and prominent eosinophilic infiltrates, w ithout viral inclusion bodies, suggestive of an adverse drug reaction. A week later, fulminant drug-induced hepatitis, associated with the presenc e of anti-nuclear autoantibodies (but not with other markers of autoimmunit y), and accompanied by multi-organ failure and sepsis, supervened. She subs equently died some 5 weeks after the commencement of her drug therapy. Post-mortem examination showed evidence of massive hepatocellular necrosis, acute hypersensitivity myocarditis, focal acute tubulo-interstitial nephri tis and extensive bone marrow necrosis, with no evidence of malignancy. It is thought that the clinico-pathological features and chronology of this ca se bore the hallmarks of the so-called "3-week sulphasalazine syndrome", a rare, but often fatal, immunoallergic reaction to sulphasalazine. (C) 2001 Elsevier Science Ireland Ltd. All rights reserved.