DRESS: Return of the 3-Week Sulfasalazine Syndrome …

Complications of complications: diagnosis and treatment of recurrent macrophage activation syndrome in a patient with well-controlled systemic juvenile idiopathic arthritis

“…While well documented in patients with inflammatory bowel disease and in patients with rheumatoid arthritis being treated with sulfasalazine, this is the first reported case in a rheumatic patient receiving prophylactic TMP/SMX. 14 15 Typical symptoms are fever, lymphadenopathy, dermatitis, haematological abnormalities and hepatitis around the third week of TMP/SMX initiation, 7 and treatment requires the withdrawal of the offending agent and may require the use of other immune modulators.…”

“…Treatment of sulfasalazine-induced 3-week syndrome and DRESS is discontinuation of the medication, so TMP/SMX was stopped, and prophylaxis was changed to intravenous pentamidine. 7 As with his first episode of MAS, pulse steroids stabilised him clinically but did little to improve his lab abnormalities. Given his incomplete response to emapalumab, he was started on ruxolitinib, based on recent findings in a series of patients with EBV-driven MAS and secondary haemophagocytic lymphohistiocytosis (HLH).…”

“…The timing and symptoms of his illness matched closely with that of both DRESS and sulfasalazine-induced 3-week syndrome, presenting approximately 3 weeks after starting TMP/SMX, a sulfasalazine-containing medication, and with symptoms of fever, rash, facial oedema, conjunctival oedema, haematological abnormalities and elevated transaminases. 7–9 DRESS was considered given his symptoms; however, he only scored a 4 on the RegiSCAR scoring system for DRESS, and he did not fulfil Bocquet’s or the Japanese criteria. 10 11 Bacterial and severe viral infections were ruled out with negative blood cultures and viral testing, and decreased EBV viral load.…”