326 DRESS syndrome should be included in the differential diagnosis of life-threatening systemic symptoms in patients with cystic fibrosis

Abstract

A stable 20-year-old patient with CF (M, F508del/F508del, last FEV1: 92% pr) was admitted for sudden onset of high-grade fever, FEV1 drop (72% pr) without increased cough, diffuse pain and generalized lymphadenopathies. Initial check-up revealed the presence of prominent and very recent bilateral hilar lymphadenopathies along with a marked inflammatory syndrome. In 4 weeks, IgG level has risen from 15.7 to 30.4 g/L. Within 24 hours, the patient developed a generalized non-pruritic purpuric rash. Despite broad-spectrum IV AB (tobramycin and ceftazidime), daily fever persisted (up to 40°C). On the 6th day, the clinical picture worsened and became life-threatening with impaired renal function, multiple serositis, need for supplemental oxygen, hepatic cytolysis and coagulation abnormalities, major hypergammaglobulinemia (43.9 g/L) and marked hypereosinophilia (4.870/µL). At this point, all non-essential drugs were interrupted with the exception of ceftazidime. Within 7 days, clinical, biological and radiological features all improved. The patient was released under his previous outpatient treatment and without precise diagnosis as all hematologic, autoimmune and viral detection tests were negative. He came back 2 days later due to the recurrence of malaise, fever, rash and biological abnormalities. Rifampicin and moxifloxacin were immediately interrupted and the picture resolved quickly. The patient then disclosed that he kept taking these drugs (no more prescribed) till day 6 of the hospitalization. We concluded that our patient suffered from a drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome, rifampicin being the most likely causal agent.