Università degli Studi di Roma “La Sapienza” Dipartimento di Scienze Oftalmologiche - Servizio Immunovirologia Oculare Multi-drug resistance and side-effects in a patient with Behçet’s disease M. Accorinti, M. Gilardi, D. De Geronimo, R. Spena, P. Tortorella Introduction: Behçet’s disease (BD) ocular manifestations are one of the few ocular diseases requiring immunosuppressive therapy as the first line treatment. Recently new drugs have become available for the treatment of BD, nevertheless some patients seem to be anyway unresponsive. We report the case of a patient multiresistant to immunosuppressive treatments who developped also many immunosuppressive-related side-effects 1998 22 years old male Diagnosis of BD (oral ulcers, erythema nodosum, arthralgia, epididymitis, bilateral panuveitis and retinal vasculitis, HLAB51+). Tx: Predn 1 mg/kg/d + CyA 5 mg/kg/d 1999 5 uveitis relapses, 4 episodes of pyodermitis, relapsing oral ulcers, ematuria, melena. All examin. neg Tx: oral (min 17,5 mg/day) + peribulbar steroids + CyA 5 mg/kg/d January - March 2000 MTX 20 mg/w, CyA 4 mg/kg + steroids Uveitis relapses, arthralgia and pyodermitis. Increase creatinine, proteinuria, ematuria. Stop CyA. High dose i.v. steroids December 2000 – June 2001 Erythema nodosum and relapsing arthralgia Progressive increase of CHL up to 12.5 mg/d Vertebral fracture. Leukopenia Stop CHL. 20 days after uveitis relapse OU June – December 2000 Uveitis relapse OU, depression, panic attack, one suicide attempt. Cushing syndrome Stop IFN. Start CHL 5 mg/kg 10 days after uveitis relapse March - June 2000 Start IFN 3M/U 3 times/w + steroids+ MTX 15/w Oral ulcers, EN, uveitis relapse RE Increase liver enzymes Stop MTX July 2001 - January 2002 Start CyA 5 mg/kg + oral steroids 4 uveitis relapses, progressive decrease of renal function. nephrolithiasis. Progressive decrease Cya A dosage Stop CyA February 2002 - April 2002 Start MTX 15 mg/w + Aza 100 mg/d 3 uveitis relapses, increase liver enzymes Stop MTX + AZA. Start Infliximab 5 mg/kg April 2002 - June 2002 No uveitis relapse Oral steroids decreased to 16 mg/d Pulmonary TBC Stop Infliximab. Start antiTB therapy June 2002 - January 2005 Completed tx for TB. Patient refused prophylaxis Tx steroids (min prednisone 30 mg/d) 14 uveitis relapses, EN, arthralgia, ureteral lithiasis (2 surgical procedures), 2 vertebral fractures November 2005 Completed TB prophylaxis Start infliximab 5 mg/kg After 3° infusion : pulmonary legionellosis Stop infliximab January 2005 – October 2005 Start AZA 100 mg/d + TB prophylaxis + oral steroids Steroid induced glaucoma, 4 uveitis relapses, additional vertebral fractures Last examination April 2010 RE: Visual acuity 9/10 LE: hand motion Intraocular pressure 16 mmHg (under topical and systemic therapy) No signs of ocular inflammation Chronic tx: prednisone 25 mg/d December 2005 - March 2007 Mycophenolate mofetil 2-3 gr/day 3 uveitis relapses, oral ulcers, arthralgia Cataract OU surgically operated (phacoemusification + IOL implantation) Stop Mycophenolate April 2007 - January 2010 Oral steroids (mean prednisone 25 mg/d) 2 uveitis relapses after MMF stop Then free of ocular and systemic symptoms Conclusions Usually Behçet’s disease patients are responsive to immunosuppressive drugs, especially the newest ones, such as infliximab. Nevertheless there is also the possibility of a multi-drug resistance as well as of multiple drug-related side-effects. After quite a long period of activity the disease can eventually quite down