1. 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 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 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 June 2002
No uveitis relapse
Oral steroids decreased to 16 mg/d
Pulmonary TBC
Stop Infliximab. Start antiTB therapy
June 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 March 2007
Mycophenolate mofetil 2-3 gr/day
3 uveitis relapses, oral ulcers, arthralgia
Cataract OU surgically operated (phacoemusification + IOL implantation)
Stop Mycophenolate
April 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