Naslov
Treatment challenges in patient with refractory adult Still's disease - a case report

Autori
Sentić, Mirna ; Barešić, Marko ; Anić, Branimir ; Bosnić, Dubravka ; Mayer, Miroslav ; Cerovec, Mislav ; Mihelčić Čikeš, Nada

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, stručni

Izvornik
Abstracts extracted from XIII Mediteranean congress of Rheumatology / Grazio S, Ivanišević G, Durmiš Kovač K - , 2009, 733-733

Skup
XIII Mediteranean Congress of Rheumatology

Mjesto i datum
Cavtat, Hrvatska, 18.11.2009. - 21.11.2009

Vrsta sudjelovanja
Predavanje

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Still's dissease
(Still's disease; refractory; therapy; treatment; IL-1 blocade)

Sažetak
We present a case of 32-year-old female with treatment refractory adult Still’s disease. Initial symptoms started in 2004 as migratory arthralgias and culminated in 2005 as painfull swelling of the right arm with periods of prolonged fevers, fatigue and malaise. Thorough evaluation excluded infectious origin of the fever and well defined inflammatory systemic diseases. Apart from elevated acute phase reactants (ESR, CRP) other laboratory findings were non-significant. Diagnosis of seronegative spondyloarthritis with erosive sacroileitis has been established and glucocorticoid, sulphasalazine and NSAR therapy initiated. Headaches were the reason for stopping sulphasalazine and introducing azathioprine with minimal clinical improvement. Few months later erythematous macular rush on the palmar and photoexposed skin developed. Diagnosis of adult form of Still’s disease has been established and she started with IVIg (every three to four weeks). Despite combined therapy (glucocorticoid, azathioprine and IVIg) low grade fever continued, sometimes with itching and sweating. Iron substitution was initiated due to pronounced anemia. Due to relatively high doses of imunosuppressive drugs needed to control the disease significant comorbidities and side effects occured (iatrogenic Cushing's syndrome and repeated urinary infections and bilateral pneumonia). Because of premanently active disease azathioprine was replaced with cyclosporin A. Several weeks later she has been hospitalized again with abdominal pain most probably caused by adnexitis. Ineffective cyclosporin has been replaced with MTX. During further reevaluation acute phase reactants and fever persisted and MTX was switched to cyclophosphamide, but due to nausea, vomiting and headache patient has been switched back to MTX. Attempt to switch patient to MMF was also unsuccessful because of nausea, vomiting, headache and vertigo. In April 2009 a new re-examination of the patient’s condition showed active disease while taking high doses of glucocorticoids, MTX, IVIg and NSAR. The decision to start anakinra subcutaneously with low-dose glucocorticoid was made. The patient’s general condition and acute phase reactants rapidly and dramatically improved. Unfortunately, because of the most common side-effect – injection-site reaction (well-defined erythema and edema) anakinra application was spaced and later on tapered. Several unsuccessful treatment regimens and side-effects to a number of drugs make the future treatment options very limited.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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