Early cyclophosphamide treatment in new-onset progressive cutaneous systemic sclerosis – a case report and literature review

Stela Hrkač, Joško Mitrović, Jadranka Morović-Vergles

Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by fibrosis of the skin and internal organs. Treatment of SSc remains a challenge, as it depends on disease extent, pattern and organ involvement. The use of cyclophosphamide (CYC) is mainly recommended for treatment of SSc interstitial lung disease, however its efficacy on skin involvement in SSc has been demonstrated by several studies. We present a case of a patient with new-onset progressive cutaneous diffuse SSc, who was successfully treated with a three-month course of CYC. Significant improvement of skin fibrosis was recorded, as well as a favourable disease outcome in the five-year follow up period. A brief literature review is also presented, demonstrating that CYC can be an effective and safe first-line treatment option in similar cases.

Sistemska skleroza (SSc) je kronična autoimunosna bolest koju karakterizira fibroza kože i unutarnjih organa. Liječenje SSc-a izazov je za liječnika jer izbor terapije ovisi o proširenosti i obrascu bolesti, kao i zahvaćenosti unutarnjih organa. Upotreba ciklofosfamida (CYC) većinom je preporučena za liječenje intersticijske plućne bolesti u SSc-u. Međutim, njegova učinkovitost u liječenju kožnih manifestacija SSc-a dokazana je u više istraživanja. Prikazujemo slučaj bolesnice s novonastalom progresivnom, difuznom kožnom SSc, koja je uspješno liječena upotrebom CYC-a tijekom tri mjeseca. Zabilježeno je značajno poboljšanje kožne fibroze, kao i povoljan ishod bolesti tijekom idućih pet godina praćenja. Prikazan je i kraći pregled literature koji govori u prilog tomu da CYC može biti učinkovita i sigurna prva linija liječenja u sličnim slučajevima.