Clinical subsets, skin thickness progression rate, and serum antibody levels in systemic sclerosis patients with anti–topoisomerase I antibody

Abstract

Objective. To describe the clinical and laboratory features and natural history of the disease in systemic sclerosis (SSc; scleroderma) patients with antitopoisomerase I (anti-topo I) antibody who have different skin thickness progression rates (STPRs).Methods. SSc patients (n ‫؍‬ 212) who were antitopo I antibody positive were divided into 5 subgroups based on STPRs. Skin thickness was measured using the modified Rodnan skin thickness score (MRSS). Anti-topo I IgG antibody levels were determined.Results. Sixty patients who were anti-topo I antibody positive had diffuse cutaneous SSc (dcSSc) with rapid progression, 82 had dcSSC with intermediate progression, and 29 had dcSSc with slow progression, 14 had limited cutaneous SSc (lcSSc) that became dcSSc, and 27 had lcSSc that did not change throughout. Patients beginning with lcSSc were younger at disease onset and had longer disease duration when diagnosed as having SSc. Interstitial lung disease was common and was equally distributed across the subgroups. Renal crisis occurred most often in patients with rapid progression (22%) and was absent in lcSSc patients. Cardiac involvement was most frequent in the dcSSc subgroups. Both kidney and heart disease occurred most often within 3 years after the onset of skin thickening. The 10-year cumulative survival rate was <40% for patients with rapid and intermediate progression. Renal and cardiac causes of death were disproportionately frequent in these 2 subgroups. Anti-topo I antibody levels correlated with the STPR and the MRSS.Conclusion. Anti-topo I antibody-positive patients with SSc with a rapid STPR have reduced survival rates, primarily due to early and often fatal renal and cardiac involvement. Anti-topo I antibody levels parallel the MRSS at the first visit and the STPR. This information is important for managing physicians and researchers planning clinical trials involving patients with early dcSSc.

Effector CD8+ T cells in systemic sclerosis patients produce abnormally high levels of interleukin‐13 associated with increased skin fibrosis

“…Published international consensus recommendations for each organ system were used to determine disease severity (38). The time of disease onset was defined as the date of the first symptom attributable to SSc.…”

Systemic Scleroderma in Childhood: A Case Report

“…Early treatment initiation is crucial to achieve the maximum clinical benefit and avoid any irreversible fibrotic tissue damage. 9,26,27 Management approaches which can be appropriate for pediatric patients should be chosen considering any possible adverse effects of the medications (i.e., corticosteroid-induced growth failure and osteoporosis) as well as the psychosocial impact of the chronicity and physical deformity for both the child and his/her parents. Having no vital organ involvement and being diagnosed at an early stage, our patient was subcutaneously administered methotrexate 20 mg/week and low-dose steroid following intravenous immunoglobulin and pulse methylprednisolone.…”

Recent Advances in Systemic Scleroderma in Childhood

“…However, no controlled clinical trial has yet been conducted [41]. Individual risk factors for developing renal crises include new anaemia, new cardiac events, diffuse skin thickening, rapidly progressive skin thickening (a greater than 40 units of skin score increase per year), an SSc disease duration of more than 4 years, anti-RNA polymerase III antibody positivity and most importantly steroid use [50,51]. In conclusion, SSc, and steroid-recieving patients in particular, should be regularly monitored for renal function and blood pressure and if needed, treated with ACE-inhibitors according to the EULAR consensus [41].…”