Clinical Features of Systemic Sclerosis–Mixed Connective Tissue Disease and Systemic Sclerosis Overlap Syndromes

Abstract

Objective To describe the clinical characteristics and outcomes of systemic sclerosis–mixed connective tissue disease (SSc–MCTD) and SSc overlap syndrome. Methods We included patients from the Australian Scleroderma Cohort Study who met American College of Rheumatology/European Alliance of Associations for Rheumatology criteria for SSc. Three mutually exclusive groups were created: SSc–MCTD, SSc overlap, and SSc only. Univariate comparison of clinical features was performed by analysis of variance or chi‐square test. Survival analysis was performed using Kaplan‐Meier (KM) curves and Cox proportional hazards regression models. Results Of 1,728 patients, 97 (5.6%) had SSc–MCTD, and 126 (7.3%) had SSc overlap. Those with MCTD–SSc were more commonly Asian (18.3% versus 10.1% in SSc overlap, and 3.6% in SSc only; P < 0.0001) and younger at disease onset (38.4 years versus 46.5 or 46.8 years, P < 0.0001). Those with SSc–MCTD or SSc overlap were more likely to have limited cutaneous SSc. All 3 groups had similar frequency of interstitial lung disease (ILD), although pulmonary arterial hypertension (PAH) was less common in SSc overlap. Synovitis and myositis were more common in SSc overlap and SSc–MCTD than in SSc only. KM curves showed better survival in SSc–MCTD than SSc overlap or SSc only (P = 0.011), but this was not significant after adjustment for sex and age at disease onset. SSc‐specific antibodies were survival prognostic markers, with antinuclear antibody centromere or anti‐RNP conferring better survival than anti–Scl‐70 or anti–RNA polymerase III (P = 0.005). Patients with SSc–MCTD and SSc overlap had lower mortality following diagnosis of ILD and PAH than patients with SSc only. Conclusion This study provides insights into the clinical characteristics of patients with SSc–MCTD, SSc overlap, and SSc only and shows that anti‐RNP antibodies are associated with better survival than anti–Scl‐70 and anti‐RNA polymerase III antibodies.

Nailfold capillary scleroderma pattern may be associated with disease damage in childhood-onset systemic lupus erythematosus: important lessons from longitudinal follow-up

“…Interestingly, our data do suggest that patients with cSLE with anti-RNP seem to be a more severe subgroup with more SLE-related disease damage, which was also stated by Dayal and Isenberg 28. On the other hand, a recent study in SSc showed that the presence of anti-RNP antibodies fits with a subgroup of SSc with a better prognostic outcome in terms of survival 29. Thus, the detection of anti-RNP antibodies might have a different prognostic meaning in these different systemic autoimmune diseases or scleroderma spectrum disorders.…”

“… 28 On the other hand, a recent study in SSc showed that the presence of anti-RNP antibodies fits with a subgroup of SSc with a better prognostic outcome in terms of survival. 29 Thus, the detection of anti-RNP antibodies might have a different prognostic meaning in these different systemic autoimmune diseases or scleroderma spectrum disorders. Most patients with anti-RNP antibodies in the scleroderma pattern group also had multiple, more SLE-specific, auto-antibodies detected, besides other clinical SLICC criteria.…”

Nationwide and international registries on scleroderma. Past, present, and future

“…The ASIG registry has contributed to the field of PAH screening 123,124 , GI involvement 125 , overlap syndrome 126 , quality of life 127 , and activity impairment 128 . Furthermore, in the immunological characterization of patients, especially patients with anti-Ku, anti-RNA pol III, and anti-PM75-100 86,[129][130] , also the relation with occupational silica exposure 131 .…”

Síndrome De Sobreposição Esclerose Sistêmica E Lúpus Eritematoso Sistêmico: Perfil Epidemiológico

“…Acerca dele, a literatura sugere que os pacientes têm curso de doença e envolvimento de órgãos diferentes daqueles que atendem puramente aos critérios não superpostos. Em vista disso, esses pacientes, com SP ES-LES devem ser considerados como um subgrupo distinto [7][8][9] . A denominação SP é utilizada para aqueles que manifestam características de múltiplas DDTC mas que não apresentam o anticorpo anti--RNP em altos títulos.…”

“…A denominação SP é utilizada para aqueles que manifestam características de múltiplas DDTC mas que não apresentam o anticorpo anti--RNP em altos títulos. Se o paciente apresentar fenômeno de Raynaud ou puffy hands, associada às manifestações clínicas de pelo menos duas das DDTC, LES e ES ou dermatomiosite, e anti-RNP em altos títulos, a entidade passa a se chamar doença mista do tecido conjuntivo 8 .…”