Scleroderma-specific autoantibodies embedded in immune complexes mediate endothelial damage: an early event in the pathogenesis of systemic sclerosis

Table 1 Demographic and clinical data of enrolled SSc patients

	ATA (n = 4)	ACA (n = 4)	ARA (n = 2)	Anti-Th/To (n = 2)
Age (years)	51 [44.25–55.25]	51 [43.5–60]	37.5 [31.25–43.75]	41.5 [38.75–44.25]
Females (%)	4 (100%)	4 (100%)	2 (100%)	2 (100%)
Disease duration (months)*	31 [29.5–33.5]	30.5 [26–34.5]	28 [25–31]	31.5 [30.25–32.75]
dcSSc/lcSSc°	3/1	3/1	2/0	0/2
ILD (%)^¶	2 (50%)	0	0	1 (50%)
PAH (%)^§	0	1 (25%)	0	1 (50%)
Joint involvement (%)	1 (25%)	0	1 (50%)	0
SRC (%)^#	0	0	0	0
Muscle involvement (%)^$	0	0	0	1 (25%)
Severe GI involvement (%)^&	0	1 (25%)	0	0
Heart involvement (%)^£	1 (25%)	0	0	0
Digital ulcers (%)	1 (25%)	1 (25%)	0	0
Raynaud’s phenomenon (%)	4 (100%)	4 (100%)	2 (100%)	1 (50%)
DMARDs
HCQ	1	2	1	1
AZA	0	0	0	1
MTX	2	0	1	0
MMF	1	0	0	0

Continuous variables are expressed as median [interquartile range]
dcSSc diffuse cutaneous systemic sclerosis, lcSSc limited cutaneous systemic sclerosis, RP primary Raynaud’s phenomenon, n number, ACA antibodies against centromeric proteins, ATA antibodies against DNA topoisomerase I, ARA antibodies against RNA polymerase III, anti-Th/To antibodies against Th/To, ILD interstitial lung disease, SRC scleroderma renal crisis, GI gastrointestinal, DMARDs disease-modifying anti-rheumatic drugs, HCQ hydroxychloroquine, AZA azathioprine, MTX methotrexate, MMF mycophenolate
*From the onset of the first non-Raynaud’s phenomenon symptom to study inclusion
^¶Forced vital capacity (FVC) or carbon monoxide diffusing capacity of the lung (DL_CO) < 55% of predicted or a 15% decline from baseline in FVC or DL_CO, with evidence of fibrosis on high-resolution CT
^§Mean pulmonary arterial pressure > 25 mmHg at right heart catheterization
^#New-onset systemic hypertension > 150/85 mmHg with a decrease in the estimated glomerular filtration rate > 30%
^$Objective muscle weakness (score < 4 on a 5-point Likert scale) and an elevated total creatine kinase level (> 4-fold the upper limit of normal)
^&At least 3 episodes of intestinal pseudoobstruction requiring hospitalization or requiring > 6 weeks of enteral or parental nutritional support
^£Hemodynamically significant arrhythmias, pericardial effusion, or congestive heart failure
°According to LeRoy [16]

ISSN: 1478-6362