Our study indicated that there is important clinical value of anti-SmD1 Abs in the SLE as the positivity for anti-SmD1 only was found in 14.00% of naive SLE patients and 16.00% of non-naive SLE patients, and the presence of anti-SmD1 antibodies was significantly associated with malar rash, rash, nonscarring alopecia, renal disorder, proteinuria, seizures, PAH, and hypocomplementemia. This evidence concerns the gene SNRPD1 and pulmonary arterial hypertension.