Systemic Sclerosis Sine Scleroderma and Systemic Lupus Erythematosus - A Rare Overlap Syndrome

Abstract

BACKGROUNDThere are many reports about systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) overlap syndromes, but few associations with the sine scleroderma form. We aim to relate a case and discuss whether all manifestations could be attributed to SLE. CASE REPORTWe report the case of a 60-year-old white female patient that presented to the Rheumatology Service with a one-year evolution of malnutrition and significant weight loss, as well as facial telangiectasias, dysphagia, polyarthritis and Jaccoud syndrome, oral ulcers, xerostomia, xerophthalmia, Raynaud syndrome without pitting scars, leukopenia with lymphocytopenia and confusional states with complex hallucinations. There was no skin induration upon physical examination. The patient was admitted for investigation. Laboratory workup showed a positive antinuclear factor of 1:5,120 with a cytoplasmic dense fine speckled pattern, anti-DNA of 1:10, isolated consumption of C4 and polyclonal gammopathy with an IgG of 3,261. She also showed indeterminate reactivity to anti-SSA and anti-U1-RNP autoantibodies. Anti-Sm, anti-SCl-70 and anti-centromere were negative. Chest tomography showed initial nonspecific interstitial pneumonia. Capillaroscopy was compatible with SD pattern and transthoracic echocardiogram revealed an elevated pulmonary artery systolic pressure. Precapillary pulmonary hypertension was confirmed by right ventricle catheterization, with a borderline pulmonary artery mean pressure of 21 mmHg. The patient was initially treated with pulse therapy with methylprednisolone 3 g/day for 3 days, with general status improvement. The patient fulfilled the 2019 EULAR/ACR classification criteria for SLE, as well as ACR/EULAR 2013 systemic sclerosis classification criteria. Azathioprine was initiated and the patient was discharged after two months on prednisone reduction. CONCLUSIONThis case showed a patient with mixed features of SLE and SSc, but neither scleroderma nor specific autoantibodies for systemic sclerosis were found. We do know that many of those manifestations could possibly be attributed to SLE, even the SD pattern being described in 5% of SLE patients. Only one case of SLE and systemic sclerosis sine scleroderma overlap was found on the literature. Overlap syndromes and even SLE are more prevalent in younger ages, the opposite of this patient. Overall, classification criterion for both diseases were fulfilled and the patient received adequate treatment directed to systemic manifestations.