Systemic Sclerosis Sine Scleroderma (ssSSc) is a less common form of systemic sclerosis that can be a challenge to diagnose.
Frequency reported: 10% of patients with systemic sclerosis
Clinical findings:
(1) absence of skin fibrosis and sclerodactyly
(2) variable skin telangiectasia
(3) variable digital findings (puffy fingers, pitting ulcers, digital ulcers, Raynaud's)
(4) variable gastrointestinal symptoms (esophagus, stomach, intestines)
(5) variable synovitis
(6) presence of autoantibodies
Autoantibodies that may be present:
(1) anti-Scl-70
(2) anticentromere
(3) antinuclear antibodies
(4) anti-topoisomerase I
(5) anti-RNA polymerase antibodies
(6) anti-U1 ribonuclease proteins
Skin telangiectasia are associated with diastolic dysfunction.
The presence of anti-Scl-70 or anti-RNA polymerase antibodies as associated with delayed appearance of skin fibrosis.
Complications:
(1) pulmonary fibrosis (> 40%)
(2) renal crisis (3%)
