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Diseases","Systemic Sclerosis (SSc)","Systemic sclerosis (SSc), also known as scleroderma, is a rare chronic autoimmune connective tissue disease characterized by abnormalities of small blood vessels, immune dysregulation, and fibrosis of the skin and, in some patients, internal organs.",[68,74,78,82,86],{"key":69,"title":70,"html":71,"icon":72,"updatedAt":73},"","Overview","\u003Cp style=\"margin: 7px 0px; text-align: left;\">\u003Cspan style=\"font-size: 15px;\">Systemic sclerosis is a systemic autoimmune disease involving three major processes: vascular injury, abnormal immune activity, and tissue fibrosis.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0px; text-align: left;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0px; text-align: left;\">\u003Cspan style=\"font-size: 15px;\">Skin changes are characteristic, but SSc is not simply a skin disorder. It can also affect the gastrointestinal tract, lungs, heart, kidneys, and musculoskeletal system.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0px; text-align: left;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0px; text-align: left;\">\u003Cspan style=\"font-size: 15px;\">The two major clinical subsets are limited cutaneous systemic sclerosis and diffuse cutaneous systemic sclerosis.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0px; text-align: left;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0px; text-align: left;\">\u003Cspan style=\"font-size: 15px;\">Limited cutaneous disease primarily affects the fingers, hands, forearms, and face, whereas diffuse cutaneous disease may extend to the upper arms, thighs, or trunk and is associated with a higher risk of certain internal organ complications.\u003C\u002Fspan>\u003C\u002Fp>","⚕️","2026-10-08",{"key":69,"title":75,"html":76,"icon":77,"updatedAt":73},"Causes and Risk Factors","\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">The exact cause of systemic sclerosis is unknown.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Current evidence suggests that genetic susceptibility, abnormal immune responses, vascular injury, and environmental factors may all contribute. Immune activation can stimulate fibroblasts and excessive collagen deposition, leading to fibrosis of the skin and internal organs.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">SSc has a genetic component, but it is not inherited in a simple pattern. Having a family member with the disease does not mean that other relatives will necessarily develop it.\u003C\u002Fspan>\u003C\u002Fp>","🧬",{"key":69,"title":79,"html":80,"icon":81,"updatedAt":73},"Signs and Symptoms","\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Symptoms vary widely between patients. Common manifestations include:\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cul class=\" list-paddingleft-2\" style=\"list-style-type: disc;\">\u003Cli>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Raynaud phenomenon\u003C\u002Fspan>\u003C\u002Fp>\u003C\u002Fli>\u003Cli>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Swollen fingers or hands\u003C\u002Fspan>\u003C\u002Fp>\u003C\u002Fli>\u003Cli>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Progressive skin thickening and tightening\u003C\u002Fspan>\u003C\u002Fp>\u003C\u002Fli>\u003Cli>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Reduced finger mobility\u003C\u002Fspan>\u003C\u002Fp>\u003C\u002Fli>\u003Cli>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Painful fingertip ulcers\u003C\u002Fspan>\u003C\u002Fp>\u003C\u002Fli>\u003Cli>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Joint pain or stiffness\u003C\u002Fspan>\u003C\u002Fp>\u003C\u002Fli>\u003Cli>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Acid reflux or heartburn\u003C\u002Fspan>\u003C\u002Fp>\u003C\u002Fli>\u003Cli>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Difficulty swallowing\u003C\u002Fspan>\u003C\u002Fp>\u003C\u002Fli>\u003Cli>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Bloating, diarrhea, or constipation\u003C\u002Fspan>\u003C\u002Fp>\u003C\u002Fli>\u003C\u002Ful>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Some patients also develop telangiectasias or calcium deposits beneath the skin.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Lung involvement may cause persistent dry cough, shortness of breath with activity, or reduced exercise tolerance.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Cardiac involvement may cause chest discomfort, palpitations, shortness of breath, or abnormal heart rhythms.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Scleroderma renal crisis can cause a sudden rise in blood pressure and rapid decline in kidney function and requires urgent treatment.\u003C\u002Fspan>\u003C\u002Fp>","📋",{"key":69,"title":83,"html":84,"icon":85,"updatedAt":73},"Diagnosis","\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">There is no single diagnostic test for systemic sclerosis. Diagnosis is based on the overall combination of clinical features, autoantibodies, and assessment of internal organs.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Common tests include ANA, anticentromere antibodies, anti-topoisomerase I antibodies, anti-RNA polymerase III antibodies, blood and urine testing, nailfold capillaroscopy, pulmonary function tests, high-resolution chest CT, and echocardiography.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">The 2013 ACR\u002FEULAR classification criteria remain widely used. They consider features such as skin thickening, puffy fingers, fingertip lesions, telangiectasia, abnormal nailfold capillaries, Raynaud phenomenon, interstitial lung disease or pulmonary arterial hypertension, and SSc-related autoantibodies. \u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Skin biopsy is not routinely required for diagnosis.\u003C\u002Fspan>\u003C\u002Fp>","🩺",{"key":69,"title":87,"html":88,"icon":89,"updatedAt":73},"Treatment","\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">There is no single medication that treats every aspect of systemic sclerosis. Treatment is tailored to the specific involvement of the skin, blood vessels, lungs, gastrointestinal tract, heart, and kidneys.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Current EULAR recommendations organize treatment according to major clinical domains including Raynaud phenomenon, digital ulcers, pulmonary arterial hypertension, scleroderma renal crisis, skin fibrosis, interstitial lung disease, gastrointestinal manifestations, and arthritis.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Raynaud phenomenon and digital ulcers may be managed with cold protection and vasodilator therapies.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">For SSc-associated interstitial lung disease, mycophenolate is an important treatment option. Depending on disease severity and progression, cyclophosphamide, rituximab, tocilizumab, or nintedanib may also be considered.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Pulmonary arterial hypertension requires specialist-directed treatment with pulmonary vasodilator therapies, often using combination treatment according to risk.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Gastrointestinal symptoms are managed according to the specific problem, including acid suppression for reflux and targeted treatment for motility disorders or bacterial overgrowth.\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp style=\"margin: 7px 0;\">\u003Cspan style=\"font-size: 15px;\">Scleroderma renal crisis requires urgent treatment, with ACE inhibitors playing a central role.\u003C\u002Fspan>\u003C\u002Fp>","💊",{"author":69,"reviewedBy":69,"lastReviewed":69,"datePublished":69,"citations":91,"disclaimer":69},[],false,1792294354258]