Systemic sclerosis(SSc): Symptoms & Treatments

Systemic sclerosis is an invasion of internal organs, and can be divided into diffuse and limited. The cause is not known yet, and it is said to occur in middle-aged women.

Symptoms
Limited SSc has a long period of time from the appearance of Raynaud phenomenon to the appearance of scleroderma, and pulmonary HTN is the predominant systemic symptom. The associated autoantibody is known as anti-centromere and the 10-year survival rate is 75% higher than diffuse SSc. Diffuse SSc is characterized by rapid progression of the distal to proximal sclerosis and occurs simultaneously with the Raynaud phenomenon. Interstitial lung disease and kidney disease cause systemic symptoms and related autoantibody is anti-topoisomerase. The 10-year survival rate is 55%.
Raynaud phenomenon is a typical clinical phenomenon, which is caused by seizure vasoconstriction induced by external stimuli such as cold, stress, and vibration. It is also a characteristic symptom of SSc in which symmetrical skin hardening is distinguished from other connective tissue diseases. Progressing from distal to proximal, loss of hair follicles, sweat glands and sebaceous glands. Pulmonary symptoms occur in most patients and are currently the most important cause of death. Scleroderma renal crisis causes renal vasoconstriction leading to RAAS hyperactivity, resulting in malignant HTN, oliguria, MAHA, and proteinuria.

Treatments
As with other rheumatic diseases, curing is difficult, and slowing the progression of the disease and controlling symptoms are the main goals of therapy. To treat the Raynaud phenomenon, warm clothing, avoiding exposure to cold, and the use of DHP-based CCB for pharmacological treatment. BB, amphetamine, and ergotamine are contraindications. Use D-penicillamine to slow down Fibrosis. ACEi can be used to treat Scleroderma renal crisis, and cyclophosphamide is used to slow the progression of the disease.

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