(Scleroderma)
In systemic sclerosis (SSc) is a rare chronic disease of unknown etiology, in the skin, joints and internal organs (especially the esophagus, lower gastrointestinal tract, lungs, heart and kidneys) by diffuse fibrosis, degenerative changes and Gefäßabnormitäten is characterized. Common symptoms include Raynaud’s syndrome, polyarthralgia, dysphagia, heartburn and swelling and eventually skin thickening and contracture of the fingers. An infestation of lung, heart and kidney is responsible for most deaths. The diagnosis is primarily clinical, but there are useful laboratory tests to confirm. Treatment is difficult, often the complications are mainly treated.
SSc affects women four times more often than men, they most often occurs in the 3rd to 5th decade of life and is rare in children. A SSc may develop as part of a MCTD.
In systemic sclerosis (SSc) is a rare chronic disease of unknown etiology, in the skin, joints and internal organs (especially the esophagus, lower gastrointestinal tract, lungs, heart and kidneys) by diffuse fibrosis, degenerative changes and Gefäßabnormitäten is characterized. Common symptoms include Raynaud’s syndrome, polyarthralgia, dysphagia, heartburn and swelling and eventually skin thickening and contracture of the fingers. An infestation of lung, heart and kidney is responsible for most deaths. The diagnosis is primarily clinical, but there are useful laboratory tests to confirm. Treatment is difficult, often the complications are mainly treated. SSc affects women four times more often than men, they most often occurs in the 3rd to 5th decade of life and is rare in children. A SSc may develop as part of a MCTD. Etiology Immunologic mechanisms and heredity (with special HLA subtypes) play an etiological role. SSc-like symptoms may be caused by exposure to vinyl chloride, bleomycin, pentazocine, epoxy compounds and aromatic hydrocarbons, contaminated rapeseed oil or l-tryptophan. Pathophysiology The pathophysiology includes vascular damage and fibroblast activation as important factors; Collagen and other extracellular proteins are produced in different tissues. In SSc skin increasingly compact form collagen fibers in the reticular dermis, it comes to thinning of the epidermis, the loss of power fixtures and atrophy of the skin appendages. It can lead to accumulation of T cells and excessive fibrosis in the dermal and subcutaneous layers. In the Nagelfalzregion the capillary loops dilate and it comes to Rarefication microvascular loops. In the extremities to chronic inflammation and fibrosis of the synovial membrane and surface and the periarticular soft tissues develop. The esophageal motility is reduced, the lower esophageal sphincter is insufficient; gastroesophageal reflux and secondary strictures can arise. The intestinal mucosa muscular degeneration, incur a pseudodiverticula in colon and ileum. Furthermore, it can lead to interstitial and peribronchial fibrosis or intimal hyperplasia of the pulmonary arteries, this results in long-lasting developing pulmonary hypertension. In the heart, a myocardial fibrosis and conduction abnormalities may develop. In the kidney, intimal hyperplasia of interlobar and arch arteries leading to renal ischemia and hypertension. In general, the SSc varies in severity and its progression very strong, which range from a generalized skin thickening with rapidly progressive and often fatal organ involvement (SSc with diffuse scleroderma) to an isolated skin involvement (often just the fingers and face on) and very slow progression (often over several decades), to the organ involvement. The latter form is limited cutaneous scleroderma or CREST syndrome called (calcinosis cutis, Raynaud’s phenomenon, Ösophagusmotilitätsstörung, Sclerodactyly, telangiectasia). Moreover, SSc can show overlapping symptoms with other rheumatic autoimmune diseases such. B. with the MCTD and the Sklerodermatomyositis (characterized by hard skin and muscle weakness which is the classical polymyositis indistinguishable). Symptoms and signs Most often the disease begins with Raynaud’s syndrome and gradual swelling of the limbs and thickening of the skin on the fingers, often with polyarthralgia. Occasionally, gastrointestinal or respiratory symptoms such as heartburn and dysphagia or dyspnea the Erstbefund represents. Skin and nail manifestations The skin swelling occurs symmetrically on and proceeds to the induration. You may be limited or virtually affect the whole body on the fingers (Sclerodactyly) and hands. The skin finally gets a taut consistency and becomes translucent and hypo- or hyperpigmented. The face is mask-like, nodular type of fingers, chest, face, lips and tongue. Subcutaneous calcifications most often develop on the fingertips and over bony prominences. Digital ulcers are often v. a. at the fingertips and on the finger joints and node calcified. abnormal capillary and microvascular loops in the nail bed can be Kapillarmikroskopisch recognize. Systemic sclerosis hand with permission of the publisher. From Pandya A .: Gastroenterology and Hepatology: Stomach and duodenum. Edited by M. Feldman. Philadelphia, Current Medicine, 1996. var model = {thumbnailUrl: ‘/-/media/manual/professional/images/systemic_sclerosis_hand_high_de.jpg?la=de&thn=0&mw=350’ imageUrl: ‘/ – / media / manual / professional / images / systemic_sclerosis_hand_high_de.jpg lang = en & thn = 0 ‘, title:’? Systemic sclerosis hand ‘, description:’ u003Ca id = “v37892782 ” class = “”anchor “” u003e u003c / a u003e u003cdiv class = “”para “” u003e u003cp u003eIn this image can be seen as a result of surgery (so-called sclerodactyly) of a glossy and thickened skin with disappearance of normal skin drawing. u003c / p u003e u003c / div u003e ‘credits’ with permission of the publisher. From Pandya A .: Gastroenterology and Hepatology: Stomach and duodenum. Edited by M. Feldman. Philadelphia