Systemic Sclerosis: Clinical Manifestations

Jelena      Blagojevic; Marco      Matucci-Cerinic

doi:10.2174/157339710793205675

Abstract

Systemic sclerosis (SSc) is a severe autoimmune disease that involves skin and internal organs. Clinical manifestations of SSc are very heterogeneous and derive from microvascular, inflammatory and fibrotic tissue changes. Besides the skin, lung, gastrointestinal tract, heart and kidneys are mainly affected. Over the last years, a progress in the treatment was made, especially in the field of renal involvement and pulmonary hypertension, but prognosis of the disease remains still bad. More efforts are needed to improve the management of organ impairment by early detection and prompt treatment, in order to prevent the development of severe complications.

Keywords: Systemic sclerosis, interstitial lung disease, autoantibodies, vasculopathy, autoimmune disease, pulmonary hypertension, limited cutaneous (lcSSc), Raynaud's phenomenon (RP), pulmonary arterial hypertension (PAH), Anti-topo I, Anti-centromere antibodies (ACA), Autoantibodies to RNA polymerase (RNAP) I, Sclerodactyly, Calcinosis cutis, Telangiectasias, anti-polymyositis/Scl, anti-endothelial cells, anti-cardiolipin antibodies, digital ulcers (DU), ischemia, Repetitive microtrauma, gangrene, amputation, endothelin receptor antagonist bosentan, phosphodiesterase-5 inhibitor, intractable osteomyelitis, Calcinosis, scleroderma renal crisis, angiotensin-converting enzyme (ACE), mild proteinuria, haema-turia, granular castes, tendon friction rubs, pericardial effusion, anaemia, cardiac insufficiency, ACE inhibitor, Haemodialysis, Acute glomerulonephritis, Glomerular filtration, Renal biopsy, GASTRO-INTESTINAL INVOLVEMENT, oesophageal sphincter, achalasia-like syndrome, gastroesophageal reflux, Barrett's oesophagus, heartburn, nausea, dysphagia, motility dysfunction, Gastric antral vascular ectasia (GAVE), atrophic gastritis, achlorhydria, vitamin B12 deficiency, luminal dilatation, malabsorption, Diarrhoea, pancreatic insufficiency, Anti-diarrhoeal agents, GIT Score, orthopnoea, palpitations syncope, atherosclerotic coronary artery, Myocardial fibrosis, Myocarditis, anti-topoisomerase antibodies, digital ulcerations, interstitial lung disease (ILD), nonspecific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), High resolution lung computer tomography (HRCT), Bronchoalveolar lavage (BAL), cyclophosphamide, pulmonary veno-occlu-sive disease, thoracic discomfort, Electrocardiography (ECG), DLCO/VA, calcium channel blockers (CCBs), Epoprostenol, haemodynamics, Inflammatory tenosynovitis, Distal phalangeal resorption (acro-osteolysis), COXIBs, NSAIDS, gastric mucosa, Neuropsychiatric disorders, optic neuropathy, psychosis, anxiety disorder, SSc patients