systemic scleroderma
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This document discusses systemic scleroderma, a chronic connective tissue disorder characterized by skin thickening and internal organ involvement. It causes skin thickening due to excess collagen deposition and damage to blood vessels. Genetic, immunological, and environmental factors may play a role. There are two main types: limited, affecting only the skin, and diffuse, also affecting internal organs. Symptoms vary but commonly include skin tightening, Raynaud's phenomenon, and involvement of the lungs, heart, kidneys, and gastrointestinal system. Diagnosis involves clinical exam, antibody testing, and imaging. Treatment focuses on lifestyle changes, medications to improve symptoms, and immunosuppressants in severe cases.
systemic scleroderma
- 1. DANYLO HALYTSKY NATIONAL MEDICAL UNIVERSITY. DEPARTMENT: FAMILY MEDICINE. NAME: NWACHUKWU LINDA. GROUP: 3 TOPIC: SYSTEMIC SCLERODERMA.
- 2. Definition: it is a chronic multisystem connective tissue disorder characterized by: • Skin thickening caused by accumulation of connective tissue. • Visceral organ involvement. Pathogenesis: The CD4 cells(lymphocytes) reacted against some antigens in her body resulting to accumulation of the lymphocytes and thus cytokines are released causing vascular cell injury. These damaged endothelium produces less vasodilators and more vasoconstrictors which causes decrease oxygen supply and resulting in tissue hypoxia. Also tissue growth factor is released by yhe damaged tissue thus causing activation of fibroblast resulting in the fibroblast to start producing collagen and extracellular proteins in excess. Etiology: Genetic/hereditary Immunology Chemical radiation exposure Idiopathic.
- 3. Classification: • Localized scleroderma (localized cutaneous fibrosis): which can be morphea or linea. • Systemic scleroderma (cutaneous and non cutaneous): which can either be diffuse, limited or sine i.e. without skin thickening. Here, skin and organs are affected. Clinical manifestation: • Localized: two forms are morphea, which occurs as single or multiple plaques of skin indurations, and linear scleroderma, which involves an extremity or face. Linear scleroderma of one side of the forehead and scalp produces a disfiguration referred to as en coup de sabre because it resembles a wound from a sword. It may be associated with hemi atrophy of the same side of the face. NOTE: systemic scleroderma also occurs in association with features of other connective tissue diseases. The term overlap syndrome has been used to describe such patients. Undifferentiated connective tissue disease has been suggested as a designation for patients who do not have diagnostic criteria for any one connective tissue disease. Main clinical manifestations of scleroderma are: Generalized morphea, Diffuse oedema of hands, Firm thickened skin, Flexion contractures of fingers, Reynaud's phenomenon (pallor phase).
- 4. Systemic: • diffuse Cutaneous Systemic Sclerosis · Onset of Reynaud's within 1 year of onset of skin changes (puffy or hidebound) · Truncal and a crest involvement · Presence of tendon friction rubs · Early and significant incidence of interstitial lung disease, oliguric renal failure, diffuse gastrointestinal disease, and myocardial involvement · Absence of anti centromere antibodies · Nail fold capillary dilation and capillary destruction · Anti topoisomerase antibodies (30% of patients) • Limited Cutaneous Systemic Sclerosis · Reynaud's phenomenon for years (occasionally decades) · Skin involvement limited to hands, face, feet, and forearms · A significant late incidence of pulmonary hypertension, with or without interstitial lung disease, trigeminal neuralgia, skin calcifications, telangiectasias · A high incidence of anticentromere antibodies (70%-80%) · Dilated nail fold capillary loops, usually without capillary dropout 1. skin: swollen fingers, Reynaud phenomenon, generalized morphea, firm thickened skin, mask face, telangiectasis, epidermal thinning etc 2. GIT: pneumatosis intestinalis, dysphagia , oesophageal strictures, barret oesophagus, decreased peristalsis, post prandial bloating, collagen hypo motility etc 3. heart: collagen myocardial fibrosis, pericarditis, pericardial effusion, conduction abnormalities etc 4. lungs: interstial fibrosis, intimal hyperplasia, pulmonary hypertension, pulmonary alveolitis etc 5. muscle: myopathy, arthralgias, arthritis, turner carpal syndrome etc. 6. Renal: scleroderma renal crisis, malignant hypertension, fibrinoid necrosis, hyperplasic arteriosclerosis,
- 5. CRITERIA • Major criterion: • Proximal scleroderma: symmetric thickening, tightening, and indurations of the skin of the fingers and/or the skin proximal to the metacarpophalangeal or metatarsophalangeal joints. The changes may affect the entire extremity, face, neck, and trunk (thorax and abdomen). • Minor criteria: • 1. Sclerodactyly: preceding skin changes limited to the finger. • 2. Digital pitting scars or loss of substance from the finger pad: depressed areas at tips of fingers or loss of digital pad tissue as a result of ischemia. • 3. Bibasilar pulmonary fibrosis: bilateral reticular pattern of linear or line nodular densities most pronounced in basilar portions of the lungs on standard chest roentgenogram; may assume appearance of diffuse mottling or “honeycomb lung”. These changes should not be attributable to primary lung disease. POSITIVE: major criteria + at least two of the minor criteria.
- 6. DIAGNOSIS • Laboratory: Cbc: may be increased ESR, decreased hemoglobin, thrombocytopenia. Biochemical: may be increased creatine phosphokinase, increased c-reactive protein, hypergammaglobulinemia. Antibodies specific for scleroderma: • Nucleolar autoantibodies • Antibodies to scl-70 • Anticetromere antibodies(limitted) • Antitopoisomerase(diffuse) • Can be antibodies agaist type1 and 4 collagen. • Instrumental: Chest x-ray Bone radiography Oesophageal scintigraphy Doppler echocardiography Spirometry Capillary microscopy.
- 7. TREATMENT • Basic therapy: 1. Diet : eat foods with less fat and carbohydrate. Eat less spicy food. Eat more vegetables and fruits. Stand for some minutes after eating. 2. Avoid cold exposure. Stay in a warm place. 3. Avoid alcohol intake or smoking. Healthy life style. 4. Bed rest and massage. • Medication: 1. Nifedipine/ iv prostanoids (iloprost) : 0.5-2mg/kg/minutes for 5days Or if diffuse with multiple digital ulcers, Bosentan 20,40, 80 mg/3times daily orally. for patients with skin or raynod phenomenon. 2. cyclophosphamide: 1-2mg/kg/day orally ( lung) 3. Captopril/enalapril; 20mg/3times a day orally (renal) 4. omeprazole: 10mg thrice a day orally (GIT) 5. Acetaminophen: 100md/day orally (muscle pain)