systemic sclerosis


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Related to systemic sclerosis: progressive systemic sclerosis, CREST syndrome

sclerosis

 [sklĕ-ro´sis]
an induration or hardening, especially of a part from inflammation, or in disease of the interstitial substance. The term is used chiefly for such a hardening of the nervous system due to hyperplasia of the connective tissue or for hardening of the blood vessels. Called also induration. adj., adj sclerot´ic.
amyotrophic lateral sclerosis see amyotrophic lateral sclerosis.
arteriolar sclerosis arteriolosclerosis.
disseminated sclerosis multiple sclerosis.
familial centrolobar sclerosis a progressive familial form of leukoencephalopathy marked by nystagmus, ataxia, tremor, parkinsonian facies, dysarthria, and mental deterioration.
focal glomerular sclerosis focal segmental glomerulosclerosis.
glomerular sclerosis glomerulosclerosis.
hippocampal sclerosis loss of neurons in the region of the hippocampus, with gliosis; sometimes seen in epilepsy.
lateral sclerosis a form seated in the lateral columns of the spinal cord. It may be primary, with spastic paraplegia, rigidity of the limbs, and increase of the tendon reflexes but no sensory disturbances, or secondary to myelitis, with paraplegia and sensory disturbance.
medial calcific sclerosis (Mönckeberg's sclerosis) Mönckeberg's arteriosclerosis.
multiple sclerosis see multiple sclerosis.
systemic sclerosis systemic scleroderma.
tuberous sclerosis a congenital heredofamilial disease, transmitted as an autosomal dominant trait, characterized principally by the presence of hamartomas of the brain (tubers), retina (phakomas), and viscera, mental retardation, seizures, and adenoma sebaceum, and often associated with other skin lesions.

sys·tem·ic scle·ro·sis

1. a systemic disease characterized by formation of hyalinized and thickened collagenous fibrous tissue, with thickening of the skin and adhesion to underlying tissues (especially of the hands and face), dysphagia due to loss of peristalsis and submucosal fibrosis of the esophagus, dyspnea due to pulmonary fibrosis, myocardial fibrosis, and renal vascular changes resembling those of malignant hypertension; Raynaud phenomenon, atrophy of the soft tissues, and osteoporosis of the distal phalanges (acrosclerosis), sometimes with gangrene at the ends of the digits, are common findings. The term progressive systemic sclerosis is commonly used and is appropriate for cases with initially widespread skin involvement including the trunk. However, when skin involvement is limited to the distal extremities and face, there is often prolonged delay in appearance of visceral manifestations.
See also: CREST syndrome.
2. Synonym(s): scleroderma

systemic sclerosis

Scleroderma Rheumatology An idiopathic connective tissue disease with clinical and pathologic features of GVHD, characterized by subcutaneous and visceral fibrosis, anticentromere and antitopoisomerase antibodies and prominent microvascular changes with endothelial cell damage and proliferation of subendothelial connective tissue; ♀:♂ ratio, 3-8:1-after child-bearing yrs Peak Age 45 to 55. See Connective tissue disease, Graft-versus-host disease.

systemic sclerosis

A generalized CONNECTIVE TISSUE disorder featuring FIBROSIS and degeneration of the skin and the internal organs. There is severe RAYNAUD'S PHENOMENON, swelling of the fingers, pigmentation, shiny atrophy and tightening of the skin so that the face becomes mask-like and there is difficulty in opening the mouth. There is no effective treatment.

Systemic sclerosis

A rare disorder that causes thickening and scarring of multiple organ systems.
Mentioned in: Scleroderma
References in periodicals archive ?
Table 1 Cutaneous findings, systemic features and laboratory parameters in Systemic Sclerosis (n=54)
Furst said that the purpose of the Combined Response Index for Systemic Sclerosis (CRISS) is to assess whether new pharmacologic agents have an impact on overall disease activity/severity in studies of diffuse cutaneous systemic sclerosis.
Systemic sclerosis can involve almost all structures of cardiovascular system like conduction pathway, heart valves, endocardium, myocardium, pericardium, coronary arteries and pulmonary arteries.
Fleischmajer et al., "Scleroderma (systemic sclerosis): classification, subsets and pathogenesis," The Journal of Rheumatology, vol.
Zhou, "Systemic sclerosis is a complex disease associated mainly with immune regulatory and inflammatory genes," The Open Rheumatology Journal, vol.
Baron, "Special issue on rheumatology targeted therapy in systemic sclerosis," Rambam Maimonides Medical Journal, vol.
Zhang et al., "Nintedanib inhibits macrophage activation and ameliorates vascular and fibrotic manifestations in the Fra2 mouse model of systemic sclerosis," Annals of the Rheumatic Diseases, vol.
Fiorentino, "Characterization of patients with clinical overlap of morphea and systemic sclerosis: A case series," Journal of the American Academy of Dermatology, vol.
Serum nitric oxide (NO) levels in systemic sclerosis patients: correlation between NO levels and clinical features.
There were more than 90% of systemic sclerosis (SSc) patients developing gastrointestinal tract involvement with affecting esophagus mostly.