progressive systemic sclerosis

sclerosis /scle·ro·sis/ (-ro´sis) an induration or hardening, especially from inflammation and in diseases of the interstitial substance; applied chiefly to such hardening of the nervous system or to hardening of the blood vessels.

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amyotrophic lateral sclerosis  Lou Gehrig disease: progressive degeneration of the neurons that give rise to the corticospinal tract and of the motor cells of the brain stem and spinal cord, resulting in a deficit of upper and lower motor neurons; it usually has a fatal outcome within 2 to 3 years.

arterial sclerosis  arteriosclerosis.

arteriolar sclerosis  arteriolosclerosis.

diffuse cerebral sclerosis  the infantile form of metachromatic leukodystrophy.

disseminated sclerosis  multiple s.

familial centrolobar sclerosis  Pelizaeus-Merzbacher disease.

glomerular sclerosis  glomerulosclerosis.

hippocampal sclerosis  loss of neurons in the region of the hippocampus, with gliosis; sometimes seen in epilepsy.

lateral sclerosis  degeneration of the lateral columns of the spinal cord, leading to spastic paraplegia. See amyotrophic lateral sclerosis and primary lateral sclerosis

Mönckeberg's sclerosis  see under arteriosclerosis.

multiple sclerosis  (MS) demyelination occurring in patches throughout the white matter of the central nervous system, sometimes extending into the gray matter; symptoms of lesions of the white matter are weakness, incoordination, paresthesias, speech disturbances, and visual complaints.

primary lateral sclerosis  a form of motor neuron disease in which the degenerative process is limited to the corticospinal pathways.

progressive systemic sclerosis  systemic scleroderma.

tuberous sclerosis  an autosomal dominant disease characterized by hamartomas of the brain (tubers), retina, and viscera; mental retardation; seizures; and adenoma sebaceum.

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progressive systemic sclerosis

n.

A systemic disease marked by formation of hyalinized and thickened collagenous fibrous tissue, with thickening and adhesion of skin to underlying tissues, especially of the hands and face. Also called scleroderma.

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progressive systemic sclerosis (PSS).

See scleroderma.

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progressive systemic sclerosis

Sclerodema An idiopathic connective tissue disorder, characterized by localized or generalized induration of skin due to ↑ collagen deposition in skin, GI tract, lungs, heart, muscle, kidneys; PSS may be confined to skin for long periods of time, but usually encroaches on the viscera, causing malabsorption, respiratory insufficiency before death ensues, usually from heart or renal failure Clinical Raynaud's phenomenon is the initiating event in most Pts; other features include arthralgias, arthritis, early–subcutaneous edema, late–skin induration, skin ulcers, pinched face, dyspnea due to lung fibrosis, cor pulmonale Immunology Autoantibodies–eg, anti-nuclear, anti-centromere, and anti-topoisomerase 1–Scl-70 antibodies Lab Normochromic, normocytic anemia, microangiopathic anemia, polyclonal gammopathy, ↑ ESR Imaging Osteoporosis, interstitial markings in CXR, loss of esophageal and colonic peristalsis, irregular narrowing in renal arteriogram Management CCBs for vasodilation in Pts with Raynaud's phenomenon; penicillamine may slow the disease progression; preventive measures include avoiding tobacco and cold temperature, antibiotics and digitalis as needed Prognosis 5-yr survival, ± 40%. See CREST complex, Raynaud's phenomenon.