Clinical
Raynaud syndrome
  • Idiopathic paroxysmal bilateral cyanosis of the digits due to arterial and arteriolar contraction; caused by cold or emotion. See Also: Raynaud phenomenon. Syn: symmetric asphyxia, Raynaud disease.
Arthritis (similar to rheumatoid)
Dysphagia (fibrosis esophagus)
  • Difficulty in swallowing. See Also: aglutition.
Pulmonary failure (lung fibrosis)
Malignant hypertension & renal failure
  • Malignant hypertension:  severe hypertension that runs a rapid course, causing necrosis of arteriolar walls in kidney, retina, etc.; hemorrhages occur, and death most frequently is caused by uremia or rupture of a cerebral vessel.
  • Renal Failure:  loss of renal function, either acute or chronic, that results in azothemia and syndrome of uremia.
Cardiac arrhythmia & heart failure
Diffuse scleroderma
  • The term "scleroderma" refers to a  thickening and induration of the skin caused by new collagen formation, with atrophy of pilosebaceous follicles; either a manifestation of progressive systemic sclerosis or localized (morphea). Clinically, the disease "Scleroderma" refers to progressive systemic sclerosis.
rapid progression
characteristic ANA
  • ANA:  antinuclear antibody
    • An antibody showing an affinity for nuclear antigens including DNA and found in the serum of a high proportion of patients with systemic lupus erythematosus, rheumatoid arthritis, and certain collagen diseases, in some of their healthy relatives; also in about 1% of normal individuals.
Inflammatory myopathies
polymyositis
  • Inflammation of a number of voluntary muscles simultaneously.
dermatomyositis
  • A progressive condition characterized by symmetric proximal muscular weakness with elevated serum levels of muscle enzymes and a skin rash, typically a purplish-red erythema on the face, and edema of the eyelids and periorbital tissue; affected muscle tissue shows degeneration of fibers with a chronic inflammatory reaction; occurs in children and adults, and in the latter may be associated with visceral cancer or other disorders of connective tissue.
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Fig. 5-34 Dermatomyositis. A, Rash (Heliotrope) affecting the eyelids. B, Histologic appearance of muscle shows perifascicular inflammation and atrophy. C, Inclusion body myositis shows a vacuole within a myocyte. (Courtesy of Dr. Dennis Burns, Department of Pathology, University of Texas Southwestern Medical School, Dallas.)
CREST Syndrome
  • A variant of systemic sclerosis characterized by calcinosis, Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia.
Female-male ratio 3:1
peak incidence 50 - 60
Clinical manifestations of CREST Syndrome:
  • Calcinosis
    • A condition characterized by the deposition of calcium salts in nodular foci in various tissues other than the parenchymatous viscera; the two well-known forms, calcinosis circumscripta and calcinosis universalis, are not associated with tissue damage or demonstrable metabolic disease; other forms are the result of abnormal calcium and/or phosphorous metabolism. See: metastatic calcification.
  • Raynaud syndrome
  • Idiopathic paroxysmal bilateral cyanosis of the digits due to arterial and arteriolar contraction; caused by cold or emotion. See Also: Raynaud phenomenon.Syn: symmetric asphyxia, Raynaud disease.
  • Esophageal dysmotility
  • Difficulty swallowing
  • Sclerodactyly
    • Stiffness and tightness of the skin of the fingers, with atrophy of the soft tissue and osteoporosis of the distal phalanges of the hands and feet; a limited form of progressive systemic sclerosis occurring with Raynaud phenomenon and scleroderma of the forearms. See: CREST syndrome.Syn: acroscleroderma, acrosclerosis.
  • Telangiectasia
    • A lesion formed by a dilated capillary or terminal artery, most commonly on the skin. See: telangiectasia.
Localized Scleroderma (Morphea)
  • Skin involvement only
  • No visceral involvement
  • Usually limited in distribution
  • May be widespread
  • Probably not related to progressive systemic sclerosis (PSS)
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