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Sjögren's syndrome in patients with the CREST variant of progressive systemic scleroderma. 1991

A A Drosos, and Y L Pennec, and M Elisaf, and A Lamour, and N C Acritidis, and J R Jouquan, and H M Moutsopoulos, and P Youinou
Department of Internal Medicine, School of Medicine, Universita of Ioannina, Greece.

Twenty-three patients with the CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) variant of progressive systemic sclerosis, were clinically, histopathologically and serologically examined for the presence of Sjögren's syndrome (SS). Fourteen were found to be positive. No significant difference could be demonstrated between them and the remaining 9. Characteristics of patients with CREST were compared with those of 29 randomly chosen patients with primary SS. Parotid gland enlargement was more frequently present (p less than 0.01) in the latter than in the former. Virtually no patients with CREST with SS had antibodies to Ro(SSA)/La(SSB).

UI MeSH Term Description Entries
D011928 Raynaud Disease An idiopathic vascular disorder characterized by bilateral Raynaud phenomenon, the abrupt onset of digital paleness or CYANOSIS in response to cold exposure or stress. Cold Fingers, Hereditary,Raynaud Phenomenon,Raynaud's Disease,Raynauds Disease
D002114 Calcinosis Pathologic deposition of calcium salts in tissues. Calcification, Pathologic,Calcinosis, Tumoral,Microcalcification,Microcalcinosis,Pathologic Calcification,Calcinoses,Calcinoses, Tumoral,Microcalcifications,Microcalcinoses,Tumoral Calcinoses,Tumoral Calcinosis
D005121 Extremities The farthest or outermost projections of the body, such as the HAND and FOOT. Limbs,Extremity,Limb
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001323 Autoantibodies Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them. Autoantibody
D012594 Scleroderma, Localized A term used to describe a variety of localized asymmetrical SKIN thickening that is similar to those of SYSTEMIC SCLERODERMA but without the disease features in the multiple internal organs and BLOOD VESSELS. Lesions may be characterized as patches or plaques (morphea), bands (linear), or nodules. Dermatosclerosis,Morphea,Scleroderma, Circumscribed,Frontal Linear Scleroderma en Coup de Sabre,Linear Scleroderma,Scleroderma, Linear,Circumscribed Scleroderma,Localized Scleroderma,Morpheas,Sclerodermas, Localized
D012595 Scleroderma, Systemic A chronic multi-system disorder of CONNECTIVE TISSUE. It is characterized by SCLEROSIS in the SKIN, the LUNGS, the HEART, the GASTROINTESTINAL TRACT, the KIDNEYS, and the MUSCULOSKELETAL SYSTEM. Other important features include diseased small BLOOD VESSELS and AUTOANTIBODIES. The disorder is named for its most prominent feature (hard skin), and classified into subsets by the extent of skin thickening: LIMITED SCLERODERMA and DIFFUSE SCLERODERMA. Sclerosis, Systemic,Systemic Scleroderma,Systemic Sclerosis
D012859 Sjogren's Syndrome Chronic inflammatory and autoimmune disease in which the salivary and lacrimal glands undergo progressive destruction by lymphocytes and plasma cells resulting in decreased production of saliva and tears. The primary form, often called sicca syndrome, involves both KERATOCONJUNCTIVITIS SICCA and XEROSTOMIA. The secondary form includes, in addition, the presence of a connective tissue disease, usually rheumatoid arthritis. Sicca Syndrome,Sjogren Syndrome,Sjogrens Syndrome,Syndrome, Sicca,Syndrome, Sjogren's
D012871 Skin Diseases Diseases involving the DERMIS or EPIDERMIS. Dermatoses,Skin and Subcutaneous Tissue Disorders,Dermatosis,Skin Disease
D013577 Syndrome A characteristic symptom complex. Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes

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