[Lymphocytic interstitial pneumonia in Gougerot-Sjögren syndrome with scleroderma]. 1984

P Allard, and J Kermarec, and P L'Her, and F Natali, and M Lebarbu, and R Le Vagueresse

The authors report the case of a 70 year old woman with a 15 year history of the Gougerot-Sjogren syndrome, Raynaud's phenomenon and sclerodactyly. Progressive respiratory symptoms in this case were not due to pulmonary fibrosis secondary to scleroderma but to diffuse interstitial lymphocytic pneumonia (ILP) secondary to the Gougerot-Sjogren syndrome. The authors emphasize the diagnostic value of the significant, permanent lymphocytosis in the aspirate after broncho-alveolar lavage. The differential diagnosis is difficult because the radiological and clinical signs of pulmonary complications of the Gougerot-Sjogren syndrome and of scleroderma are very similar. Reports of the triple association of ILP-Gougerot-Sjogren syndrome-scleroderma are uncommon in the medical literature. However, prospective studies suggest that paraclinical stigmata of the Sjogren syndrome are relatively common in patients with scleroderma. These observations suggest that some patients with clinical "pulmonary fibrosis" may in fact have ILP secondary to slowly progressive Sjogren's syndrome.

UI MeSH Term Description Entries
D008218 Lymphocytosis Excess of normal lymphocytes in the blood or in any effusion. Lymphocytoses
D011658 Pulmonary Fibrosis A process in which normal lung tissues are progressively replaced by FIBROBLASTS and COLLAGEN causing an irreversible loss of the ability to transfer oxygen into the bloodstream via PULMONARY ALVEOLI. Patients show progressive DYSPNEA finally resulting in death. Alveolitis, Fibrosing,Idiopathic Diffuse Interstitial Pulmonary Fibrosis,Fibroses, Pulmonary,Fibrosis, Pulmonary,Pulmonary Fibroses,Alveolitides, Fibrosing,Fibrosing Alveolitides,Fibrosing Alveolitis
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
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
D013997 Time Factors Elements of limited time intervals, contributing to particular results or situations. Time Series,Factor, Time,Time Factor

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