Approach to a Patient with Connective Tissue Disease.
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Date
2010-10
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Abstract
Connective tissue disease (CTDs), though rare in
childhood, are an important cause of morbidity. Most of
them involve multiple organ systems and are associated
with presence of autoantibodies. Systemic lupus eryethematosus
(SLE) is the most common CTD, the others being
Juvenile dermatomyositis, systemic sclerosis, mixed connective
disease and Sjogren syndrome. The clinical presentation
of CTD in childhood can range from an acute severe
illness mimicking a serious infection, to an insidious onset
of disease with gradual accumulation of symptoms and
signs over wks to months. The presence of multi-system
involvement, evidence of inflammation and lack of any
obvious cause should alert a clinician to the possibility of
CTD. Diagnosis is usually clinical and features like malar
rash, Raynaud’s phenomenon, Gottron’s rash, photosensitivity,
oral ulcers suggest a possibility of CTD. Presence of
autoantibodies like anti-nuclear antibodies, anti-dsDNA etc.
provide supportive evidence to a diagnosis of CTD. Most
CTDs are treated with immunosuppressive drugs with good
success. Early recognition and prompt treatment results in
excellent outcome.
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Keywords
Autoantibodies, Autoimmune diseases, Connective tissue disease, Systemic lupus erythematosus
Citation
Sathish Kumar T, Aggarwal Amita. Approach to a Patient with Connective Tissue Disease. Indian Journal of Pediatrics. 2010 Oct; 77 (10): 1157-1164.