Mixed Connective Tissue Disease in Childhood

Abstract

Mixed connective tissue disease (MCTD) seems to be a distinct entity that has some manifestations of systemic lupus erythematosus, scleroderma, polymyositis, and Sjögren's syndrome and is serologically characterized by the presence of an antibody to ribonucleoprotein. We report the cases of three children with MCTD with high titers of antibody to ribonucleoprotein. Two fulfilled criteria of lupus erythematosus, two had polymyosis; all three had suggestive features of scleroderma, fulfilled criteria for the diagnosis of juvenile rheumatoid arthritis, and had Sjögren's syndrome. Additional superimposed features of another connective tissue disease should arouse suspicion of MCTD. All three of our patients responded adequately to corticosteroid treatment that makes recognition of this entity by the pediatrician all the more important.

Clinical features and outcome of mixed connective tissue disease in developmental age – observational study from one center

“…The disease was described for the first time in 1972. Soon, there were reports on the infantile form of MCTD [2,3]. Mixed connective tissue disease is characterized by concomitant occurrence of clinical symptoms typical of systemic lupus erythematosus (SLE), systemic sclerosis (SSc), dermatomyositis (DM) and sometimes also juvenile idiopathic arthritis (JIA), without meeting clear diagnostic criteria for those diseases.…”

Η Δερματο - Πολυμυοσιτις Κατα Την Παιδικην Ηλικιαν

“…Το 1972 ό Sharp 147 εις ΗΠΑ περιέγραψε μίαν σειράν ασθενών έμφανιζόντων κοινάς εκδηλώσεις δερματομυοσίτιδος, σκληροδέρματος και συστη ματικού έρυθηματώδους λύκου μέ ύψηλον τίτλον αντισωμάτων ()1: 1.000) ε ναντίον αντιγόνου τίνος εξ εκχυλίσματος πυρήνων εμβρυϊκού βοείου θύμου αδένος (extraxtable nuclear antigen ή ΕΝΑ), το όποιον είναι ευαίσθητον εις τήν έπίδρασιν του ενζύμου ριβονουκλεάση (RNase sensitive).Έκτοτε, άρκεταί περιπτώσεις έχουν άνακοινωθή οχι μόνον εις ενήλικας, άλλα και εις παιδία 53 , γνωρίζομεν δέ δτι τό ως άνω άντιγόνον είναι ή πυρηνική ριβοπυρηνοπρωτεΐνη (RNP). Τα άτομα αυτά έχουν συνήθως καλοήθη πρόγνωσιν, δέν εμφανίζουν σπειραματονεφρίτιδα, απαντούν δέ εύνοϊκώς εις τήν χορήγησιν γλυκοκορτικοειδών.…”