Nineteen cases of the rarer connective tissue disorders diagnosed between 1948 and 1972 have been reviewed. Of these 6 were of systemic lupus erythematosus (SLE), 3 of dermatomyositis and 5 each of progressive systemic sclerosis (PSS) and polyarteritis nodosa (PAN). Of the 6 cases of SLE, 4 were of true SLE, one of ethosuximide induced lupus syndrome and one newborn with placental transfer of lupus erythematosus factor. Three cases of true SLE died of renal failure. None of the patients with dermatomyositis or PSS died. Of the 5 cases of PAN, 2 died and one could not be traced. The disease is quiescent in the other two.
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Goel et al. (1974) studied this question.
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