Extensive vascular calcification in association with juvenile rheumatoid arthritis and amyloidosis.

Amyloidosis is an uncommon complication of juvenile rheumatoid arthritis.Forsyth (1960) described such a case, where, in additon, there was small vessel calcification.The following report of a similar case is of interest in view of the extensive arterial calcification, amyloidosis, and arteritis seen at necropsy.Case History A.D. was a normal boy until the age of 21 years when he was admitted to hospital with a history of one week's anorexia, pyrexia, difficulty in walking, and generalized pains.There was a rash over the abdomen and pain in his hip and knee joints.The only laboratory findings of note were a white cell count of 23,000 (polymorphs 75%) and Hb 9-6 g./100 ml.He was treated with penicillin and streptomycin, made a good recovery, and was discharged after one month in hospital.When aged 21 years the fever and generalized pains recurred and he was again admitted to hospital.He was thin with painful swellings of both wrists, and reduced cervical spine movement; and had splenomegaly, generalized lymphadenopathy, and a faint erythematous rash over the trunk and hands.Temperature 40. 5 C. ESR 58 mm./1 hr.; Hb 8-9 g./100 ml.; WBC 48,000, with 86% polymorphs; serum protein 6-6%, albumin 2-9%, globulin 3-7%; urine, albumin+.X-ray of wrist showed slight decalcification.He was given soluble aspirin for one month, but then relapsed and was treated with prednisone, 20 mg./day, with marked improvement, and he was eventually discharged when aged 3j years on no treatment.A month later there was a recurrence of the arthralgia, rash, and pyrexia.Again there was a poor response to salicylate treatment, and gold therapy was started, but after a single injection of 1 mg.soluble aurothiomalate he became jaundiced: serum alkaline phosphatase was then 106 KA units, SGOT 160 units, and SGPT 175 units.No further gold was given and prednisone was restarted.Aged 4 years 1 month he was discharged on 5 mg.prednisone twice weekly, being fully mobile and having no joint pains.He remained well, and steroid therapy was stopped a year later.