Digital artery thrombosis and vasculitis in juvenile rheumatoid arthritis

A case of juvenile rheumatoid arthritis with vasculitis is presented. Sixteen months after the onset of the disease the patient developed digital artery thrombosis with incipient gangrene. Both the latter and the skin lesions resolved during treatment with azathioprine.     

Sulphasalazine in rheumatoid arthritis.

Seventy-four patients with rheumatoid arthritis were treated with sulphasalazine. There was a significant improvement in clinical score, with substantial falls in serum C-reactive protein concentrations and erythrocyte sedimentation rate four weeks after starting the drug. Improvement was maintained in the 38 patients who remained on the durg for one year. The mean Rose-Waaler titre did not change. There was little difference between the results in seropositive and seronegative patients. The commonest adverse effect was dyspepsia, but five patients developed a megaloblastic anaemia and one patient neutropenia; all made a complete recovery. The results suggest that the drug has a disease-modifying action not attributable to its "salicylate" content. The mode of action might be by an antibacterial effect on gut flora.     

HLA-linked rheumatoid arthritis.

Twenty-eight pedigrees were ascertained through pairs of first-degree relatives diagnosed with rheumatoid arthritis (RA). RA was confirmed in 77 pedigree members including probands; the absence of disease was verified in an additional 261 pedigree members. Pedigree members were serologically typed for HLA. We used likelihood analysis to statistically characterize the HLA-linked RA susceptibility locus. The genetic model assumed tight linkage to HLA. The analysis supported the existence of an HLA-linked RA susceptibility locus, estimated the susceptibility allele frequency as 2.16%, and estimated the lifetime penetrance as 41% in male homozygotes and as 48% in female homozygotes. Inheritance was recessive in males and was nearly recessive in females. In addition, the analysis attributed 78% of the variance within genotypes to genetic or environmental effects shared by siblings. The genetic model inferred in this analysis is consistent with previous association, linkage, and familial aggregation studies of RA. The inferred HLA-linked RA susceptibility locus accounts for approximately one-half of familial RA, although it accounts for only approximately one-fifth of the RA in the population. Although other genes may account for the remaining familial RA, a large portion of RA cases may occur sporadically.     

Fungal arthritis simulating juvenile rheumatoid arthritis.

Petriellidium boydii is often isolated from maduromycosis but has recently been associated with arthritis. A previously healthy 6-year-old boy developed chronic purulent arthritis of the knee after a bicycle accident. Culture of aspirate grew no pathogens and antibiotic treatment had no effect. Culture of synovial fluid grew P boydii, which responded initially to amphotericin but reappeared after six months. Subsequent treatment with miconazole was stopped after development of haematuria. The fungus was sensitive to ketoconazole, and treatment with this drug cured the infection. With the introduction of ketoconazole it is of practical importance to recognise fungal infections.     

Ulnar artery thrombosis.

Forty-six patients with ulnar artery thrombosis were evaluated. A comparison of the results was made between those patients who were treated by sympathectomy and those in whom we attempted to reestablish the blood flow. A reverse vein graft was often used in the latter group. Based on the results of this study, we believe the preferred method of treatment for ulnar artery thrombosis is an attempt at the reestablishment of blood flow.     

Autonomic neuropathy in rheumatoid arthritis.

Patients with seropositive and seronegative rheumatoid arthritis (RA) and age-matched controls were investigated for the presence of autonomic neuropathy. Significantly more patients with RA had abnormal autonomic function, suggesting that autonomic neuropathy occurs more commonly in RA than hitherto suspected. The existence of an autonomic neuropathy may be an important complicating factor in rheumatoid disease and may lead to increased morbidity and mortality.     

Retinal vasculitis in rheumatoid arthritis.

A woman with exacerbation of severe rheumatoid arthritis developed lesions compatible with retinal vasculitis. Laboratory studies confirmed the diagnosis, and the rapid clinical improvement that accompanied a fall in circulating immune complexes suggested that the vasculitis was a direct consequence of the rheumatoid disease. From these observations retinal vasculitis should probably be sought in any patient with rheumatoid disease and the vasculitis added to the list of ocular complications of rheumatoid arthritis.     

Remission-inducing drugs in rheumatoid arthritis.

The administration of certain drugs to patients with established rheumatoid arthritis frequently results in improvement that is slow to appear but persists for long periods, even after the drug is discontinued. The three main drugs with this effect, whose efficacy and toxicity are reviewed in this paper, are gold salts, D-penicillamine and chloroquine. The cytotoxic agents used to treat rheumatoid arthritis, which likely have nonspecific anti-inflammatory actions and have serious long-term side effects, are also briefly reviewed. A new drug, levamisole, is currently being tested in patients with rheumatoid arthritis. It is suggested that the time for considering the introduction of a remission-inducing drug in patients with progressive rheumatoid arthritis is after an adequate trial of therapy with salicylates or other nonsteroidal anti-inflammatory agents, or both, and before the oral administration of steroids. It is difficult, however, on the basis of rigorous clinical comparisons, to recommend which of the three main remission-inducing drugs should be tried first, although gold salts have been used the most. Patients who have improved with 6 months of chrysotherapy may continue treatment for at least 3 years, during which time the frequency of mucocutaneous and renal toxic effects will steadily decrease. Some aspects of the medical economics of therapy with remission-inducing drugs for rheumatoid arthritis are discussed.     

Anti-keratin antibodies in rheumatoid arthritis.

A naturally occurring antibody that reacts with the keratinised tissue of animal oesophagus was found in the serum of 75 out of 129 patients (58%) with classical or definite rheumatoid arthritis (RA) but not in sera from 105 healthy people. Detection of the antibody, which is unrelated to rheumatoid factor, is more specific for RA than the reaction in the sheep-cell agglutination test but less sensitive.