Risk factors for adult Still's disease.

OBJECTIVE: To assess risk factors for adult Still's disease (ASD). METHODS: A matched case-control study of 60 patients with ASD and 60 same sex siblings closest in age was conducted. Subjects were recruited from cohorts in Eastern Canada, Pittsburgh, and the Arthritis, Rheumatism, and Aging, Medical Information Systems (ARAMIS). A questionnaire was used to obtain data on demographic characteristics, education, income, occupation, exposure to toxic substances, stress, and medical history. RESULTS: 116 patients with ASD were identified, of which 104 participated. 86 identified same sex siblings, of which 60 replied. When compared to same sex siblings, ASD patients were similar with respect to education and occupation but had a trend to higher median income. There were no significant associations of ASD with smoking, alcohol consumption, individual toxic substances, vaccination, blood transfusion, minor or major surgery, pregnancy, or diet in the year preceding disease onset. There were no significant associations with tonsillectomy or adenoidectomy, appendectomy, asthma, hay fever, allergy shots, or pregnancy at any time preceding the onset of disease. There was a statistically nonsignificant increase in a history of exposure to coal dust [odds ratio (OR) 3.0; 95% confidence interval (CI) 0.30 to 28.84], in allergy preceding the onset of disease (OR 2.67; 95% CI 0.71 to 10.05), and in oral contraceptive use in the year preceding onset (OR 2.00; 95% CI 0.18 to 22.06). Stressful life events (OR 2.56; 95% CI 1.18 to 5.52) in the year preceding onset was significantly associated with increased risk for ASD. This positive association should be treated with caution unless confirmed by a separate study. CONCLUSION: This exploratory study of risk factors for ASD draws attention to stress as a potentially important risk factor, while likely excluding a considerable number of others.

Acute apatite podagra with negative birefringent spherulites in the synovial fluid.

We describe a young male patient who presented with acute podagra after jogging activity. He had no underlying pathology. Polarized light microscopy of the synovial fluid from his first metatarsophalangeal joint revealed numerous negative birefringent spherulites about 6 microns in diameter, presenting the typical appearance of Maltese crosses. The molar calcium/phosphorus ratio of these spherulites as determined by X-ray energy dispersive analysis was virtually identical to that of synthetic or pathologic apatite.

Adult Still's disease: manifestations, disease course, and outcome in 62 patients.

Clinical and laboratory manifestations, disease course, outcome, and HLA associations were studied in an inception cohort of 62 subjects with adult Still's disease (ASD) from 5 Canadian universities. Twenty-eight patients (45%) were female and the median age at disease onset was 24 years. In general, the clinical features observed in our patients were identical to those in other published series. However, significantly higher frequencies of sore throat (92%), weight loss (76%), lymphadenopathy (74%), pleuritis (53%), pneumonitis (27%), and abdominal pain (48%) were noted in our patients compared to those in a recent literature review. Liver involvement with hepatomegaly (44%) or abnormal liver function tests (LFTs) (76%) was common and was responsible for the 2 deaths attributed to Still's disease in our series. Severe liver failure always occurred in conjunction with aspirin or NSAID therapy. Therefore, whether or not aspirin or other NSAIDs are used, we recommend close monitoring of LFTs in patients with ASD, especially early in the disease course. Laboratory manifestations were similar to those already reported. Leukocytosis (greater than or equal to 15,000/mm3) was present in 50 patients (81%), a normochromic, normocytic anemia (hemoglobin less than or equal to 10 g/dl) in 42 (68%), and an elevated ESR in all. The mean follow-up of the 62 patients was 70 months (range, 2-163). Twenty-one patients (34%) had a self-limited disease course, 15 (24%) an intermittent course, and 22 (36%) a chronic disease course. Four patients (6%) died, and 2 of these deaths were attributed to Still's disease. For those patients who experienced a recurrence of ASD, the flares were usually of shorter duration and milder in severity than the initial episode. No initiating factor for disease exacerbation was identified in our patients. Although 22 of 62 patients (36%) had a chronic disease course, 52 (90%) were in ARA Functional Class I, and only 4 and 2 patients were in ARA Functional Class II and III, respectively. Patients with Still's disease had higher scores than the controls on the Pain (P less than 0.01) and Physical Disability (P less than 0.05) subscales of Arthritis Impact Measurement Scales health status questionnaire. Joint radiographs performed at the follow-up evaluation disclosed typical carpometacarpal and intercarpal involvement in 16 of 39 patients. In our series, HLA-B17, B18, B35, and DR2 were significantly associated with ASD. Three significant predictors of an unfavorable outcome, either a chronic disease course or a longer time to clinical remission, were identified.(ABSTRACT TRUNCATED AT 400 WORDS)

Arthritis impact measurement scales: reliability of a French version and validity in adult Still's disease.

The Arthritis Impact Measurement Scales (AIMS) questionnaire was administered to 57 patients with adult Still's disease (ASD) and 104 controls. Six of the 9 scales were consistent for both groups (Cronbach's alpha greater than or equal to 0.70). Two scales, Mobility and Dexterity, were consistent for ASD only (alpha greater than or equal to 0.70). The reliability of a French version of the AIMS was similar to the standard AIMS. Of the 13 associations evaluating criterion related validity of the AIMS in ASD, 11 were statistically significant. We conclude that the French and standard AIMS are comparable and that this questionnaire is reliable and valid for assessing outcome in ASD.

Comparison between sodium aurothiomalate and auranofin in rheumatoid arthritis. Results of a two-year open randomized study.

We compared the effectiveness of Auranofin, and sodium aurothiomalate (SATM) given for 2 years to patients with rheumatoid arthritis. This was an open trial with randomized entry to 2 treatment groups of 60 patients each. Only 40% of patients completed 2 years of treatment but these showed significant improvement in their indices of disease activity. Adverse reactions were a more common reason for withdrawal from SATM than from Auranofin, while insufficient therapeutic response was more common with Auranofin. This study also shows that inefficacy, toxicity, complicating illnesses, and lack of compliance limit the beneficial outcome of long-term chrysotherapy.

One-year comparative study of gold sodium thiomalate and auranofin in the treatment of rheumatoid arthritis.

The therapeutic benefits and toxicity profile of auranofin (AF) have been compared with that of gold sodium thiomalate (GSTM) over a one-year period in 120 patients with rheumatoid arthritis. A similar number of patients on both drugs remained in the study at one year (approximately 60%), and showed similar statistically significant improvement (p less than 0.01) in all clinical variables measured. At no time during the study was there a statistically significant difference in the clinical benefit obtained with either drug. Withdrawal from the study through lack of therapeutic benefit was twice as frequent with AF as with GSTM (11 compared to 6). Withdrawal due to a toxic reaction was twice as frequent and were potentially more serious with GSTM than with AF (15 compared to 9). We conclude that AF has a therapeutic potential similar to that of GSTM, however side effects were less prevalent and, when they did cause withdrawal of the drug, were potentially less serious.

Reactive arthritis with eosinophilic synovial infiltration.

A 17-year-old Cambodian-born female was recently evaluated for subacute seronegative arthritis involving both ankles and the right midfoot. Suboptimal response to a nonsteroidal anti-inflammatory drug prompted open synovial biopsy, which showed intensive eosinophilic infiltration of the synovial structures. Slight peripheral eosinophilia had been found before her admission to hospital, and Ankylostoma duodenale was isolated. Antiparasitic treatment offered little more relief. She was also found out to be a carrier of the histocompatibility antigen HLA B27, and it may be that despite unusual histological findings she had a B27-related reactive type of arthropathy. The relation to the parasitic infestation remains speculative.

Vasculitic neuropathy in rheumatoid disease and Sjögren syndrome.

Two patients with rheumatoid arthritis and one with Sjögren syndrome had a severe sensorimotor neuropathy preceding or up to 5 years after the onset of the disease. Electrophysiologic and sural nerve biopsy studies revealed an axonal neuropathy. Myelinated fibers were affected to a greater extent than unmyelinated axons. Peripheral nerve damage was related to occlusion of the vasa nervorum, since vasculitic involvement of epineurial vessels was observed in all patients. Despite the severity of the neuropathy, it may recover, because, compared with axons, Schwann cells are perhaps less vulnerable to ischemia.

Gold therapy in rheumatoid arthritis. Interim report of the Canadian multicenter prospective trial comparing sodium aurothiomalate and auranofin.

One hundred and twelve patients with classical or definite rheumatoid arthritis (RA) were randomly assigned to receive either sodium aurothiomalate (GSTM) or auranofin (AF). Monthly clinical assessments (morning stiffness, grip strength, articular index, pain, quality of life) and concurrent hematological, biochemical, and urine studies were performed to monitor the efficacy/toxicity (E/T) ratio. Ninety-two patients have completed 3 months; 65, 6 months; 47, 9 months; and 30, 12 months. The groups were numerically balanced at each time period. Analysis of the 0-6 month period suggests that both drugs were equally and significantly beneficial after 3 months and that this was maintained at 6 months. Toxicity was as frequent in both groups but more serious in the GSTM group. The main side effects were gastrointestinal (diarrhea) in the AF group and mucocutaneous in the GSTM group. Half of the withdrawals (14 in each group) were because of side effects in the GSTM group and for inadequate therapeutic efficacy in the AF group. This study suggests that after 6 months of treatment the E/T ratio of AF is greater than or equal to that of GSTM. These conclusions will need to be confirmed during the ongoing longer observation period. A significant clinical difference between the 2 drugs is that in a given patient treated with GSTM, the onset of toxicity coincides with a good therapeutic effect. This relationship does not appear to exist during AF treatment.

Posterior subtalar joint synoviography and corticosteroid injection in rheumatoid arthritis.

Ten posterior subtalar joints of 8 patients with rheumatoid arthritis and 12 posterior subtalar joints of 6 cadavers were studied by contrast synoviography. In the rheumatoid group the abnormalities included posterior capsule distension, filling defects caused by hypertrophic synovitis, limited or irregular filling of the anterior recess of the joint in 5 out of 10, and communication with the ankle joint in 3 out of 10. None of the cadaveric joints showed posterior capsule distension or limited or irregular filling of the anterior recess of the joint, but communication with the ankle was present in 2 joints. The joints of the patients were injected with a 1:1 mixture of sodium iothalamate 70% (Conray 420) and triamcinolone hexacetonide (Lederspan) 20 mg/ml. All patients noticed a decrease in and an improvement in walking beginning 24-48 hours after the examination. Quantitative thermography was done immediately before and 1 after injection in 2 patients who showed an improvement in thermographic index. We conclude that hindfoot inflammatory pain arising from the posterior subtalar joint is caused by distension with hypertrophic synovitis which can be difficult to detect clinically.