A baseline skin score > or =20 was associated with heart involvement at baseline and predicted mortality and SRC over the subsequent 4 years. Improvement in skin score in these patients with diffuse cutaneous scleroderma was associated with improvement in hand function, inflammatory indices, joint contractures, arthritis signs, overall functional ability, and the examining investigator's global assessment of improvement.

The course of the skin score and the frequencies of SRC and mortality in the high-dose D-Pen group were not different from those in the low-dose D-Pen group. Eighty percent of the adverse event-related withdrawals occurred in the high-dose D-Pen patients. Although this study cannot answer the question of whether low-dose D-Pen is effective, it does suggest that there is no advantage to using D-Pen in doses higher than 125 every other day.

Multicentric reticulohistiocytosis is a rare granulomatous disease of unknown etiology, characterized by cutaneous nodules and destructive arthritis. Skin lesions can cause significant deformity, and approximately half of affected patients develop a severe disabling arthritis. The disease is often associated with malignancy; however, the paraneoplastic nature of multicentric reticulohistiocytosis is not established. The diagnosis is confirmed by the presence of oncocytic ("ground-glass") histiocytes and multinucleated giant cells on histopathology of the cutaneous nodules and the synovial membrane.

Objective. The reported frequency of scleroderma M01-R renal crisis (SRC) in diffuse systemic sclerosis (SSc; scleroderma) is 15-20%. Early use of angiotensinconverting enzyme (ACE) inhibitors has markedly improved outcome. The present analysis reexamines the prognostic factors for and outcome of SRC in a prospective cohort of patients with early diffuse SSc.Methods. We retrospectively evaluated the cohort of SSc patients who participated in the High-Dose Versus Low-Dose D-Penicillamine in Early Diffuse SSc trial. Patients with diffuse cutaneous scleroderma were enrolled if their disease duration was <18 months. Because the trial failed to show a difference between treatment groups, the data were pooled.Results. One hundred thirty-four SSc patients entered the observation period a mean ؎ SD of 0.8 ؎ 0.3 years after onset of SSc. SRC occurred in 18 patients a mean ؎ SD of 0.9 ؎ 1.1 years after entry. During a mean ؎ SD 4.0 ؎ 1.1 years of followup after entry, 9 of the 18 patients died (mean ؎ SD 0.6 ؎ 0.9 years after SRC onset). Baseline characteristics that predicted SRC included a modified Rodnan skin thickness score of >20 (P < 0.01), enlarged cardiac silhouette on radiograph (P ‫؍‬ 0.04), large joint contractures (wrist, elbow, knee) (P ‫؍‬ 0.008), and prednisone use at entry (P ‫؍‬ 0.01). Baseline characteristics that did not predict SRC included age, sex, race, Health Assessment Questionnaire score, fist closure, handspread, lung involvement, muscle weakness, erythrocyte sedimentation rate, and platelet count. In 5 of 10 subjects for whom at least 2 sequential skin scores were available, skin scores increased significantly (P ‫؍‬ 0.012) in the 6 months before onset of SRC.Conclusion. SRC occurred in 13% of patients soon (mean 11 months) after entry into the cohort.

Two cases of septic sacroiliac (SI) joint arthritis are presented to illustrate the difficulty of diagnosing and treating this uncommon osteoarticular infection. The patients presented are a 68-year-old woman with septic sacroiliitis caused by Streptococcus agalactiae and a 20-year-old man with Salmonella infantis infection involving the SI joint. The recent literature is reviewed and compared with previously published series. Of the 177 cases we reviewed, 47 (27%) occurred in pediatric patients. The mean age was 20 years. Only six patients (3%) were older than 60 years of age. Magnetic resonance imaging (MRI) is most useful for defining extent of infection, osteomyelitis, and abscess formation in the SI joint. Computed tomography (CT) is valuable for defining the extent of bone involvement and for guidance of percutaneous needle arthrocentesis. Other imaging modalities are useful primarily in the initial evaluation of patients with a nonspecific presentation. Four to six weeks of intravenous (i.v.) antibiotic therapy is recommended. Indications for surgical intervention include abscess formation, osteomyelitis, sequestrum of necrotic bone, and failure to respond to i.v. antibiotic therapy.

Objective. To evaluate functional impairment in systemic sclerosis (SSc) patients with diffuse cutaneous scleroderma at the time of entry into a trial of a therapeutic intervention (D-penicillamine).Methods. The 20-item Disability Index of the Health Assessment Questionnaire (HAQ-DI) was administered to 134 patients as they entered a multicenter trial of high-dose versus low-dose D-penicillamine. All patients had diffuse SSc of <18 months' duration. SSc patients who had severe organ system involvement and recent renal crisis and who were receiving prednisone >10 mg/day were excluded from entry. Logistic regression modeling was used to examine the relationship of HAQ-DI scores to SSc skin and organ system involvement. Odds ratios (OR) and 95% confidence intervals (95% CI) were used to estimate effects. Conclusion. Increased HAQ-DI scores at baseline were correlated with reduced fist closure, reduced handspread, elevated platelet count, presence of tender joints, older age, and female sex. The most important contributor to functional impairment was hand dysfunction. Even within the first 18 months after SSc onset, moderate-severe functional impairment (HAQ-DI scores >1.0) was frequent (53%) in this group of diffuse SSc patients. In early diffuse SSc, the self-administered HAQ-DI is therefore a valuable assessment of function that correlates with objective physical and laboratory

The purpose of this investigation was to examine the safety and efficacy of four dosages of natural human interferon-alpha (nHuIFN-alpha) delivered over a 12-week period orally in lozenges (150 IU and 450 IU, once [QD] or three times [TID] daily) compared to placebo in subjects with primary Sjögren's syndrome. This randomized, double-blinded clinical trial demonstrated that nHuIFN-alpha at a dose of 150 IU administered TID by oral lozenge significantly improved stimulated whole saliva output compared to placebo after 12 weeks of treatment. The 150 IU TID dose also was suggestive of benefit for 5 of 7 subjective measures of oral and ocular comfort. IFN lozenges demonstrated a good safety profile, with no serious adverse events found in any treatment group. There were no significant differences between the placebo and the four doses of IFN for adverse events by total number, organ system, severity, dropouts, and number judged to be related to treatment. In conclusion, these results demonstrated that the use of 150 IU IFN lozenges TID for 12 weeks in subjects with primary Sjögren's syndrome improved salivary output and decreased complaints of xerostomia without causing significant adverse medical events.

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