Objective Autoantibodies recognizing 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) are associated with statin exposure, the HLA allele DRB1*11:01, and necrotizing muscle biopsies in adult myositis patients. The aim of this study was to characterize the features of pediatric anti-HMGCR-positive myositis patients. Methods The sera of 440 juvenile myositis patients were screened for anti-HMGCR autoantibodies. Demographic and clinical features, responses to therapy, and HLA alleles were assessed. The features of anti-HMGCR-positive patients were compared to those of previously described adult patients with this autoantibody and to children with other myositis-specific autoantibodies (MSAs). Results Five (1.1%) of 440 patients were anti-HMGCR-positive; none had taken statin medications. Three patients had rashes characteristic of juvenile dermatomyositis and two patients had immune-mediated necrotizing myopathies. The median highest creatine kinase (CK) level of anti-HMGCR-positive subjects was 17,000 IU/L. All patients had severe proximal muscle weakness, distal weakness, muscle atrophy, joint contractures, and arthralgias, which were all more prevalent in HMGCR-positive subjects compared to MSA-negative patients or those with other MSAs. Anti-HMGCR-positive patients had only partial responses to multiple immunosuppressive medications and often a chronic course. The DRB1*07:01allele was present in all 5 patients compared to 26.25% of healthy controls (Pcorrected=0.01); none of the 5 pediatric patients had DRB1*11:01. Conclusions Compared to children with other MSAs, muscle disease appeared to be more severe in those with anti-HMGCR autoantibodies. Like adults, children with anti-HMGCR autoantibodies have severe weakness and high CK levels. In contrast to adults, anti-HMGCR-positive children have a strong association with HLA DRB1*07:01.

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OBJECTIVE:We developed an instructional program to teach aspiration and injection techniques of the knee and shoulder to medical students and residents. METHODS:Residents and fourth-year medical students participating in a rheumatology elective were assigned by deterministic allocation into 3 groups: the Traditional group received no specific instruction in arthrocentesis but simply rotated through rheumatology, learning injection techniques only if they saw patients who required them; the Lecture-only group received only the didactic lecture and did not have the opportunity to practice on the models; the Program group participated in the newly developed program of instruction that combined a didactic lecture and a hands-on workshop using the anatomic models to practice arthrocentesis techniques. RESULTS:The scores on the written examination for those in the Program group (mean score 37.46 out of 40 possible) and the Lecture-only group (mean 37.75) were significantly higher than those of the Traditional group (mean 33.15) (P < .05). The scores on the practical examination for those in the Program group (mean score 24.08 out of 26 possible) were significantly higher than those of the Lecture-only (mean 20.50) and Traditional (mean 17.33) (P < .05) CONCLUSION:The addition of this type of instruction to supplement a traditional internal medicine rotation can enhance a learner's ability to perform joint/soft-tissue injection and aspiration. J oint/soft-tissue injection and aspiration (JSIA) are procedures frequently performed by both specialists and primary care providers. Seventy-two percent of general internists and 87% of family physicians use these procedures in their practice. 1,2 Instruction in injection techniques should be a part of residency training. When queried however, only 36% of internal medicine residency program directors reported that all of their residents master JSIA compared to 87% for blood gas analysis and 83% for ECG interpretation. 3 Traditionally, JSIA is taught without the benefit of an organized approach. Experience in JSIA depends on whether a patient is seen who requires JSIA, and whether the staff physician feels comfortable supervising a learner. It is possible under such a system for a learner to complete his/her training without adequate instruction or experience in JSIA. Elnicki et al. have noted that, unfortunately, only a minority of office-level procedures are taught by faculty in medical training settings. 4 How then should teaching JSIA be approached? Instruction in core procedural skills is an essential component of almost all residencies. Since the didactic lecture format is not well suited for the acquisition of complex manual operations, these skills have been primarily taught through demonstration, followed by supervised performance on patients. However, concerns regarding the safety of patients and the lack of readily available faculty and patient populations limit the utility of such an approach. For this reason, there is a need for an instructional alternative that all...

Objective. To test the usefulness and cost savings resulting from application of the new American College of Rheumatology (ACR) guidelines for assessing the risk for the development of clinically significant liver disease in rheumatoid arthritis (RA) patients treated with methotrexate (MTX).Methods. One‐hundred twelve MTX‐treated RA patients were prospectively followed up for MTX hepatotoxicity and underwent liver biopsies according to modified guidelines of the Psoriatic Task Force (PTF). All biopsies were graded according to the Roenigk classification. The new ACR recommendations were then retrospectively applied to test their usefulness and cost‐effectiveness in this cohort.Results. Based on the PTF guidelines, 66 patients underwent liver biopsies; a total of 110 liver biopsies were performed. Two patients had biopsy‐related complications. Five patients were found to have Roenigk grade IIIB or IV histologic abnormalities. The total cost for this group was $111,380. Applying the new ACR criteria, only 15 patients would have undergone liver biopsies; there would have been a total of 18 biopsies, with no complications. Four of the 5 patients with Roenigk grade IIIB or IV liver abnormalities would have been identified. One patient with insulin‐dependent diabetes mellitus (IDDM) who was found to have cirrhosis (Roenigk grade IV) on liver biopsy as a result of use of the PTF guidelines would have been missed with use of the ACR guidelines. The total cost for the group receiving biopsies based on the ACR guidelines would have been $16,956. Overall, the new ACR guidelines had 80% sensitivity and 82% specificity and resulted in a cost savings of $1,430 per patient.Conclusion. The new ACR guidelines on MTX monitoring and biopsy surveillance appear to be clinically useful and result in considerable cost savings. However, 1 IDDM patient with significant liver histologic abnormalities would have been missed. We suggest that IDDM be added to the ACR guidelines as a risk factor for MTX hepatotoxicity.

Objective. Patients with systemic lupus erythematosus (SLE) who exhibit defective in vitro responses to recall antigens and normal responses to alloantigens have been shown to have an abnormality in antigenpresenting cell (APC) function. This study was undertaken to further characterize this defect in APC function in lupus patients.Methods. Mononuclear cells (MNC) from the peripheral blood of patients with SLE and from normal individuals were cultured in the presence of either recall antigen tetanus toxoid (TT), anti-CD3 (OKT3) monoclonal antibody, or alloantigens, and proliferative or interleukin-2 responses were assessed. Cell surface expression of B7-1 was assessed by flow cytometry.Results. MNC from all normal individuals and from 7 patients with SLE responded to both TT and alloantigen and were designated +/+. Twelve SLE patients did not respond to TT but did respond to alloantigen stimulation and were designated -/+. In ---The opinions and assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense.