Clinical Characteristics of Juvenile Idiopathic Inflammatory Myopathy and Comparison With Adult Patients: Analysis From a Multicentric Cohort in Spain.

METHODS: This study reviewed the medical records of patients from the REMICAM cohort, a multicentric longitudinal study carried out in patients with IIM, followed up between 1980 and 2014 in 12 hospitals in Madrid, Spain. Patients with definite or probable JPM, JDM, adult DM, and adult PM according to the modified Bohan and Peter criteria were selected. We compared the characteristics between JDM and JPM, and between JIIM and adult IIM. RESULTS: Eighty-six juvenile patients (75 JDMs and 11 JPMs) and 283 adult patients (133 DMs and 150 PMs) were included. Compared with patients with JDM, patients with JPM were older at diagnosis, had more fever and arthritis, and were less frequently treated with disease-modifying antirheumatic drugs (these differences were not statistically significant). Compared with patients with adult DM, those with JDM presented more frequently with calcinosis (33.8% vs 6.9%, p < 0.0001) and had less severe infections (4.3% vs 23.4%, p < 0.0001), malignancies (1.3% vs 25.6%, p < 0.0001), and mortality (3.5% vs 33%, p < 0.0001). Patients with JDM were treated less frequently with azathioprine (10.8% vs 44.7%, p < 0.0001). CONCLUSIONS: Our findings confirm that JIIMs are a heterogeneous group of diseases with relevant differences compared with adult IIMs.

Radiotherapy for ostheoarticular degenerative disorders: When nothing else works.

Objectives: Show the results in pain and functionality, using low-dose radiotherapy in osteoarticular degenerative disorders (OADD). Review of the evidence. Material and methods: Patients suffering from OADD with no response to other treatments, receive 6Gy in 6 fractions of 1 Gy, each other day, repeating the scheme if necessary. Evaluation of pain based on Visual Analogic Scale, analgesia intake and VonPannewitz score. Results: Results observed in our series of patients treated with low doses of radiotherapy are similar to those previously published and reinforce the consideration of radiotherapy as an useful option for degenerative musculoskeletal disorders. Conclusion: Low dose radiotherapy seems to be a good alternative for aged patients suffering from OADD.

223Ra-dichloride response in a patient with Paget disease and bone metastases secondary to castration-resistant prostate cancer.

PURPOSE: Paget disease is commonly asymptomatic and discovered when an imaging test is performed for another clinical indication or when elevated serum alkaline phosphatase is found. Bone pain usually appears late in the disease process and is only present in a minority of patients. For diagnosis, X-ray and bone scan are the most recommended imaging methods; radionuclide imaging of the skeleton has become the standard, since it is the most sensitive test for detecting increased bone activity. For treatment, either bisphosphonates or calcitonin are recommended. METHODS: We present a 74-year-old patient diagnosed with prostate cancer in 2001 who developed bone metastases concomitant with a Paget bone disease. RESULTS: This patient received treatment with Ra-223, having stable disease in bone scan and no relevant toxicities. CONCLUSIONS: There is no clinical experience with Ra-223 and Paget disease, since it is characterized classically as a high bone turnover disease and therefore there is no rationale to administer a drug that has a high bone affinity. Nevertheless, Ra-223 is not contraindicated.

Multicenter registry on inflammatory myositis from the Rheumatology Society in Madrid, Spain: Descriptive Analysis. / Registro de pacientes con miopatía inflamatoria de la Sociedad Madrileña de Reumatología: análisis descriptivo.

OBJECTIVE: To analyze clinical characteristics, survival and causes of death of patients diagnosed with autoimmune inflammatory myositis in the REMICAM registry from the Society of Rheumatology in the Community of Madrid (SORCOM). METHODS: Multicenter cohort of patients diagnosed with autoimmune inflammatory myopathy with follow-up between January 1980 and December 2014. A total of 313 variables concerning demographic, clinical and morbidity data were collected, and a comparison was performed between clinical subgroups. RESULTS: A total of 479 patients were recruited from 12 centers, with 14% of patients lost to follow-up. Seventy-four percent of cases were women, age at diagnosis of 44±23 years and a mean follow-up period of 10±8 years. The most frequent clinical subgroups were primary myositis (PM 29%, DM 22%), followed by overlap myositis (20.5%), juvenile myositis (18%), myositis associated with cancer (8%), immune-mediated necrotizing myositis (1%) and inclusion body myositis (1%). During the follow-up period, a total of 114 deaths (28%) were registered, the main causes being cancer (24%), infections (23%) and cardiovascular events (21%). CONCLUSIONS: A total of 479 patients were recruited in the REMICAM registry of inflammatory myopathies. Including sociodemographic, clinical and prognostic information, it represents the largest Spanish multicenter registry to date in rheumatology, and constitutes an important source for conducting further substudies.

Influence of the IL6 gene in susceptibility to systemic sclerosis.

OBJECTIVE: Systemic sclerosis (SSc) is a genetically complex autoimmune disease; the genetic component has not been fully defined. Interleukin 6 (IL-6) plays a crucial role in immunity and fibrosis, both key aspects of SSc. We investigated the influence of IL6 gene in the susceptibility and phenotype expression of SSc. METHODS: We performed a large metaanalysis including a total of 2749 cases and 3189 controls from 6 white populations (Germany, The Netherlands, Norway, Spain, Sweden, and United Kingdom). Three IL6 single-nucleotide polymorphisms (SNP; rs2069827, rs1800795, and rs2069840) were selected by SNP tagging and genotyped using TaqMan(®) allele discrimination technology. RESULTS: Individual SNP metaanalysis showed no evidence of association of the 3 IL6 genetic variants with the global disease. Phenotype analyses revealed a significant association between the minor allele of rs2069840 and the limited cutaneous SSc clinical form (Bonferroni p = 0.036, OR 1.14, 95% CI 1.04-1.25). A trend of association between the minor allele of the rs1800795 and the diffuse cutaneous SSc clinical form was also evident (Bonferroni p = 0.072, OR 0.86, 95% CI 0.77-0.96). In the IL6 allelic combination analyses, the GGC allelic combination rs2069827-rs1800795-rs2069840 showed an association with overall SSc (Bonferroni p = 0.016, OR 1.13, 95% CI 1.04-1.23). CONCLUSION: Our results suggest that the IL6 gene may influence the development of SSc and its progression.