Download the Free Unbound MEDLINE PubMed App to your smartphone or tablet.
Available for iPhone, iPad, iPod touch, and Android.
Rheumatology AND Dermatomyositis [keywords]
- Facial and laryngeal edema in a patient with dermatomyositis. [Journal Article]
- Intern Med 2014; 53(8):921.
- [A multi-center retrospective study of organ involvement in adult patients with polymyositis or dermatomyositis]. [English Abstract, Journal Article]
- Zhonghua Yi Xue Za Zhi 2014 Jan 7; 94(1):43-6.
To explore the prevalence and characteristics of main organ involvement in adult patients with polymyositis (PM) or dermatomyositis (DM) and determine their specific relative factors.Using unified questionnaire, we retrospectively collected the medical records of 1 387 confirmed adult PM/DM patients from 2007 to 2012 at 22 rheumatology centers in China. Statistical analyses were performed with chi-square or Fisher exact test and multivariate analyses with logistic regression.A total of 1 387 patients were collected with 460 (33.2%) PM and 927 (66.8%) DM. The female:male ratio was 2.4: 1. Their onset age was ( 47 ± 14) years. A total of 1 031 (74.3%) patients had organ involvement. The prevalence of pulmonary involvement, arthritis, gastrointestinal and cardiac involvement were 44.6%, 32.3%, 21.9% and 20.3% respectively. The multivariate analysis indicated that older onset age (P < 0.01) was positively associated with pulmonary involvement while myalgia (P < 0.05) was negatively associated. Fever (P < 0.05), weight loss (P < 0.05) and Raynaud's phenomenon (P < 0.01) were positively associated with arthritis while muscle weakness (P < 0.05) negatively associated. Weight loss (P < 0.05), Raynaud's phenomenon (P < 0.01) and muscle weakness (P < 0.05) were positively associated with gastrointestinal involvement. Weight loss (P < 0.05) and swollen limbs (P < 0.05) were positively associated with cardiac involvement.The prevalence of organ involvement is high in adult PM/DM patients. Our study may aid the diagnosis of organ damage in PM/DM patients.
- Serum interferon-α is a useful biomarker in patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis. [JOURNAL ARTICLE]
- Mod Rheumatol 2014 Apr 9.
Objective. We have tried to clarify the clinical importance of the measurement of serum type-I interferon (IFN) in patients with anti-melanoma differentiation-associated gene 5 Ab (MDA5 Ab)-positive dermatomyositis (DM). Methods. We studied 30 patients with DM: 10 were anti-MDA5 Ab-positive and 20 were anti-MDA5 Ab-negative. At each patient's initial visit, serum IFN-α, IFN-β, interleukin 18 (IL-18), ferritin, and the titer of anti-MDA5 Ab were measured using enzyme-linked immunosorbent assays (ELISAs). The associations between the IFNs and with the other variables were examined. Results. Rapidly progressive interstitial lung disease (RPILD) was confirmed in 10 patients, most of whom were complicated in the anti-MDA5 Ab-positive DM patients. The presence of clinically amyopathic dermatomyositis (CADM) as well as the serum concentrations of IFN-α and ferritin was significantly higher in the anti-MDA5 Ab-positive DM patients. Serum concentration of IL-18 did not differ between anti-MDA5 Ab-positive and anti-MDA5 Ab-negative groups; however, a positive correlation was found between IFN-α and IL-18 in the anti-MDA5 Ab-positive DM patients (r = 0.8139, p = 0.0146). Conclusion. Serum IFN-α can be used as a useful biomarker in patients with anti-MDA5 Ab-positive DM, which may reflect the presence of RPILD.
- B-cell activating factor as a serological biomarker for polymyositis and dermatomyositis. [Journal Article]
- Biomark Med 2014 Mar; 8(3):395-403.
Aim:To investigate serum levels of B-cell activating factor (BAFF) in the patients with polymyositis (PM) and dermatomyositis (DM), and to systematically examine the association between serum BAFF levels and disease activity in PM/DM patients. Patients & methods: A cross-sectional analysis included 92 PM/DM patients and 25 healthy control subjects. A longitudinal study followed 24 patients. Serum BAFF concentrations were detected by the ELISA method.
Results:Serum BAFF levels in PM/DM patients were significantly higher than those in healthy controls. A cross-sectional assessment revealed a modest correlation between serum BAFF levels and global disease activity and a mild correlation between serum BAFF levels and muscle disease activity. The longitudinal study showed that serum BAFF levels modestly correlated with global disease activity and muscle disease activity.
Conclusion:Resulting data showed high serum BAFF levels in PM/DM patients and suggested BAFF as a serological biomarker for PM/DM disease activity.
- Rituximab therapy in patients with refractory dermatomyositis or polymyositis: differential effects in a real-life population. [JOURNAL ARTICLE]
- Rheumatology (Oxford) 2014 Apr 4.
Objectives. While a double-blind trial has not met its endpoint, rituximab (RTX) is still seen as useful in refractory DM and PM. In this study we analysed the charts of all patients receiving RTX for myositis in our institutions for objective outcome parameters.Methods. In a retrospective way, the charts of all patients with PM or DM who received RTX were analysed for glucocorticoid dose, creatine phosphokinase (CPK) and lung function tests, as well as for serious adverse events.Results. A total of 19 patients were identified, 1 of whom died from aspiration pneumonia 3 weeks after the first RTX infusion. The charts of 18 patients (13 PM, 5 DM) could be further analysed. In addition to the fatal pneumonia, six more severe infections were seen. One patient developed hypogammaglobulinaemia. Two patients had mild infusion reactions. Under RTX, both CPK and daily prednisolone dose were reduced by week 18. Six of eight patients with alveolitis improved under RTX. Overall, 9 of 13 PM patients responded. Six of the responders and two patients without documented response, all anti-synthetase syndrome patients, were re-treated. In contrast, all five DM patients responded and none required re-treatment.Conclusion. In a real-life population of patients with severe, refractory PM or DM, objective improvement was seen in the majority of patients with regard to CPK and lung function tests, and glucocorticoids could be reduced. In contrast to the subgroup with DM, where one cycle of RTX appeared sufficient, patients with anti-synthetase syndromes commonly experienced flares necessitating RTX re-treatment. Infections are of concern.
- Juvenile dermatomyositis in a Nigerian girl. [Journal Article]
- BMJ Case Rep 2014.
Juvenile dermatomyositis is an autoimmune connective tissue disease occurring in children less than 16 years old. It is part of a heterogeneous group of muscle diseases called idiopathic Iiflammatory myopathies. It had previously been reported in black Africans resident in UK. However, there is no documented case reported from Africa. The index sign of heliotrope rashes is often difficult to visualise in the black skin. An 11-year-old Nigerian girl presenting with clinical, laboratory and histopathological features of juvenile dermatomyositis is presented here. It is hoped that this case will heighten the index of suspicion of this condition among medical practitioners in Africa.
- Prevalence and clinical characteristics of adult polymyositis and dermatomyositis; data from a large and unselected Norwegian cohort. [JOURNAL ARTICLE]
- Ann Rheum Dis 2014 Apr 2.
The occurrence of polymyositis (PM) and dermatomyositis (DM) in the general population is largely unknown and unbiased data on clinical and laboratory features in PM/DM are missing. Here, we aim to identify and characterise every PM/DM patient living in southeast Norway (denominator population 2.64 million), 2003-2012.Due to the structure of the Norwegian health system, all patients with PM/DM are followed at public hospitals. Hence, all public hospital databases in southeast Norway were screened for patients having ICD-10 codes compatible with myositis. Manual chart review was then performed to identify all cases meeting the Peter & Bohan and/or Targoff classification criteria for PM/DM.The ICD-10 search identified 3160 potential myositis patients, but only 208/3160 patients met the Peter & Bohan criteria and 230 the Targoff criteria (100 PM, 130 DM). With 56 deaths during the observation period, point prevalence of PM/DM was calculated to 8.7/100 000. Estimated annual incidences ranged from 6 to 10 /1 000 000, with peak incidences at 50-59 (DM) and 60-69 years (PM). Myositis specific antibodies (Jo-1, PL-7, PL-12, signal recognition particle (SRP) and Mi-2) were present in 53% (109/204), while 137/163 (87%) had pathological muscle MRI. Frequent clinical features included myalgia (75%), arthritis (41%) dyspnoea (62%) and dysphagia (58%). Positive anti-Jo-1, present in 39% of DM and 22% of PM cases, was associated with dyspnoea, arthritis and mechanic hands.Our data indicate that the population prevalence of PM/DM in Caucasians is quite low, but underscores the complexity and severity of the disorders.
- Disease activity and prognostic factors in juvenile dermatomyositis: a long-term follow-up study applying the Paediatric Rheumatology International Trials Organization criteria for inactive disease and the myositis disease activity assessment tool. [JOURNAL ARTICLE]
- Rheumatology (Oxford) 2014 Mar 31.
Objectives. The aims of this study were to examine disease activity by the Paediatric Rheumatology International Trials Organization (PRINTO) criteria for inactive disease and the Myositis Disease Activity Assessment Tool (MDAAT) in JDM patients after long-term follow-up and to identify predictors of these outcomes.Methods. A retrospective inception cohort of 59 patients diagnosed with JDM was clinically examined in a cross-sectional study a median of 16.8 years (range 2.0-38.1) after symptom onset. Patients were divided by the PRINTO criteria into clinically inactive and active disease. Disease activity was also measured by MDAAT and other validated tools. Medical records were reviewed for early disease variables and medication.Results. By the PRINTO criteria, 31/59 (51%) patients were active and 29/59 (49%) were inactive. By MDAAT, 43/59 (73%) of the patients had measurable disease activity, most commonly found in the skin (59%) and skeletal (27%) systems. MDAAT showed moderate to strong correlations with other disease activity measures (rsp 0.39-0.87, P < 0.05) except for muscle enzymes. Active patients had higher disease activity than inactive patients measured by MDAAT (P < 0.001) and other disease characteristics (all P ≤ 0.002) except for patients' global assessment of disease activity. After controlling for gender and follow-up time, calcinosis during disease-course predicted high MDAAT, age<9 years at diagnosis predicted active disease and organ damage present 6-12 months post diagnosis predicted both outcomes.Conclusion. After 16.8 years, 51-73% of JDM patients had active disease. Disease activity by the PRINTO criteria and MDAAT were moderately to highly associated with most other disease characteristics and was predicted by early damage.
- A51: c1q deficiency and autoimmunity-a single centre experience. [Journal Article]
- Arthritis Rheumatol 2014 Mar.:S76.
C1q deficiency is a rare form of monogenic autoimmune syndrome, associated with predisposition to bacterial infections and systemic lupus erythematosus (SLE) like phenotype. The aim of the study was to describe the clinical characteristics, including presentation, diagnostic features, treatment and outcome in children with primary C1q deficiency from a tertiary paediatric rheumatology service.Retrospective case notes review of children presenting with C1q deficiency to the Paediatric Rheumatology and Immunology services at Great Ormond Street Hospital for Children between 2002 and 2012, We included in the study all the children with a diagnosis of C1q deficiency, identified on initial screening as having low/absent functional classic complement pathway activity, with subsequent identification of absent C1q levels, in the absence of anti-C1q antibodies. Where possible, genetic confirmation of a mutation in C1q was obtained.Five children were identified as having primary C1q deficiency; 4 had complete deficiency; 1 had partial deficiency. One case had confirmation of C1q genetic mutations. The median age at disease onset was 12 months (range 6-34 months). Four children presented with autoimmune manifestations: SLE-like presentation (n = 3); JDM/SLE (juvenile dermatomyositis) overlap syndrome (n = 1). The patient with SLE/JDM phenotype had an extremely severe onset of autoimmune symptomatology, complicated by macrophage activation syndrome. None of the patients had nephritis. The patient with partial deficiency only had recurrent infections. One patient, the sibling of an index case, was asymptomatic at diagnosis but developed an SLE-like phenotype later on. Four patients had recurrent infections, 3 had severe invasive infections with encapsulated bacteria. Of the 4 patients with SLE symptomatology, none was positive for anti-dsDNA antibodies, 3 were ANA positive, and 3 were ENA positive. Three patients had transient moderate neutropenia at presentation. Treatment included steroids (n = 3/5) combined with Azathioprine (n = 3/5), Hydroxychloroquine (n = 2/5), or Methotrexate (n = 1/5) and antibiotic prophylaxis for infections (5/5). At median 7.9 (0.7-9.3) years follow up the current status of the patients was: all patients are alive; 3 have absence of symptoms on DMARDs, the patient with partial deficiency is on antibiotic prophylaxis with mild recurrent infections. The patient sibling of an index case who was initially asymptomatic, presented with SLE cutaneous manifestations 6 months after diagnosis, currently still active one month after commencing Azathioprine. None of the patients had a further episode of invasive life threatening infection.These data confirm a wide spectrum of severity and variability in the clinical phenotype of C1q deficiency. Most of the patients present with SLElike cutaneous manifestations, although severe invasive infection is a major concern. Early onset of lupus-like features with low prevalence of anti-dsDNA antibodies in the context of recurrent or severe invasive infections may provide a valuable diagnostic clue for the presence of C1q deficiency.