Rheumatology AND Dermatomyositis [keywords]
- Autoimmune reaction after anti-tetanus vaccination-description of four cases and review of the literature. [JOURNAL ARTICLE]
- Immunol Res 2016 Jul 19.
Autoimmune reaction after vaccination is sporadically reported in the medical literature. Vaccinations are generally safe and have an important role in eradicating endemic diseases worldwide. Nevertheless, the question arises as to whether there is a possibility of post-vaccination autoimmune phenomena. The anti-tetanus vaccine is being used since 1924, and it is part of the recommended immunization schedules for children. There are few reports of autoimmune diseases, such as rheumatoid arthritis and anti-phospholipid syndrome after anti-tetanus vaccination. Herein, we describe four cases, of which we believe, show a clear temporal relation between anti-tetanus vaccination and the appearance of dermatomyositis, systemic lupus erythematosus, type 1 diabetes mellitus and anti-phospholipid syndrome. We also suggest some of the pathogenic mechanisms that promote a pathogenic autoimmune response.
- A Curious Case of Acute Respiratory Failure: Is It Antisynthetase Syndrome? [Journal Article]
- Case Rep Crit Care 2016.:7379829.
Antisynthetase (AS) syndrome is a major subgroup of inflammatory myopathies seen in a minority of patients with dermatomyositis and polymyositis. Although it is usually associated with elevated creatine phosphokinase level, some patients may have amyopathic dermatomyositis (ADM) like presentation with predominant skin involvement. Interstitial lung disease (ILD) is the main pulmonary manifestation and may be severe thereby determining the prognosis. It may rarely present with a very aggressive course resulting in acute respiratory distress syndrome (ARDS). We report a case of a 43-year-old male who presented with nonresolving pneumonia who was eventually diagnosed to have ADM through a skin biopsy without any muscle weakness. ADM may be associated with rapidly progressive course of interstitial lung disease (ADM-ILD) which is associated with high mortality. Differentiation between ADM-ILD and AS syndrome may be difficult in the absence of positive serology and clinical presentation may help in clinching the diagnosis.
- Autoimmune/Inflammatory Arthritis Associated Lymphomas: Who Is at Risk? [Journal Article, Review]
- Biomed Res Int 2016.:8631061.
Specific autoimmune and inflammatory rheumatic diseases have been associated with an increased risk of malignant lymphomas. Conditions such as rheumatoid arthritis (RA), primary Sjögren's syndrome (pSS), systemic lupus erythematosus (SLE), dermatomyositis, and celiac disease have been consistently linked to malignant lymphomas. Isolated cases of lymphomas associated with spondyloarthropathies and autoinflammatory diseases have also been reported. Direct association between autoimmunity and lymphomagenesis has been reinforced by large epidemiological studies. It is still uncertain whether disease specific determinants or phenotypic or treatment related characteristics increase likelihood of lymphomagenesis in these patients. For example, recent literature has indicated a positive correlation between severity of inflammation and risk of lymphomas among RA and Sjögren's syndrome patients. It is also debated whether specific lymphoma variants are more commonly seen in accordance with certain chronic autoimmune arthritis. Previous studies have revealed a higher incidence of diffuse large B-cell lymphomas in RA and SLE patients, whereas pSS has been linked with increased risk of mucosa-associated lymphoid tissue lymphoma. This review summarizes recent literature evaluating risk of lymphomas in arthritis patients and disease specific risk determinants. We also elaborate on the association of autoimmune arthritis with specific lymphoma variants along with genetic, environmental, and therapeutic risk factors.
- Survival rates of cancer patients with and without rheumatic disease: a retrospective cohort analysis. [Journal Article]
- BMC Cancer 2016.:381.
To compare the outcomes of gastric, colon, lung, and breast cancer patients with and without rheumatic diseases (RD).This retrospective study compared the cancer survival rates of a cohort of 122 cancer patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), dermatomyositis/polymyositis (DM/PM), or systemic sclerosis with that of a cohort of 366 age-, sex-, and, cancer type-matched patients without RD who received medical care from 2000 to 2014. Staging, comorbidities, and functional status were ascertained. Survival was compared using the Kaplan-Meier method. Relative risk of death was estimated as a hazard ratio (HR) using Cox regression analysis.The mean age of the RD patients at the time of cancer diagnosis was 58.7 ± 11.5 years. The overall survival rate of gastric cancer patients did not differ between the cohorts. The survival of lung or breast cancer was worse in patients with RA or DM/PM than in those without RD (all, p < 0.05). After adjusting for cancer stage, comorbidity index, performance status and age at the time of cancer diagnosis (as well as interstitial lung disease for lung cancer group), the mortality rate among lung cancer patients with RA was significantly higher (HR, 1.81; 95 % CI, 1.03-3.18) than that of lung cancer patients without RD, whereas SSc was associated with decreased mortality of lung cancer (HR, 0.16; 95 % CI, 0.04-0.58). DM/PM were associated with increased mortality of breast cancer patients (HR, 297.39; 95 % CI, 4.24-20842.33).RA and DM/PM seemed to be associated with a higher mortality in patients with lung or breast cancers, whereas SSc seemed to be associated with decreased mortality in patients with lung cancer. It is warranted to explore the survival effect of tailored cancer treatments according to specific RD.
- Capillaroscopy - a role in modern rheumatology. [Journal Article, Review]
- Reumatologia 2016; 54(2):67-72.
Capillaroscopy is a non-invasive, easy and safe diagnostic technique designed to evaluate small vessels of the microcirculation in the nailfold. It can reveal both the general architecture of capillary rows and fine details of particular vessels. The most important indications for performing capillaroscopy include differential diagnosis of primary and secondary Raynaud's phenomenon, as well as assessment of scleroderma spectrum disorders. In systemic sclerosis capillary abnormalities appear and evolve in a clearly defined sequence called the scleroderma pattern, which correlates with internal organ involvement. Capillaroscopy is also listed as a systemic sclerosis classification criterion recognized by the European League Against Rheumatism (EULAR). With digitized equipment, capillaroscopy allows for precise qualitative and quantitative evaluation of the microcirculation and is a valuable tool in the rheumatologists' daily practice.
- Power Doppler ultrasonography for detection of increased vascularity in the fascia: A potential early diagnostic tool in fasciitis of dermatomyositis. [JOURNAL ARTICLE]
- Arthritis Rheumatol 2016 Jul 7.
We previously demonstrated that fasciitis is a common lesion of dermatomyositis (DM) that is detectable early after disease onset by en bloc muscle biopsy combined with magnetic resonance imaging (MRI), while power Doppler ultrasonography (PDUS) is a useful method for detection of inflammation and vascularity in rheumatic diseases. We examined whether fasciitis was detectable by PDUS in patients with DM.We prospectively evaluated 7 patients with DM and 7 patients with polymyositis (PM) for the detection of fasciitis with PDUS in the current study. MRI and PDUS were both performed in all patients. Fasciitis was histologically confirmed by en bloc biopsy.Among all patients with DM, 4 showed signs of fasciitis on MRI, while increased blood flow signals were observed along the fascia by PDUS in 6 patients including those at earlier disease stages. Histologically, significant fasciitis was confirmed in 4 patients with DM. In the remaining 3 patients with DM, significant fasciitis was not evident histologically, but mild proliferation of capillaries and mild inflammation were notable in the area of the fascia. Immunohistochemical staining for CD31 indicated abnormal neovascular proliferation in the fascia in patients with DM. None of the PM patients showed signs of fasciitis or increased vascularity by MRI, PDUS or en bloc biopsy.In our limited population, PDUS is useful for the detection of fasciitis associated with DM especially in the early stage. The increased blood flow signal as detected by PDUS is involved in angiogenesis accompanying fasciitis in patients with DM. This article is protected by copyright. All rights reserved.
- [The application of (18)F fluorodeoxyglucose-positron emission tomography/computed tomography in the diagnosis and treatment of dermatomyositis]. [English Abstract, Journal Article]
- Zhonghua Nei Ke Za Zhi 2016 Jul 1; 55(7):525-30.
To investigate the value of (18)F fluorodeoxyglucose-positron emission tomography/computed tomography ((18)F FDG-PET-CT) in the diagnosis and the evaluation of disease activity and remission of dermatomyositis(DM).DM patients who were admitted to the Department of Rheumatology, the People's Liberation Army General Hospital (PLAGH) and underwent (18)F FDG-PET-CT examination were retrospectively reviewed from January 2012 to May 2015.Gender and age matched healthy controls (HC) were also enrolled.The standardized uptake value (SUV) of proximal limb girdle muscles in both groups were recorded and compared, so as between patients with DM or subclinical DM.The correlation between myodynamia of proximal limb girdle muscle, creatine kinase(CK), CK-MB, serum ferritin and SUV of each muscle group were analyzed.There were 58 patients with DM and 29 controls consecutively recruited in the study.The SUV of upper arms (1.814±0.830) g/ml, shoulders (2.134±0.797) g/ml and hips (1.883±0.683) g/ml in patients with classic DM were significantly higher than those with subclinical DM [(0.938±0.218) g/ml, (1.152±0.315) g/ml, (0.945±0.249) g/ml; P<0.05]. SUV of muscles in newly diagnosed patients was (1.051±0.031) g/ml, which was higher than that in subclinical patients.But the difference was not statistically significant (P>0.05). The average SUV of evaluated muscles in DM group (2.033±0.858) g/ml was significantly higher than that in controls (1.076±0.167) g/ml (P<0.001). Receiver operating characteristic(ROC) analysis revealed the area under the curve(AUC) of abnormal SUV detected by (18)F FDG-PET-CT for diagnosing DM was 0.953.The myodynamia of upper arms and SUV was negatively correlated (rs=-0.440, P=0.031). However, the level of serum creatine kinase and SUV was positively correlated (rs=0.500, P=0.013). The average SUV of patients whose time to remission was less than 3 months (1.746±0.466) g/ml was obviously less than that of patients with 3 to 6 months to obtain remission (2.815±0.848) g/ml (P=0.015).The SUV of proximal limb girdle muscles detected by (18)F FDG-PET-CT has a positive diagnostic value for DM.Moreover, the SUV in upper arms is correlated with the muscle strength and the level of creatine kinase, which reflect disease activity. (18)F FDG-PET-CT might be an alternative method to evaluate the response of treatment.
- The PRINTO juvenile dermatomyositis trial - Authors' reply. [Comment, Letter]
- Lancet 2016 Jun 25; 387(10038):2601.
- Anti-MDA5-Positive Dermatomyositis Presenting as Fever of Unknown Origin. [JOURNAL ARTICLE]
- J Gen Intern Med 2016 Jun 27.
Dermatomyositis is a chronic systemic autoimmune disease characterized by inflammatory infiltrates in the skin and muscle. The wide variability in clinical and serologic presentation poses a diagnostic challenge for the internist. Appreciation of the clinical variants of dermatomyositis allows for expedient diagnosis and avoidance of diagnostic error. We illustrate these challenges with the case of a 51-year-old Vietnamese-American man who initially presented with fever of unknown origin in the absence of overt skin and muscle manifestations. The diagnosis of dermatomyositis was not evident on several clinical encounters due to the absence of these hallmark symptoms. We review the variable clinical manifestations of a subtype of dermatomyositis associated with an autoantibody against melanoma differentiation-associated protein 5 (anti-MDA5) and suggest consideration of dermatomyositis as a diagnosis in patients presenting with systemic illness and markedly elevated ferritin, even in the absence of elevated muscle enzymes and classic autoantibodies.
- Increased Cumulative Incidence of Dermatomyositis in Ulcerative Colitis: a Nationwide Cohort Study. [Journal Article]
- Sci Rep 2016.:28175.
On a molecular level, two autoimmune diseases: ulcerative colitis (UC) and dermatomyositis share common genetic determinants. On a clinical level, case reports evidenced the co-occurrence of these two diseases. We therefore hypothesize that UC is potentially associated with increased cumulative incidence of dermatomyositis. The goals of this retrospective cohort study were to evaluate whether UC is associated with increased cumulative incidence of dermatomyositis independent of sex and age. For comparison, we also assessed the cumulative incidence of polymyositis in UC and control subjects. The study enrolled 3,133 UC subjects and 14,726 control subjects. The cumulative incidence of dermatomyositis was significantly higher in UC than that of control subjects (p = 0.026), but the cumulative incidence of polymyositis was comparable between UC and control subjects (p = 0.596). UC was independently associated with the increased incident dermatomyositis (hazard ratio: 6.19, 95% confidence interval = 1.77-21.59, p = 0.004) after adjusting for sex, age, and concomitant rheumatoid arthritis, systemic lupus erythematosus, and systemic sclerosis. Similar trends of increased dermatomyositis in UC were observed when patients were stratified based on sex and age. In conclusion, our findings suggest that UC is probably associated with increased cumulative incidence of dermatomyositis, independent of sex, age, and concomitant autoimmune diseases.