Course Of Dermatomyositis Research Articles

Impact of Exercise on the Course of Dermatomyositis: A Narrative Review of Recent Studies

Introduction and Purpose: Dermatomyositis (DM) is a rare autoimmune inflammatory myopathy characterized by muscle weakness and distinctive skin manifestations, impacting both children and adults. This narrative review aims to examine the latest studies assessing the impact of physical activity on the course of dermatomyositis and determine which forms of exercise are beneficial for DM patients. Methods: A comprehensive literature review was conducted using the PubMed and Google Scholar databases, focusing on articles published between 2018 and 2024. State of Knowledge: The review identified consistent evidence supporting the safety and efficacy of exercise in DM patients. Exercise regimens, including resistance, aerobic, and stretching exercises, were found to improve muscle strength, aerobic capacity, and metabolic functions without increasing disease activity. Novel exercise approaches, such as blood flow restriction training and aquatic plyometric exercises, also showed promising results. However, the heterogeneity of the trials and small sample sizes highlight the need for larger, more diverse cohorts and long-term studies Summary: The findings underscore the potential of structured exercise programs to enhance muscle strength, aerobic capacity, and overall physical function in DM patients. These improvements are achieved without exacerbating disease activity, highlighting the safety of exercise interventions. The review emphasizes the importance of incorporating physical activity into treatment plans for DM, advocating for personalized exercise prescriptions tailored to the unique inflammatory nature of idiopathic inflammatory myopathies (IIM).

Calcifications of the skin and subcutaneous tissues in the course of dermatomyositis

Calcifications of the skin and subcutaneous tissues in the course of dermatomyositis

Evaluation of the frequency and risk factors of spontaneous intramuscular hemorrhage associated with dermatomyositis

Dermatomyositis (DM) is an idiopathic inflammatory myositis (IIM) characterized by skin manifestations and muscle involvement. Spontaneous intramuscular hemorrhage (SIH) is a fatal complication that is very rare in the course of DM, but not well known to rheumatologists. Our aim was to determine the frequency and possible risk factors of DM-related SIH. A retrospective analysis was conducted on a cohort of DM patients who were observed in the rheumatology department of the university hospital between 1998 and January 2024. The clinical, laboratory, radiological data of the patients and the treatments they received during the follow-up were analyzed. To determine possible risk factors for the development of SIH in the course of DM, our patients with DM were analyzed together with other rare SIH cases in the literature. The study included 42 of our DM patients. 32 of the patients (76.2%) were female. The median age of the patients was 53 (24–82) years, the median age of DM diagnosis of the patients was 47 (18–75) years, and the median duration of DM of the patients was 36 (2-276) months. 7.1% of patients had dysphagia, and 16.7% had intertitial lung disease (ILD). 5 (11.9%) patients were diagnosed with malignancy. The incidence rate of SIH development in our DM cohort was 0.238/100 patient years (95% CI 0.006–1.256). We tried to identify independent risk factors for SIH development by comparing our 41 DM patients without SIH with the data of patients with 23 DM-related SIH collected from the literature by adding our 1 patient (24 pts). Male sex (OR 4.97, 95% CI 1.66–14.92, p = 0.003), ILD presence (OR 9.71, 95% CI 2.99–31.47, p < 0.001), anti-MDA5 positivity (OR 16.0, 95% CI 1.60-159.3, p = 0.006), anti-Ro52 positivity (OR 11.6, 95% CI 2.93–46.34, p < 0.001), heparin use (OR 4.42, 95% CI 2.68–7.24, p < 0.001), intravenous immunoglobulin (IVIG) use (OR 11.7, 95% CI 2.26–60.54, p < 0.001), and steroid dose (OR 1.03, 95% CI 1.00-1.05, p = 0.005) were identified as risk factors for the development of SIH in the univariate analysis. The death rate due to hemorrhage was 50%. No single risk factor was found to be associated with death. As a result, SIH may occasionally arise in patients with DM. Rheumatologists should be aware that patients with dysphagia and/or ILD, who are on heparin, getting high doses of steroids, and test positive for anti-MDA5 and/or anti-Ro52 antibodies may develop SIH in the early stages of DM.

A case of autoimmune pulmonary alveolar proteinosis during the course of treatment of rapidly progressive interstitial pneumonia associated with anti-MDA5 antibody-positive dermatomyositis.

Autoimmune pulmonary alveolar proteinosis (APAP) is a diffuse lung disease that causes abnormal accumulation of lipoproteins in the alveoli; however, its pathogenesis remains unclear. Recently, APAP cases have been reported during the course of dermatomyositis. The combination of these two diseases may be coincidental; however, it may have been overlooked because differentiating APAP from a flare-up of interstitial pneumonia associated with dermatomyositis is challenging. This didactic case demonstrates the need for early APAP scrutiny. A 50-year-old woman was diagnosed with anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatitis and interstitial pneumonia in April 2021. The patient was treated with corticosteroids, tacrolimus, and cyclophosphamide pulse therapy for interstitial pneumonia complicated by MDA5 antibody-positive dermatitis, which improved the symptoms and interstitial pneumonia. Eight months after the start of treatment, a new interstitial shadow appeared that worsened. Therefore, three additional courses of cyclophosphamide pulse therapy were administered; however, the respiratory symptoms and interstitial shadows did not improve. Respiratory failure progressed, and 14months after treatment initiation, bronchoscopy revealed turbid alveolar lavage fluid, numerous foamy macrophages, and numerous periodic acid-Schiff-positive unstructured materials. Blood test results revealed high anti-granulocyte-macrophage colony-stimulating factor (GM-CSF) antibody levels, leading to a diagnosis of APAP. The patient underwent whole-lung lavage, and the respiratory disturbance promptly improved. Anti-GM-CSF antibodies were measured from the cryopreserved serum samples collected at the time of diagnosis of anti-MDA5 antibody-positive dermatitis, and 10months later, both values were significantly higher than normal. This is the first report of anti-MDA5 antibody-positive dermatomyositis complicated by interstitial pneumonia with APAP, which may develop during immunosuppressive therapy and be misdiagnosed as a re-exacerbation of interstitial pneumonia. In anti-MDA5 antibody-positive dermatomyositis, APAP comorbidity may have been overlooked, and early evaluation with bronchoalveolar lavage fluid and anti-GM-CSF antibody measurements should be considered, keeping the development of APAP in mind.

Clinical value of YKL-40 in patients with polymyositis/dermatomyositis: A cross-sectional study and a systematic review.

IntroductionWe performed a cross‐sectional study to investigate the clinical usefulness of YKL‐40 in patients with dermatomyositis (DM) and conducted a systematic review to summarize the clinical value of YKL‐40 in patients with polymyositis (PM)/DM.Materials and methodsA cross‐sectional study and a systematic review were performed to study the clinical value of YKL‐40 in patients with PM/DM. Serum YKL‐40 level was detected using enzyme‐linked immunosorbent assay, and its association with clinical and laboratory parameters was analyzed. In the systematic review, electronic databases of OVID Embase, OVID Medline, and web of science were searched to collect studies that reported clinical use of YKL‐40 in patients with PM/DM.ResultsIn the cross‐sectional study, serum YKL‐40 level was higher in patients with DM than in healthy controls (median [interquartile range]: 84.09 [52.72–176.4] ng/ml versus 27.37 [12.30–53.58] ng/ml, p < 0.0001). Serum levels of YKL‐40 were associated with the course of DM (r = −0.469, p < 0.001), CRP (r = 0.303, p = 0.043), CK (r = 0.263, p = 0.037), and global disease activity (r = 0.628, p < 0.001). The area under the ROC curve was 0.835 (95% confidence interval 0.751–0.920). In the systematic review, a total of four studies were included with moderate to high quality. Serum level of YKL‐40 has the possibility for diagnosing PM/DM, identifying PM/DM patients with interstitial lung disease (ILD) or rapid progress ILD, and predicting death.ConclusionSerum YKL‐40 level is a possible useful biomarker for PM/DM diagnosis and may be used to predict prognosis.

Dermatomyositis as a paraneoplastic syndrome of head-neck-cancer: Case series & literature review

Dermatomyositis (DM) is a rare disease with the clinical manifestation of weakness and pain of proximal muscles as well as lilac-coloured skin lesions. One fifth of the cases is associated with the occurrence of a malignant tumor disease. The aim of this study is to evaluate the relevance of DM as a paraneoplastic syndrome in head and neck cancer taken into account the current literature. After retrospective analysis of medical records of head-neck-cancer patients treated between 2008 and 2018, 8 patients with DM were detected: 4 patients with tonsil carcinoma, 1 patient with nasopharyngeal carcinoma, 1patient carcinoma of the parotid gland and two patients with lymphoma. The diagnosis, therapy and treatment results of these cases are described. Furthermore, a selective analysis of the literature (pubmed) about DM with head-neck cancer was conducted. A total of 290 cases were identified: In 283cases, the tumors were located in the nasopharynx, in five cases in the tonsil and in two cases in the hypopharynx. DM as a paraneoplastic syndrome of head and neck tumors is rare and more often associated with nasopharyngeal cancer and rarely with tonsil cancer. The clustering of DM with head and neck tumors regarding ethnicity (nasopharyngeal carcinoma - Asian origin, tonsillar carcinoma- Caucasian origin) might be due to the regional different incidences of these tumor entities.In patients with DM, especially in presence of cervical lymphadenopathy a tumor in the head and neck area should be evaluated. The course of tumor-associated DM is positively influenced by tumor therapy. The consistent therapy and monitoring of DM is fundamental for a successful tumor treatment as well.

Association of Short-Term Ultraviolet Radiation Exposure and Disease Severity in Juvenile Dermatomyositis: Results From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry.

Ultraviolet (UV) radiation is considered to be an important environmental factor in the clinical course of children with juvenile dermatomyositis (DM). We aimed to evaluate the association between UV radiation and severe disease outcomes in juvenile DM. This is a cross-sectional study of patients with juvenile DM enrolled in the US multicenter Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry from 2010 to 2015. The mean UV index (UVI) in the calendar month prior to symptom onset in each subject's zip code was calculated from daily satellite solar noon measurements. Multivariable logistic regression was used to model the relationship between the mean UVI and calcinosis as well as other outcomes of severe disease. Covariates included sex, race, age, time to diagnosis, disease duration, and latitude. In a multivariable model, there was no association between the mean UVI and calcinosis. African American race was associated with a 3-fold greater odds of calcinosis. However, there was a significant statistical interaction between race and mean UVI. Accounting for this interaction, the odds of calcinosis markedly decreased in African American subjects and steadily increased in non-African American subjects over a range of increasing the mean UVI. Higher mean UVI was associated with decreased odds of using biologics or nonmethotrexate disease-modifying antirheumatic drugs and skin ulceration. We described a novel association between UV radiation, calcinosis, and race in a large cohort of patients with juvenile DM. This study furthers our knowledge of the role of UV radiation in the clinical course of juvenile DM and highlights the complex interplay between genes and environment in the clinical phenotypes and development of calcinosis in children with juvenile DM.

1. TIF1-γ associated dermatomyositis

IntroductionThis is a case of a patient presenting at the age of 39 with dermatomyositis. Subsequent investigations revealed her to have a TIFI-γ antibody and a metastatic colon adenocarcinoma. She was initially steroid responsive and her malignancy simultaneously responded to chemotherapy. A relapse of her malignancy was preceded by a flare in her dermatomyositis which proved to be non-responsive to steroids and further chemotherapy.Case descriptionThe patient presented to rheumatology at the age of 39 with a few-week history of a photosensitive rash on her face, upper chest and hands. This was followed by rapidly progressive symmetrical proximal and truncal muscle weakness and pain. She described dysphagia to solids and shortness of breath on exertion. She had a very short history of weight loss, attributed to reduced oral intake. She had no relevant past medical history and was taking no medications. Examination revealed power of 3/5 in both upper and lower limbs, a heliotrope rash with Gottrons papules with normal cardiovascular and respiratory examinations.CK at presentation was 3709. Haematology and biochemistry was otherwise normal. MRI thighs showed extensive myositis. ANA was positive with a positive TIFI-γ antibody. CT scan showed a proximal sigmoid mass with local and mediastinal lymphadenopathy and a 6mm lung nodule. Histology from a mediastinal node confirmed a metastatic adenocarcinoma.Initially, the patient was treated as an autoimmue dermatomyositis with pulsed IV methylprednisolone followed by high dose prednisolone. She responded rapidly both clinically and biochemically to steroids.Following the diagnosis of malignancy, she underwent a hemicolectomy from which she made an uneventful recovery. She then completed 12 cycles of oxaliplatin and 5FU chemotherapy, with interval CT scanning showing good partial response to treatment and she returned to work.One month after completing chemotherapy, whilst still taking prednisolone her rash reoccurred. This rapidly progressed despite an increase in her steroids and quickly became associated with weakness and further shortness of breath. CT showed a progression of her malignancy with carcinomatosis lymphangiitis. She received one cycle of Irinotecan before being admitted with neutropenic sepsis and progression of her cancer. At this time she decided to withdraw all treatment and died shortly afterwards at the age of 40, 18 months after her initial presentation.DiscussionTIF1-γ antibodies were first identified in 2006 and are involved in cell regeneration, apoptosis and innate immunity. High levels of TIFI-Y are found in the nuclei of regenerating myofibres. They are associated with dermatomyositis and are found in between 13 – 31% of adults and 22 – 29% of children.There is a strong association with malignancy in those aged over 39 (positive predictive value of 58%, sensitivity 78%, specificity 89%). There are no case reports of malignancy associated with TIF1- Y antibodies in patients under the age of 39. Malignancy typically presents early in the course of dermatomyositis, being diagnosed at presentation or within 8 months. There are case reports of TIF1- Y antibodies co-existing with Mi2 antibodies, increasing malignancy risk.In younger patients this association with cancer is not seen but the antibody is associated with skin ulceration and chronic disease. It is hypothesised that differences in HLA regions and protein conformation may account for these different phenotypes. Patients typically have a lower CK and there is a higher incidence of amyopathic dermatomyositis compared with other myositis specific antibodies.Our patient was at the lower end of the risk spectrum for malignancy and had no localising symptoms for this. She was initially very steroid responsive, which again lowered our threshold for suspicion of malignancy. Indeed, her CT was requested on the basis of shortness of breath looking for interstitial lung disease rather than anything more sinister.Key learning pointsTIF1-γ antibodies have a strong association with malignancy in patients over the age of 39. Clinicians should have a high index of suspicion even in the absence of symptoms of malignancy. Malignancy associated dermatomyositis can be steroid responsive. A relapse of dermatomyositis should raise suspicion for a relapse of malignancy.Conflicts of interestThe authors have declared no conflicts of interest.

341 A longitudinal study of cutaneous dermatomyositis

Prior studies have described the course of dermatomyositis (DM) using muscle weakness and enzymes; however, limited studies have described the course of cutaneous disease. A retrospective cohort included patients 18 years or older with clinical or histologic evidence of DM who had the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) activity subscore recorded for at least 2 years. Disease progression was classified into improved, worsened, or stable based on net area under the curve per unit time relative to baseline CDASI score and a fitted linear slope. Disease course was classified into monophasic, polyphasic, or chronic based on definitions in the literature. Subjects were divided into mild and moderate-severe (MS) baseline severity groups and outcome measures were compared. In our cohort of 40 DM patients, the majority were female (90%) and Caucasian (95%), with a mean age of 52.9 years at baseline. Disease subtype was classified as classic (52.5%) and skin predominant (47.5%). Mean follow-up time was 3.50 years (SD=1.0). More patients had MS disease at baseline (N=24, 60%) compared to mild disease (N=16, 40%). Average disease activity was significantly different between the mild and MS groups (9.10 vs. 14.96; P = 0.004). The majority of DM patients showed an improvement in disease activity (N=23, 57.5%) compared to a worsening (N=8, 20%) and stable (N=9, 22.5%) progression. Within the mild group, a majority of the patients’ disease activity remained stable (N=8, 50%) compared to improvement in the MS group (N=20, 83%). Most DM patients had a polyphasic course (N=33, 82.5%) compared to monophasic (N=5, 12.5%) and chronic (N=2, 5%) courses. Variability in disease activity over time (average flares/yr) was independent of baseline disease activity. In summary, the majority of our patients had MS disease activity at baseline that improved with a polyphasic course while fewer had mild baseline disease that mostly remained stable with a polyphasic course.

Dermatomyositis: factors predicting relapse.

The course of dermatomyositis (DM) can be chronic with relapses, which are associated with major morbidity. The aim of this study was to identify presentation features that predict DM relapses. We retrospectively reviewed data of patients with DM recorded from 1990 to 2011, including muscle biopsy results. Characteristics of patients with and without relapses were compared. Hazard ratios (HRs) were estimated using a Cox model. We identified 34 patients, with a mean age of 46 ± 17 years (range, 18-77) and 24 (71%) women. The muscle and skin abnormalities relapsed in 21 (61%) patients. By univariate analysis, two presentation features were significantly associated with a subsequently relapsing course, namely, dysphonia [HR = 3.2 (1.2-8.5)] and greater skin lesion severity defined as a Cutaneous Disease Area Severity Index [CDASI] > 20 [HR = 3.5 (1.2-7.9)]. Dysphonia and skin lesion severity at disease onset must be recorded, as they significantly predict a relapsing disease course.

A New Outlook on Clinical Course of Juvenile Dermatomyositis - Experience of a Single Center

Introduction: Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in children. Diagnostic criteria by Bohan & Peter are originally formulated for adults. A wide range of differences in clinical course of dermatomyositis between adults and children has provoked to set a new look at the existing criteria. Objective: The aim of our study was to analyze the clinical course and reaction to administered drug therapy in association with laboratory and electromyographic findings in pediatric patients with dermatomyositis. Materials and methods: The retrospective analysis included 5 children with JDM hospitalized in Department of Pediatric Cardiology and Rheumatology Medical University of Lodz, Poland between October 2000 and November 2014. Demographic data, characteristics of clinical symptoms, laboratory tests, EMG and muscle biopsy reports and reaction to administered treatment were analyzed. Results: Muscle weakness was the most common symptom observed in 80% of patients. Other symptoms present in more than a half of patients included heliotrope rash (60%) and Gottron sign (60%). Muscle enzyme levels were significantly elevated in 4 out of 5 patients. Electromyography (EMG) was performed in 3 patients and was positive in every case, as well as muscle biopsy. Initial therapy involved prednisone 1mg/kg b.w./day in all patients combined with disease modifying anti-rheumatic drug, whether methotrexate was the most common choice (60%). Complications which occurred in presented patients involve: rhabdomyolysis, severe calcinosis and contractures of elbows, exacerbation after reduction of GCS dose, hypertriglyceridemia, steatohepatitis, tachycardia.

Dermatomyositis: A Retrospective Study of Sixteen Cases

Introduction: Dermatomyositis is an inflammatory multisystemic disease characterized by muscle weakness with a characteristic and pathognomonic cutaneous eruption. Our purpose was to describe the common cutaneous, extra-cutaneous features and course of dermatomyositis in our institution. Method: In this retrospective study we reviewed the medical data of dermatomyositis registered cases in dermatology department at Treichville teaching hospital in Cote d'ivoire from 2004 to 2013. Diagnosis was assessed for each case using the Bohan and Peter criteria for dermatomyositis. Result: Sixteen cases which fulfilled at least three out of four of the Bohan and Peter criteria for dermatomyositis were analysed. Prevalence was 15.3 for 10000 consulting patients. The delay to establish the diagnosis after the onset of the disease was 4 months. The average age of patients was 39 years. The sex ratio was 1.2. Features were: hyperpigmentation of sun exposed sites in 100% of cases, symmetric erythema in 93.7% of the cases, facial oedema in 56.2% of the cases, poikilodermatous lesions in 25% of the cases. Telangiectasia was noted in 6.2% of the cases. The average delay for occurrence of muscular symptoms after the onset of cutaneous symptoms was 1 month. The longest delay was 4 months. All cases had at least proximal muscles weakness. Three cases representing 18.7% of cases displayed oesophageal .muscles involvement. Muscular biopsy analysis performed in 10 cases that represent 62.5% of cases was specific in 4 cases (40%) and not in 6 cases (60%). Electromyogram was performed in 75% of cases and displayed myogenic deficit in all of them. Serum LDH level has been abnormal in all cases. Whereas serum CPK level has been abnormal in 87.5% of the cases. Two cases representing 12.5% of the cases had interstitial pneumonia and pericarditis. In an average period follow-up of 12 months death occurred in 6.2% of the cases and invalidity was constant. We used oral prednisone in 93.7% of cases. The maximal dose useful to induce remission was 60 mg of prednisone a day. The minimal dose useful for remission control was 10 mg a day. The average delay for partial remission was 10 days. Relapse occurred in all cases when decreasing doses of prednisone. Conclusion: We confirm that dermatomyositis is an uncommon disease. Our cases of dermatomyositis are relatively younger than those classically described. There was no sex predominance.

AB0649 Clinical Features of Spontaneous Pneumomediastinum in Dermatomyositis

BackgroundSpontaneous pneumomediastinum is a rare complication and a poor prognostic factor of dermatomyositis (DM). But the mechanisms, outcome, and prognostic factors of DM associated pneumomediastinum have not yet been evaluated.ObjectivesTo...

A fatal case of acquired hemophagocytic lymphohistiocytosis (macrophage activation syndrome) in the initial course of dermatomyositis with anti‐Jo‐1 antibody

A fatal case of acquired hemophagocytic lymphohistiocytosis (macrophage activation syndrome) in the initial course of dermatomyositis with anti‐<scp>J</scp>o‐1 antibody

A Case of a Patient with Dermatomyositis Who Developed a Diffuse Alveolar Hemorrhage

Dermatomyositis is a inflammatory myopathy associated with interstitial lung disease in 5∼10% of cases. The occurrence of diffuse alveolar hemorrhage (DAH) is considered exceptional in the course of dermatomyositis. We experienced a case of DAH in a 58-year-old female with dermatomyositis. DAH was diagnosed by anemia, infiltration on chest X-ray and computed tomography, and a bloody aspirate on bronchoscopy. Combined Pneumocys

Predicting the course of juvenile dermatomyositis: Significance of early clinical and laboratory features

Juvenile dermatomyositis (DM) is a rare chronic inflammatory disease of childhood. The clinical course of juvenile DM appears to be variable, and little is known about predictors of the disease course. The aims of this study were to describe the clinical course of juvenile DM and to determine whether early clinical and laboratory features can be used to predict the time to remission and/or the disease course. Clinical and laboratory data from a cohort of 84 patients with juvenile DM were prospectively entered into a database (1990-2005). Remission was defined as a clinical state of no active skin rash, weakness, or elevated muscle enzyme levels for 6 months off medication. The disease course was defined as monophasic, polyphasic, or chronic. Data were reviewed at the time of diagnosis and at 3 months and 6 months after the diagnosis to determine predictors of the time to remission and/or the disease course. The median time to remission was 4.67 years. Sixty percent of patients had a chronic course, 37% a monophasic course, and 3% a polyphasic course. The presence of rash (most strongly indicated by Gottron's papules) at 3 months was the earliest predictor of a longer time to remission (relative risk [RR] 0.55 [95% confidence interval (95% CI) 0.37-0.81], P = 0.002). At 6 months, the presence of nailfold abnormalities and rash also predicted a longer time to remission (RR 0.35 [95% CI 0.14-0.74], P = 0.003). We were unable to determine a prediction model of disease course. The majority of patients in our cohort had a chronic disease course. The persistence of Gottron's papules and nailfold abnormalities early in the disease course was associated with a longer time to remission.

Dermatomyositis Developing in the first Trimester of Pregnancy

Dermatomyositis in pregnancy is rare. Pregnancy may be a precipitating factor at the onset or may develop during the course of dermatomyositis, which would exacerbate disease activity. In this study, we report a 22-year-old female patient who developed generalized skin rash and progressive muscle weakness in the twelfth week of pregnancy. She was diagnosed with dermatomyositis and underwent therapeutic abortion, due to the high fetal mortality rate of the disease when developed in the first trimester. Her symptoms improved with treatment of intravenous immunoglobulin and a high dose of corticosteroids.

TNFalpha-308A allele in juvenile dermatomyositis: association with increased production of tumor necrosis factor alpha, disease duration, and pathologic calcifications.

To characterize the association between the TNFalpha-308A allele and 1) duration of active disease, 2) peripheral blood mononuclear cell (PBMC) synthesis of tumor necrosis factor alpha (TNFalpha) in vitro, and 3) pathologic calcifications in patients with juvenile dermatomyositis (DM). The TNFalpha-308 alleles were determined by polymerase chain reaction in 37 white patients with juvenile DM and in 29 control subjects. Patients were grouped according to duration of immunosuppressive therapy: long (> or =36 months) or short (<36 months). Unstimulated PBMC were examined by enzyme-linked immunosorbent assay for TNFalpha production in vitro. Sixty-five white patients with juvenile DM were examined for pathologic calcifications. TNFalpha-308A was identified in 18 of 37 patients with juvenile DM, in contrast with 5 of 29 controls (P = 0.009). Sixteen of the 18 patients with juvenile DM who had the TNFalpha-308A allele had a disease course > or =36 months, compared with 6 of 19 patients with TNFalpha-308G (P = 0.001). PBMC from 16 of the 18 juvenile DM patients with TNFalpha-308A synthesized more TNFalpha (median 53 pg/ml) compared with PBMC from 9 of 19 patients with TNFalpha-308G (median 19 pg/ml) (P = 0.007). Nineteen of 22 juvenile DM patients requiring therapy for > or =36 months produced more TNFalpha (median 20.5 pg/ml) in comparison with 6 of 15 juvenile DM patients with a <36-month treatment course (median TNFalpha 0.0 pg/ml) (P = 0.005). Detectable calcifications were present in 3 of 8 children with juvenile DM who had TNFalpha-308AA, compared with 2 of 21 children with TNFalpha-308AG and 1 of 36 children who had TNFalpha-308GG (P = 0.017). A long course of juvenile DM and the presence of pathologic calcifications were associated with the TNFalpha-308A allele and with the increased production of TNFalpha, which may perpetuate the inflammatory response.

Recurrent Breast Cancer at 21 years after Resection Detected by Serum Tumor Markers and Manifested as Dermatomyositis.

A 52-year-old Japanese woman developed dermatomyositis. She had undergone a standard radical mastectomy for left breast cancer 21 years earlier. Though no physical sign of recurrent breast cancer appeared clinically, levels of tumor markers were abnormally elevated. Therefore, tamoxifen and CAF therapy were given. Further, the clinical course of dermatomyositis almost paralleled the level of serum tumor markers and the clinical course of her recurrent breast cancer. These markers were useful for detecting the recurrence, following the metastatic disease, and monitoring her response to therapy.

Breast cancer and second primary ovarian cancer in dermatomyositis

We report six female patients with breast cancer who developed dermatomyositis and compare our data with those from other reports. The development of dermatomyositis in two patients led to the discovery of a second primary ovarian carcinoma, whereas the development of dermatomyositis in another two patients led to the discovery of recurrent breast cancer. In three patients the diagnosis of dermatomyositis preceded the diagnosis of breast cancer, while the rest developed dermatomyositis after the diagnosis of breast cancer. A parallel clinical course of dermatomyositis and breast cancer was seen in only one patient. Coexisting dermatomyositis and breast cancer is a rare phenomenon, and dermatomyositis that develops during the course of breast cancer may indicate the occurrence of a second primary malignancy or recurrent breast cancer. The onset of dermatomyositis may precede, coincide with, or follow the diagnosis of breast cancer. The clinical course of dermatomyositis sometimes, but not always, parallels the course of breast cancer. There are no specific clinical or laboratory markers to distinguish patients with dermatomyositis who have malignancy from those without cancer.