Involvement In Dermatomyositis Research Articles

Trichoscopic features in scalp dermatomyositis

Scalp involvement in Dermatomyositis (DM) represents a diagnostic challenge. Its clinical presentation is often atypical, and can be observed in other collagenosis. In this report, we outline the trichoscopic signs of two cases of DM of the Scalp (SDM).

Increased serum soluble interleukin-2 receptor levels in dermatomyositis are associated with Th17/Treg immune imbalance.

Dermatomyositis (DM) represents a multifaceted chronic inflammatory myopathy, primarily manifesting as progressive deterioration of muscular and cutaneous tissues. Despite an incomplete comprehension of DM's etiology and pathogenesis, current evidence implicates the involvement of T lymphocyte infiltration, extensive cytokine release, myositis-specific antibodies, and myositis-associated antibodies in disease development. Serum soluble interleukin-2 receptor (sIL-2R) frequently serves as a marker for T cell activation; however, its role remains elusive. Consequently, this investigation sought to elucidate the association between sIL-2R levels, peripheral blood lymphocyte subset counts, and related cytokines in DM patients, with the aim of uncovering the intricate mechanisms underlying DM and establishing a theoretical foundation for the implementation of precise, targeted, individualized immunomodulatory therapy. In this study, a cohort of 60 dermatomyositis (DM) patients, comprising 32 with inactive DM and 28 with active DM, was enrolled and stratified into inactive and active groups based on the Myositis Disease Activity Visual Analogue Scale (MYOACT). Flow cytometry was employed to quantify the absolute counts of peripheral lymphocyte subsets and CD4+T cell subsets in each group, while a flow cytometry bead array was utilized to measure serum cytokine levels. In a comparative analysis between healthy individuals and patients diagnosed with DM, we observed a marked elevation in serum sIL-2R concentrations (P < 0.001) and T-helper 17 cell/regulatory T cell (Th17/Treg) ratios (P < 0.01) within the latter group. A positive correlation was identified between serum sIL-2R levels and various parameters, including ESR, CRP, VAS, AST, CKMB, LDH, HBDH, PT, APTT, DDi, IL-6, IL-10, and IFN-γlevels (P < 0.05). In contrast, serum sIL-2R levels demonstrated a negative correlation with LY, HGB, ALB, Th17 cell populations, and Th17/Treg cell ratios (P < 0.05). Employing multivariate logistic regression, we identified serum sIL-2R concentrations as an independent risk factor for both disease activity and hepatic involvement in DM patients. Moreover, receiver operating characteristic (ROC) curve analyses revealed that serum sIL-2R levels significantly contributed to the differentiation of disease activity and the detection of liver involvement in DM patients, with areas under the ROC curve (AUC) of 0.757 (95% CI 0.630-0.884, P = 0.001) and 0.826 (95% CI 0.717-0.935, P < 0.001), respectively. This study highlights the potential utility of serum sIL-2R levels as a valuable biomarker for assessing disease activity and liver involvement in dermatomyositis. Elevated serum concentrations of sIL-2R were observed in patients with DM, exhibiting significant associations with Th17 cell populations and Th17/ Treg ratios. These findings indicate that sIL-2R may be implicated in the immunopathogenesis of DM, thereby warranting further investigation to elucidate its role in the disease process.

Gastrointestinal Involvement in Dermatomyositis.

Dermatomyositis is a systemic vasculopathy mainly affecting skin, muscle and lung, but may affect the gastrointestinal tract. We aim to describe clinical characteristics of patients with severe gastrointestinal involvement related to dermatomyositis in our center and medical literature. We retrospectively analysed these patients in our center, including cases of erosions/ulcers, perforation or digestive bleeding. Reported cases from April 1990 to April 2021 were reviewed through PubMed and Cochrane. From our cohort (n = 188), only 3 presented gastrointestinal compromise. All were women (10, 46 and 68 years). The initial symptom was abdominal pain and all had ≥2 episodes of digestive bleeding. All died due to complications of gastrointestinal involvement. Available pathological samples showed vascular ectasia. From the literature review (n = 50), 77% were women with a mean age of 49 years and the main symptom was abdominal pain (65%). All presented active muscular and cutaneous involvement at complication diagnosis. Mortality was 41.7%. The underlying lesion was perforation or ulcer (n = 22), intestinal wall thickening (n = 2), macroscopic inflammation (n = 2) or intestinal pneumatosis (n = 15). In 13 cases, vasculitis was described. Gastrointestinal involvement in dermatomyositis denotes severity, so an early intensive treatment is recommended. Pathological findings suggest that the underlying pathophysiological mechanism is a vasculopathy and not a true vasculitis.

Scalp involvement in Dermatomyositis

Scalp involvement is common in patients with dermatomyositis (DM), however it is an understudied manifestation that has been poorly described.

Scrotum involvement in dermatomyositis.

Scrotum involvement in dermatomyositis.

Altered peripheral helper T cells in peripheral blood and muscle tissue of the patients with dermatomyositis.

Peripheral helper T (Tph) cells, phenotypically PD-1hiCXCR5-CD4+, are a recently identified Th cell subset that relates to several autoimmune diseases. Contrary to PD-1hiCXCR5+CD4+ follicular helper T (Tfh) cells, Tph cells are not located in lymphoid organs but accumulate in inflamed tissues. This study investigated Tph cells to determine their involvement in dermatomyositis (DM). The frequency of circulating Tph and Tfh cells was evaluated by flow cytometry at baseline and after glucocorticoid treatment. The expression of Tph and B cells was determined in muscle tissue by immunohistochemistry (IHC). Further, the correlations between circulating Tph cells and clinical characteristics were investigated. Flow cytometry revealed that circulating Tph and Tfh cells were decreased in peripheral blood of DM patients compared with healthy controls (HCs). However, the muscular expression of Tph and B cells was upregulated in patients with DM compared to that in the controls by IHC. Interestingly, the increased B cells accumulated around Tph cells in infiltrated lesions. The frequency of circulating Tph cells was positively correlated with Tfh cells, CD3+ T cells, CD4+ T cells, and CD8+ T cells, whereas negatively correlated with erythrocyte sedimentation rate (ESR), interleukin (IL)-6, and IL-10 levels. Furthermore, the abnormal circulating Tph cells in peripheral blood were recovered after glucocorticoid treatment. These results indicate that Tph cells might be involved in the immunopathogenesis of DM and therefore might provide novel insight for the development of DM therapies.

Pattern of muscle involvement in dermatomyositis – A retrospective analysis using STIR MRI imaging

All patients with suspected Idiopathic Inflammatory Myopathy, over a 4 year period - from August 2014 to August 2018, seen in the departments of Neurology and Rheumatology of Aster Medcity, Kochi, India, were included in the retrospective analysis. 28 patients with DM were selected, who fulfilled the EULAR/ACR classification criteria. Of the 28 patients, 17 patients who had undergone fat suppression sequence, short tau inversion recovery (STIR) MRI muscle imaging were selected. The MRI images were collated and analysed by a single examiner who has over 4 years of experience with muscle MRI. Of the 17 patients with STIR imaging, 12 underwent a whole-body MRI and 5 underwent thigh muscle MRI only. We found that 76% patients were women, with a mean age of 48 yrs. 13 were adult DM, 3 were amyopathic DM and 1 case was a juvenile DM (9 years). The mean disease duration was 10.8 months, and the mean time to MRI from diagnosis and initiation of treatment was 4.7 months except in 3 cases who presented very late (1-2 years after onset). Most muscle changes were patchy, involving muscles asymmetrically. 70% cases showed involvement of lower limb muscles. And 30% showed upper limb and trunk muscle involvement. Paraspinal muscles and shoulder girdles were involved in 50% patients in whom whole-body MRI was performed. Interestingly, the muscles involved the most were the thigh (anterior and posterior compartment muscles), calf muscles, the glutei and pelvic muscles. The pelvic muscles were frequently involved (47%), especially obturator internus and externus.

Successful treatment of extensive calcifications and acute pulmonary involvement in dermatomyositis with the Janus-Kinase inhibitor tofacitinib – A report of two cases

IntroductionDermatomyositis (DM) can be complicated by calcinosis and interstitial lung disease (ILD). Calcinosis can be severely debilitating or life-threatening and to date there is no treatment with proven efficacy. In DM type I interferon contributes to pathophysiology by inducing the expression of proinflammatory cytokines and the JAK-STAT (signal transducer and activator of transcription) pathway may be involved in the regulation of mitochondrial calcium store release, a process potentially important for calcification in DM. JAK-inhibition may therefore be an attractive therapy in DM complicated by calcifications. Methods and resultsWe report on the fast and persistent response of extensive and rapidly progressive DM-associated calcifications in two patients treated with the JAK-inhibitor tofacitinib. During the 28-week observation period in both patients no new calcifications formed and existing calcifications were either regressive or stable. Furthermore, concomitant life-threatening DM-associated ILD (acute fibrinous and organizing pneumonia; AFOP) in one patient rapidly responded to tofacitinib monotherapy. Both patients were able to taper concomitant glucocorticoids. Tofacitinib was well tolerated and safe. ConclusionsThe results of our study support the role of JAK/STAT signaling in the development of calcinosis and ILD in DM. Tofacitinib may be an effective and safe treatment for calcinosis in DM and potentially for other connective tissue disease complicated by calcinosis.

Horizontal and vertical sections of scalp biopsy specimens from dermatomyositis patients with scalp involvement.

The histologic findings of scalp involvement in dermatomyositis are not well characterized on account of a lack of large series. To systematize the histologic features of scalp involvement in dermatomyositis on horizontal and vertical sections. A descriptive, prospective, cross-sectional study recruited 31 patients with pathologically and serologically confirmed dermatomyositis in Mexico City, Mexico, from June 2014 to June 2015. A total of 36 scalp biopsy specimens from 20 patients with scalp involvement in dermatomyositis were processed as 20 vertical and 16 horizontal sections. Dilated capillaries and diffuse mucin deposition were detected in all biopsy specimens, followed by interface dermatitis. Partial or segmental thickening of the basement membrane, hyperkeratosis, atrophic epidermis, and acrosyringeal hypergranulosis with hyperkeratosis were other very common findings. Preserved follicular architecture, with intact or slightly atrophic sebaceous glands, was present in most horizontal sections. There was decreased follicular density, with a terminal-to-vellus ratio of 4:1 and telogen count of 10.3%. Eosinophils were present in 15% and 25% of horizontal and vertical sections, respectively. No special stains performed. Scalp involvement in dermatomyositis shows nonscarring pattern on horizontal sections that is consistent with chronic telogen effluvium. Telangiectasia and mucin are universal histologic features; eosinophils and acrosyringeal hypergranulosis with hyperkeratosis are new findings.

Cardiovascular Magnetic Resonance Imaging of Myocardial Fibrosis in Dermatomyositis: Figure 1.

Cardiac involvement in dermatomyositis (DM) is infrequent but is associated with poor prognosis1. Myocardial fibrosis has been observed in histopathological studies of DM and has been attributed to myocardial inflammation2. Reports describing its noninvasive in vivo visualization in DM are lacking. …

Adult dermatomyositis with severe polyneuropathy: does neuromyositis exist?

Peripheral nerve involvement in dermatomyositis (DM) has been known as neuromyositis. However, the pathogenic mechanism is not clear, and the association between DM and peripheral neuropathy is still controversial. Our patient exhibited symptomatic polyneuropathy that was documented electrophysiologically in addition to typical features of DM. The sural nerve biopsy showed evidence of a continuing neuropathic process of axonal type. There was no finding of inflammatory cells infiltrating the vessels. Neither methylprednisolone nor intravenous immunoglobulin (IVIg) improved neurological symptoms including muscle weakness and sensory disturbance. Clinical, electrophysiological, and neuropathological features in our case demonstrate the association of DM and polyneuropathy. The possibility that the same pathological process affecting skin and skeletal muscles also affected peripheral nerves in our patient should be considered.

Staphylococcus Protein A‐based Extracorporeal Immunoadsorption and Thalidomide in the Treatment of Skin Manifestation of Dermatomyositis: A Case Report

Cutaneous involvement in dermatomyositis can be the prevalent component of disease, failing to respond to adequate therapies for myositis. In this case report, we describe a patient affected by dermatomyositis, characterized by prevalent skin involvement, successfully treated with Staphylococcus protein A-based extracorporeal immunoadsorption (Immunosorba, Fresenius Medical Care AG & Co. KGaA, Bad Homburg, Germany) and thalidomide. The patient showed panniculitic ulcerative lesions of the skin, difficult to treat because of side effects or ineffectiveness of various therapies. Skin manifestations rapidly improved after introduction of immunoadsorption; The association of thalidomide allowed a good maintenance of these results until the remission of skin lesions, despite several infective complications of some residual ulcers. Considering the difficulties in the management of our patient, combined therapy with Immunosorba and thalidomide has allowed a good clinical response. If confirmed, the observed beneficial effects suggest that protein A-based immunoadsorption and thalidomide can represent an alternative option in dermatomyositis, especially when cutaneous manifestations are predominant.

Dermatomyositis and supraventricular tachycardia

BackgroundDermatomyositis is an idiopathic inflammatory myopathy, often associated with an underlying malignancy. Its prevalence rate is approximately one per 100,000 in the general population, and is even rarer without evidence of a cancer. Dermatomyositis rarely involves myocardial muscle fibers, but has shown to be associated with cardiac arrhythmias.Case PresentationWe present a case of a young female patient with known history of dermatomyositis who presented to hospital with a flare up of her disease. She also complained of paroxysms of palpitation. Telemetry monitoring revealed several episodes of paroxysmal supraventricular tachycardia with heart rate reaching up to 220 beats per minute.ConclusionCardiac involvement in dermatomyositis is a very rare, but well known entity. Dermatomyositis patients with palpitations should be monitored on a Holter monitor, and appropriate therapy initiated if found to have a significant arrhythmia.

Successful treatment of cardiac involvement in dermatomyositis with rituximab

Polymyositis and dermatomyositis are autoimmune inflammatory myopathies characterized by muscle weakness and inflammation. Current recommended therapy includes corticosteroids as mainstay treatment in addition to immunosuppressant. We present herein a 25 year-old female with dermatomyositis and cardiac involvement resistant to disease modifying anti-rheumatic drugs and anti-tumor necrosis factor-α. She was treated with anti-CD20 monoclonal antibody, rituximab. The patient demonstrated a remarkable clinical and laboratory response. B-cell depletion therapy with rituximab may be a viable option in patients with dermatomyositis and heart disease.

Cyclosporine A as treatment of esophageal involvement in dermatomyositis

We report a case of a 70-year-old man with dermatomyositis complicated by esophageal involvement resistant to corticosteroid therapy. It had serious and potentially life-threatening implications, resulting in impossible oral feeding. This condition was successfully resolved by administration of cyclosporine A.

Successful treatment of refractory rash in paraneoplastic amyopathic dermatomyositis

Skin involvement in dermatomyositis does not always parallel muscle disease and can be the most active or severe component of the disease, failing to respond to therapeutic interventions that are adequate for myositis and other systemic involvement. This case refers to a resistant paraneoplastic amyopathic dermatomyositis rash which readily resolved with intravenous immunoglobulin (IVIG), practically in the form of monotherapy. This observation gains particular significance considering that IVIG is still regarded as a second-line therapeutic regimen in the treatment of dermatomyositis despite its prevalence in matters of safety.

Intramural ureteric calcification in dermatomyositis mimicking pelviureteric junction obstruction

Juvenile dermatomyositis classically manifests as necrotising vasculitis in multiple organs. It mainly involves the vessels of skin and muscle and is associated with subcutaneous deposits of calcium. Ureteric involvement in dermatomyositis is extremely rare. The authors present a case of a 14-year-old girl with juvenile dermatomyositis with intramural ureteric calcification mimicking pelviureteric junction (PUJ) obstruction.

Hyperdense pericardial effusion in dermatomyositis and contrast induced nephropathy

Pericardial involvement in dermatomyositis is rare and usually asymptomatic. In many instances, a diagnosis of pericardial involvement is not found until autopsy. Renal failure associated with connective tissue disorders can result in or potentially exaggerate pericardial inflammation. We report an unusual case of high-density pericardial effusion in a patient with dermatomyositis consequent upon contrast nephropathy as demonstrated by computed tomography. High-density pericardial effusion can be a result of an insidious cause such as pericardial inflammation rather than the more usual causes such as coronary or cardiac perforation.

Atteinte des muscles oculomoteurs révélant une dermatomyosite chez une patiente ayant une polyarthrite rhumatoïde

Introduction – Oculomotor muscles (OMM) involvement in dermatomyositis (DM) and in rheumatoid arthritis (RA) is unusual. The DM always leads to OMM inflammation, whereas the RA particularly leads to tenosynovitis of the superior oblique muscle referred to as the Brown syndrome. Observation – The patient is a 43-year-old woman who gives a 17-year-history of severe seropositive RA with bilateral coxite. She was hospitalized for acute painful proptosis. The clinical examination revealed an orbital erythema and a muscular rhizomelic weakness. The muscular enzymes were increased. The orbital CT revealed in the right side, an enlargement of the superior rectus muscle that was enhanced after intravenous injection, which is compatible with myositis involvement. The muscular biopsy practiced at the level of the calf showed the specific histological signs of the DM. This orbital involvement was resolved with a high dose of corticosteroids. Conclusion – Our observation has the specificity of associating RA with DM with an involvement of the superior rectus muscle, which is due to the DM rather than the RA.

P010 Skin involvement in dermatomyositis (DM)

P010 Skin involvement in dermatomyositis (DM)