Heliotrope Rash Research Articles

An extragonadal germ cell tumor with dermatomyositis: A case report and literature review.

The risk of malignancy in inflammatory myopathy patients is well recognized. However, the incidence of germ cell tumor (GCT) with inflammatory myopathy is low, and most reported cases of GCT also exhibit testicular tumors. Therefore, a case of extragonadal GCT with dermatomyositis (DM) is reported in the current study to better understand this paraneoplastic syndrome. A 53-year-old man presented with bilateral cervical lymph node enlargement. A lymph node biopsy showed embryonal carcinoma, and computed tomography showed multiple lymph node and lung metastases. A period of one month after bleomycin, etoposide and cisplatin (BEP) chemotherapy, this patient developed an erythematous eruption over the extensor surfaces of bilateral fingers, or Gottron's sign and facial erythema. The patient was diagnosed with DM with a positive anti-TIF-1γ-antibody result. High-dose prednisolone was effective, and there has been no evidence of cancer recurrence for over one year. The literature review identified 17 cases of GCT with inflammatory myopathy that have been reported so far, and it was indicated that this is the first case of extragonadal GCT with DM following chemotherapy. This case highlights the importance of monitoring after the completion of cancer treatment, as distinctive dermal and muscular symptoms should cause us to consider the possibility of paraneoplastic inflammatory myopathy.

The sunburn sign and the suntan sign- two novel findings in Hispanic patients with dermatomyositis.

Dermatomyositis is defined by characteristic skin features and proximal muscle weakness. Skin manifestations are crucial for diagnosing dermatomyositis.1,2 Common cutaneous manifestations include heliotrope rash edema, poikiloderma and erythema which is frequently observed in the malar area.3-5 We described two novel facial skin signs in a case series of Hispanic patients with dermatomyositis.

Malignancy in dermatomyositis: A retrospective paired case-control study of 202 patients from Central China.

Dermatomyositis (DM) is an idiopathic inflammatory myopathy that is closely related to malignant diseases. Our study aims to investigate the incidence and predictive factors for occurrence of malignancy among DM patients from Central China.We performed a retrospective, paired, case–control study of 736 DM patients admitted to the First Affiliated Hospital of Zhengzhou University between 2010 and 2017. We paired the 65 patients with malignancy with age-matched and sex-matched patients without malignancy in a ratio of 1:2. Two hundred two patients were finally enrolled and their clinical and laboratory data were collected.The incidence of malignancy in DM patients was 8.83% (65/736). Most malignancies were detected in the most recent 1 year before (9/65, 13.85%) or within 3 years after (40/65, 61.54%) the onset of DM. Males (35/65, 53.85%) and patients aged between 50 and 69 years (43/65, 66.15%) were prone to develop malignancies. Lung cancer (n = 11, 31.43%) was the most common malignancy in male patients, while for females, thyroid, breast and cervical cancer (n = 4 each, 13.33%) were more prevalent. Adenocarcinoma and squamous cell carcinoma (both 18/65, 27.69%) were the top two most common pathological types. Univariate analysis demonstrated that Gottron's sign (P = .02), dysphagia (P = .04), albumin (ALB) reduction (P = .003), aspartate aminotransferase (AST, P = .03), creatine kinase-MB (P = .02), absence of fever (P = .02), arthralgia (P = .04) and interstitial lung disease (ILD, P = .05) were closely related to the occurrence of malignancy. Multivariate analysis revealed the independent risk factors of ALB reduction (odds ratio = 1.546, P = .04) and the protective factor of ILD (odds ratio = 0.349, P = .003). There was no significant difference in the follow-up period between patients with and without ILD (P = .38).ALB reduction and the absence of ILD were the risk factors for malignancy in DM patients. The protective mechanism of ILD for DM patients needs further study.

External validation of the EULAR/ACR idiopathic inflammatory myopathies classification criteria witha Japanese paediatric cohort.

To assess the performance of the EULAR/ACR idiopathic inflammatory myopathies (IIMs) classification criteria to classify juvenile IIMs (JIIMs) in an Asian paediatric population. Sixty-eight JIIM patients and 49 non-JIIM patients diagnosed at seven major paediatric rheumatology centres in Japan between 2008 and 2015 were enrolled. Retrospective data were collected, and each patient's data form was submitted. The expert group reviewed the forms and re-examined the diagnoses. The EULAR/ACR criteria were then applied and the probability of having JIIM was determined for each case. The sensitivity and specificity of the EULAR/ACR criteria were compared with those of other existing criteria. The sensitivity/specificity of the EULAR/ACR classification criteria were 92.1/100% with muscle biopsy data (n = 38); 86.7/100% without muscle biopsy data (n = 30) and 89.7/100% in our total cohort (n = 68). The sensitivity of Bohan and Peter's criteria and Tanimoto's criteria were 80.9 and 64.7% in our total cohort, respectively. Among 68 physician-diagnosed JIIM patients, seven cases (three JDM and four overlap myositis) were not classified as JIIM because the probability did not reach the cut-off point (55%). The three JDM patients all presented with only one of the three skin manifestations that are listed in the criteria: Gottron's sign. Our validation study with Japanese JIIM cases indicates that the EULAR/ACR classification criteria for IIM generally perform better than existing diagnostic criteria for myositis.

Clinical features of anti-transcription intermediary factor 1γ (TIF1γ)-positive dermatomyositis with internal malignancy and investigation of the involvement of TIF1γ expression in tumors in the pathogenesis of cancer-associated dermatomyositis.

Anti-transcription intermediary factor 1γ (anti-TIF1γ) antibody (Ab) is significantly associated with internal malignancies in adult patients with dermatomyositis (DM). Although pathogenesis of cancer-associated DM is unknown, TIF1γ overexpression in tumors has been considered to be critical for the development of DM. The objective of this study was to investigate clinical characteristics of patients with anti-TIF1γ Ab-positive DM and elucidate risk factors that are potentially associated with internal malignancy. In addition, we compared the expression of TIF1γ in tumor tissues of patients with anti-TIF1γ Ab-positive DM, anti-TIF1γ Ab-negative DM and without DM in order to investigate the pathogenesis of cancer-associated DM. We analyzed 77 Japanese patients with DM, and found 19 patients to be positive for anti-TIF1γ Ab. Patients with anti-TIF1γ Ab-positive DM were older and presented heliotrope rash and flagellate erythema more frequently than patients without anti-TIF1γ Ab (P<0.05). Interstitial lung disease (ILD) and rapidly progressive ILD, as well as palmar violaceous erythema, were less frequent in patients with anti-TIF1γ Ab than in patients without. Furthermore, internal malignancy and dysphagia were significantly more frequent in the anti-TIF1γ Ab-positive group (P<0.01). Male sex and dysphagia were significantly associated with internal malignancy in patients with anti-TIF1γ Ab-positive DM (P<0.01 and <0.05, respectively). Using immunohistochemistry, we examined the TIF1γ expression in tumors of 11 patients with cancer-associated DM (anti-TIF1γ Ab-positive, nine; anti-TIF1γ Ab-negative, two) and 25 patients without DM. TIF1γ was highly expressed in all tumors, and there was no significant difference in TIF1γ expression between patients with and without DM. Furthermore, TIF1γ expressions in tumors were similar irrespective of the presence of anti-TIF1γ Ab. These results suggest that anti-TIF1γ antibody may not be simply induced by overexpression of TIF1γ in tumors in patients with DM, but that other mechanisms may exist.

The Diagnosis is Obvious

Clinically amyopathic dermatomyositis (CADM) is a rare entity of dermatomyositis. As a rule, CADM presents without muscular involvement. Thus, the level of creatine kinase is most commonly within the normal range. Dermal manifestations of CADM are Gottron's papules and mechanic's hands. In the case of melanoma differentiation-associated gene 5 intracellular pathogen sensor (MDa5 antibodies), CADM is often associated with a rapidly progressive and severe form of interstitial lung disease. Pulmonary function tests reveal restriction and hypoxemia of varying degree. Features of ground-glass opacities, reticulations and consolidations are detected in high-resolution CT scan. Lymphocytes are sometimes predominant in bronchioloalveolar lavage. Pathologists see a picture similar to non-specific interstitial lung disease or organizing pneumonia. Pronounced immunosuppression is the most common therapy. However, sometimes a combination of different immunosuppressive therapies is necessary. A novel strategy to treat CADM with rapidly progressive interstitial lung disease is the Janus kinase inhibitor tofacitinib. Treatment can be monitored with the level of ferritin and MDa5-antibody titer. Mortality is as high as 84 %.

Dermatomyosite de l’enfant. Série descriptive de 22 cas

To report on the characteristics of juvenile dermatomyositis (JDM). This was a retrospective, descriptive, cross-sectional, non-interventional, multicenter study conducted in Alsace between 2000and 2015. The patients, aged 0to 16years, had JDM according to both the Bohan and Peter and the EULAR/ACR criteria. A total of 17girls and 5boys were included with a median age at disease onset of 7,8years (Q1-Q3: 4.4-12.9). Median duration of JDM and median patient follow-up were 2.8years and 6.2years, respectively. The most common skin symptoms were papules or Gottron's sign (86%), nail lesions (82%), erythema of the face (77%) and eyelids (59%), photosensitivity (59%), and calcinosis (27%). One patient presented papules with a depressed and porcelain-white center ("Degos-like" lesions). One patient had algodystrophy. Two patients were clinically amyopathic. One girl had intestinal vasculitis. Respiratory function tests were abnormal in 27% of cases. Median treatment duration was 42months (Q1-Q3: 19-63). Three patients had a monocyclic form, 12had a polycyclic form, and 7had chronic disease. The frequency of cutaneous and musculoskeletal signs is comparable to that of other large cohorts of JDM. "Degos-like" lesions and algodystrophy have not yet been described in JDM. This study highlights the type and extent of the dermatological manifestations that frequently constitute the presenting complaint in this disease.

The polymorphous spectrum of dermatomyositis: classic features, newly described skin lesions, and rare variants.

Dermatomyositis belongs to a group of rare autoimmune diseases characterized by a variable degree of skin symptoms and myopathy. The clinically diagnostic hallmarks of dermatomyositis are heliotrope rash, Gottron's papules and weakness of the proximal muscles. Along with pathognomonic, characteristic, and compatible cutaneous features, several uncommon and rare skin manifestations have been reported. In addition, new skin lesions have been described in dermatomyositis patients. Furthermore, rare clinical subtypes of dermatomyositis have been reported in the literature, including Wong-type dermatomyositis, characterised by the coexistence of dermatomyositis and pityriasis rubra pilaris with hyperkeratotic, erythematous, follicular confluent papules on the back of the hands along the bony prominences. In addition, plenty of autoantibody subsets have been recently described that are related to distinct clinical features and systemic involvement, such as anti-MDA5 autoantibodies. We reviewed the English- and German-language scientific literature using the key words "dermatomyositis", "autoantibodies", and "clinical features", alone or in combination, focusing on particular cutaneous symptoms and their association with defined autoantibody profiles. Furthermore, we focused on rare subtypes of dermatomyositis, unusual clinical features, and recently described skin lesions.

AB0623 RATE AND PREDICTIVE FACTORS ASSOCIATED WITH SUSTAINED REMISSION IN IDIOPATHIC INFLAMMATORY MYOSITIS

Background:Idiopathic inflammatory myositis (IIM) is a group of heterogeneous connective tissue diseases, characterised by chronic muscle inflammation, myositis-specific or myositis-associated autoantibodies and different extra-muscular features. Achieveing low disease activity or remission in patients with IIM has proven to be difficult due to the wide clinical spectrum of the different IIM types.Objectives:To retrospectively assess any predictive factors for sustained remission in IIM patientsMethods:We retrospectively analyzed data taken from medical charts, which included age at disease onset, gender, laboratory data as well as clinical features present at onset, organ involvement and treatment history. A total of 151 adult patients with IIM followed-up for &gt; 1 year were retrospectively enrolled. Remission was defined as no clinical and laboratory evidence of disease activity persistent for more than 6 months during follow-up, while undergoing myositis therapy or under no medication. The remission of cutaneous involvement was defined as no current activity of skin rash, absence of Gottron’s papules as well as heliotrope rash and erythema, whereas the remission of pulmonary involvement was considered as no requirement for intensification of immunosuppressive therapy during follow-up. Likewise, absence of muscle weakness or hyposthenia was taken into account for evaluating muscle involvement. Moreover, the clinical features were accompanied by normalization of myogenic enzymes such as creatine kinase (CK) and lactate dehydrogenase levels.Results:Among all 151 patients, 89 (58.9%) patients achieved sustained remission. By univariate analysis, overlap myositis (7.9% vs 27.4%; p=0.003; OR:0.22), cancer-associated myositis (CAM) (7.8% vs 19.35%; p=0.046; OR:0.3), as well as the presence of anti Ku (3.37% vs 12.9%; p=0.05; OR:0.23) and anti TIF-1 gamma (1.1% vs 8%; p=0.043; OR:0.13) antibodies and polyarthritis (11.2% vs 24.19%; p=0.045; OR: 0.397) at onset were significantly associated with active IIM, not achieving remission.Out of 89 patients in remission, 79 (88.8%) achieved long-term sustained remission, lasting at least 2 years, whereas 10 patients (11.2%) showed relapse. Most of relapsed patients showed a relapse/remitting disease, with no evident trigger for the relapse. We divided our cohort of 89 patients in remission in 2 subsets: 83 patients in remission undergoing therapy (93.3%) and 6 patients in drug-free remission (6.7%). No significant differences were found between two groups, except for younger age at onset (p=0.0002) found in patients achieving drug-free remission.Conclusion:Sustained remission occurs in about one half of patients with IIM. The presence of anti Ku and anti TIF-1 gamma antibodies as well as polyarthritis at onset lowers the chance of achieving sustained remission. Younger age at diagnosis has proved to predict drug-free long-lasting remission.Disclosure of Interests:None declared

AB0610 SEASONAL VARIATION IN IDIOPATHIC INFLAMMATORY MYOPATHIES INCIDENCE AND PRESENTATION: A RETROSPECTIVE STUDY IN BEIJING AND HONG KONG

Background:Seasonal patterns of disease onset and severity in idiopathic inflammatory myopathies (IIMs) as a whole are conflicting [1-3]. In recent years, over 10 myositis-specific antibodies (MSAs) have been identified. They are able to divide patients into homogenous subgroups and inform on prognosis [4].Objectives:The objective of the study was to investigate the seasonal variation of onset of IIMs characterised serologically.Methods:This was a multi-centred retrospective observational study. Consecutive Chinese patients with IIMs admitted to the rheumatology wards of the participating major regional hospitals in Beijing and Hong Kong from July 2013 to June 2018 were recruited. The diagnosis of IIMs was based on the Bohan and Peter’s criteria with definite or probable cases being included [5]. Patients with clinically amyopathic disease must have the typical Gottron’s papules or heliotrope rash as determined by rheumatologists or dermatologists, and with no symptoms or signs of muscle involvement according to Sontheimer [6]. Patients with juvenile myositis, inclusion body myositis, cancer-associated myositis and myositis associated with other connective tissue disease were excluded. A commercial line blot immunoassay kit (EUROLINE) was used to detect the MSAs.Results:All together 495 patients were studied. The mean age of the patients at disease onset was 48.1 years (S.D. 13.3). There was a female predominance (68.3%). The subgroups of IIMs were: dermatomyositis (61.0%), polymyositis (21.8%), clinically amyopathic dermatomyositis (12.9%), immune mediated necrotising myopathy (3.8%) and nonspecific myositis (0.4%). No particular seasonal pattern in disease onset was observed in IIM patients as a whole (Figure 1) or in any classical subgroups. However, significantly more patients with any one MSA had their disease started in the first half of the year (p=0.007) as shown in Figure 2. Patients with either anti-synthetase or anti-MDA5 antibodies, which are associated with interstitial lung disease, had more frequent disease onset from November to February, which might coincide with the local flu season. It was also found that MSA positivity was associated with infection of the patient (p=0.005). Further analyses showed that patients with MSAs which are typically associated with severe skin disease (MDA5, TIF1g, NXP2, SAE) had more hospitalisation from April to September where excessive sun exposure is expected. There were no major differences between the Beijing and Hong Kong subgroups.Conclusion:Apparent seasonal patterns were noticed in our ethno-serologically defined IIM patients. Certain environmental factors, particularly infection or UV exposure, could be potential triggers. Our findings could shed light on the identification of etiologic factors and enhance our understanding of disease pathogenesis.

Amyopathic and anti-TIF1 gamma-positive dermatomyositis: analysis of a monocentric cohort and proposal to update diagnostic criteria.

Dermatomyositis (DM) is a group of autoimmune diseases characterized by a variable degree of skin symptoms and myopathy. An amyopathic form of DM (ADM) has been described, and more recently, an anti-TIF-1 gamma-positive subtype, characterized by poikiloderma and associated with a relatively high risk of cancer. To characterise a cohort of DM patients. A cohort of 29 DM patients was followed between January 2004 and March 2019, and investigated for clinical characteristics, pathological features based on electromyography and MRI, laboratory data, and auto-antibody profile. Based on the investigations, DM was shown to be heterogeneous. However, we identified a subgroup of anti-TIF-1 gamma-positive patients who all shared heliotrope erythema. Furthermore, we observed a positive correlation between serum glutamicoxaloacetic transaminase (GOT) and creatine kinase (CK) concentrations in patients with anti-TIF-1 gamma antibodies, which is not found in patients with anti-MDA-5 antibodies. Based on the findings of this study, we propose an update of the Sontheimer et al. diagnostic criteria to improve the sensitivity of diagnosis for ADM. In addition, we describe a significant association between serum GOT and CK levels in DM patients with anti-TIF-1 gamma antibodies, and further highlight the significance of heliotrope rash as a clinical hallmark for this particular subset of patients.

Dermatomyositis-Like Multicentric Reticulohistiocytosis: A Clinical Clue to Verify Underlying Malignancy

Herein, an otherwise healthy 63-year-old Caucasian woman with a three month-history of progressive symmetrical swelling and of the fingers, lumbal and abdominal pain, proximal muscle weakness, and heliotropic rash resembling V- neck, histologically verified as multicentric reticulohistiocytosis (MRH), is presented. Multiple computer tomography scans failed to detect ovarian cancer despite elevated Ca 125, however, it was verified six months later by magnetic resonance investigation. Multicentric reticulohistocytosis (MRH) is a rare systematic disease that targets skin, mucoses and synovium, resulting in skin nodules, mucosal lesions and deforming painful mutilating polyarthritis. Twenty – five percent of all patients are associated with internal malignancies, hence, MRH paraneoplastic nature remains extremely controversial. Our case showed a peculiar dermatomyositis-like clinical subtype, which we consider suggestive for verification of an underlying malignancy.

P50 Gossypiboma causing SLE and dermatomyositis overlap

Abstract Background Gossypiboma is a term referring to tumours arising from a retained, non-absorbable cotton matrix left behind during surgery. Serious implications for patients include infections, migration of the retained particles into a systemic circulation, local obstructions, as well as autoimmune reactions. To our knowledge, we describe the first case of SLE-dermatomyositis overlap associated with the gossypiboma. Methods A 44 year old lady, who is originally from Poland, presented with a few months history of oral ulcers, photosensitive rash over her chest and neck, myalgia, heliotrope rash and subcutaneous calcified nodules. Her ANA screen was positive 1:100 homogenous pattern, ENA, DsDNA negative, normal complements, CK up to 1636 u/L, nailfold capillaries demonstrated dilated capillaries and capillary dropouts. She was initially managed by a hydroxychloroquine and methotrexate combination for a presumed lupus and dermatomyositis overlap, followed by rituximab for recurrent lupus flare ups. The left knee MRI revealed a well-defined lesion medial to the tibia measuring 4.5 x 2.8 x 2.2 cm. It was heterogenous and contained calcium depositions. The initial diagnosis was in favour of peripheral nerve sheath tumour (schwannoma). However, surgical resection and histological analysis revealed foreign body sponge like material consistent with a retained surgical sponge. On revisiting the past medical history, the patient recalled some form of an operation on her left knee, evidenced by a small scar over posterior aspect of the knee, around twenty-five years ago. A subsequent operation was undertaken to remove the gossypiboma. After this, the patient’s symptoms had gradually improved and ANA screen came back as negative, and she was able to cease steroid therapy. Results Immune type of reactions secondary to adjuvants (silicone, pristane, aluminium, vaccines, etc) have been described by Israeli immunologist Shoenfeld and named ASIA syndrome (Autoimmune Syndrome Induced by Adjuvants) in 2011. He described late-onset (sometimes after many years) systemic symptoms associated with the immune reaction following silicone breast implantation. As per his theory, adjuvants influence both adaptive and innate arm of the immune system via different mechanisms, especially in genetically predisposed individuals. The great variety of manifestations have been described, among which Sjögren’s and lupus-like syndromes. We believe that our patient developed autoimmune disease secondary to constant antigenic stimulation caused by a retained cotton wool in the knee. The fact that the symptoms have improved after the removal of the foreign body combined with the negative ANA test is the evidence of the above-mentioned causality. Conclusion In summary, we believe that the presence of the foreign body in our patient’s knee has propagated the foreign body reactions and lead to an autoimmune disease. Disclosures L. Borukhson None. C. Wing None. R.J. Stratton None.

Inverse Gottron's sign in anti-MDA5 antibody-associated dermatomyositis.

Inverse Gottron's sign in anti-MDA5 antibody-associated dermatomyositis.

Myositis Specific Autoantibodies: A Clinical Perspective.

Dermatomyositis (DM) is an idiopathic inflammatory condition characterized by myositis and variable skin manifestation. The existence of myositis specific autoantibodies usually manifests with varying degrees of skin or muscle inflammations. The condition has a well-established association with most clinical phenotypes, and these autoantibodies are useful in informing the diagnosis, management and prognosis of the disease. DM-specific autoantibodies include anti-MDA5, anti-NXP2, anti-SAE, anti-Mi-2, anti-ARS, anti-TIF1-gamma. Anti-Mi-2 antibodies are widely associated with DM cases that exhibit mainly cutaneous symptoms, such as cuticular overgrowths, Gottron’s papules while being less susceptible to complications like interstitial lung disease or malignancy. The most distinct clinical features of patients with anti-SAE antibodies are their high prevalence of dysphagia and cutaneous manifestations that antecede the development of myopathies. In addition, DM patients with positive anti-PL-7 antibodies tend to have milder myositis characterized by low levels of creatine kinase as compared to patients with positive anti-Jo-1 antibodies. The anti-NXP2 antibodies are associated with transcriptional regulation and production of various proteins targeted by other DM antibodies, while anti- TIF1-γ. facilitates the transcription of deoxyribonucleic acids and regulates the growth and subsequent differentiation of body cells by controlling the signaling of TGF-β. The present review targets DM specific autoantibodies, considering their association, significance, and clinical presentation

Dermatomyositis combined with IgA vasculitis: A case report

Dermatomyositis (DM) is an autoimmune disease characterized by muscle involvement of the proximal extremities and specific skin involvement, like Gottron sign and heliotrope rash. HenochSchonlein purpura (IgA vasculitis) nephritis is characterized by hematuria and/or proteinuria clinically, with histologic evidence of IgA nephropathy, and also can be clinically characterized by non-thrombocytopenic purpura, presenting with petechiae and ecchymosis on the skin and mucous membranes, often involving multiple organs and systems, accompanied by abdominal pain, joint swelling and pain, and renal lesions. We reported here a patient with symmetric muscle weakness in her proximal limbs and typical Gottron sign, whose laboratory examination showed elevated creatine kinase (CK) level and myogenic damage electromyographically, which were concomitant with dermatomyositis. We applied prednisone combined with cyclophosphamide, and the patient's muscle strength, interstitial lung disease and all improved gradually. The patient gradually developed severe hepatic damage [significantly increased glutamic-pyruvic transaminase (ALT), glutamic oxalacetic transaminase (AST) and bilirubin], high fever (body temperature fluctuated between 38.0-39.2 °C), thrombocytopenia (limb distal purplish rash, some slightly protruded from the skin surface, some fused into a piece, which did not fade with pressure) and intractable diarrhea (waterlike stool, antidiarrheal drug treatment was not good), with new onset of the skin lesions on multiple areas of her body, as well as abrupt occurrence of massive proteinuria, which resulted in huge challenges in the following diagnosis and treatment. After extensive differential diagnosis from various directions, including pathological biopsies, it finally came out to be dermatomyositis combined with IgA vasculitis, which had been rarely reported. Both cellmediated immunity to muscle antigens and immune-complex disease might participate in the pathogenesis. There was evidence that they were immune complex diseases. Several immune mechanisms played an important role in the pathogenesis of both DM and IgA vasculitis. We conducted a substantial literature review of the above diseases. The purpose of our study is to strengthen the clinical understanding of such complicated diseases, and to highlight the importance of pathological biopsy in the diagnosis (renal biopsy pathology gave us a definite diagnosis). And what is more important is that seizing the opportunity to initiate treatment can control the disease and improve the prognosis.

Description of a dermatomyositis in a cohort of Colombian patients

Dermatomyositis is a rare autoimmune disease with primary involvement of skin, muscle and different organs. It is important to rule out overlap syndromes and hidden neoplasms. There is not enough data in Colombia regarding of this disease. To describe demographics, clinical characteristics, associations and treatment used in a cohort of dermatomyositis patients from Colombia and compare them with foreign cohorts. A retrospective descriptive study was performed with records from january 2016 and to june 2019 of patients from the Deparment of Rheumatology and Neurology of a teaching hospital in Bogotá,Colombia. 32 patients were included in this study.Demographics, clinical features and paraclinicals are summarized on Tables 1 and 2. It was found a higher prevalence in females, with the median age of 49.The amyopathic pattern was less frequent,however, an study in Singapore has demostrated that this subphenotype is more common than thought previously. In our study all patients had pathognomonic skin lesions, the most frequent were gottron's papules(75%), similar results were found in a study in China.The antisynthetase syndrome(12%) was less frequently compared to other studies where it is estimated approximately 30%, and neoplasms were higher when compared with 14% found in a systematic review. The most used treatment was steroids, as in other studies. We found similar characteristics between a cohort of Colombian patients with dermatomyositis and foreign cohorts reported on the literature. Amiopathic pattern and antisynthetase syndrome were less frequent. Hidden neoplasms were more frequent when compared to European, Asian and American cohorts.

Clinical features and poor prognostic factors of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis with rapid progressive interstitial lung disease.

Anti-melanoma differentiation-associated gene5 (MDA5) antibody has been recognized to be significantly associated with a subset of dermatomyositis patients with rapidly progressive interstitial lung disease (RP-ILD). To elucidate the clinical characteristics and poor prognostic factors in Japanese dermatomyositis patients with anti-MDA5 antibody. Clinical features of anti-MDA5 antibody-positive dermatomyositis patients and risk factors, potentially associated with a poor prognosis, were retrospectively analysed. A total of 37.3% (28/75) dermatomyositis patients were positive for anti-MDA5 antibody. The frequency of Gottron's papules, palmar violaceous macules, antihelix/helix violaceous macules, and clinically amyopathic dermatomyositis (CADM) was significantly higher in patients with anti-MDA5 antibody. Of anti-MDA5 antibody-positive dermatomyositis patients, 57.1% developed RP-ILD, and in those with RP-ILD, serum ferritin level was markedly high and partial pressure of arterial oxygen (PaO2) was significantly low at first visit. Among patients with anti-MDA5 antibody with RP-ILD, non-survivors were older and revealed lower PaO2 at first visit relative to survivors. Furthermore, patients who did not take triple therapy (prednisolone, calcineurin inhibitor and cyclophosphamide) as initial treatment resulted in poor outcome. Anti-MDA5 antibody may be associated with CADM, the progression to RP-ILD, high serum ferritin level, and characteristic skin manifestations. High serum ferritin level in patients with anti-MDA5 antibody may be associated with the development of RP-ILD and poor prognosis. Early treatment with triple therapy, including intravenous cyclophosphamide, may improve the prognosis of RP-ILD.

SMALL CELL LUNG CARCINOMA WITH THREE PARANEOPLASTIC SYNDROMES IN ONE PATIENT

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: This is a case of patient who was diagnosed with Small Cell Lung Carcinoma(SCLC)after presenting with symptoms of Dermatomyositis(DM), SIADH and Lambert–Eaton myasthenic(LEMS)syndrome CASE PRESENTATION: Patient initially presented to the clinic with erythematous patches scattered on the trunk, extremities, and scalp associated with pain, itchiness and hair loss for two months. Biopsy from rash showed perifollicular and mild superficial perivascular inflammation. The patient was treated for viral exanthem with steroids. Patient subsequently presented to the hospital with worsening pruritic patches on scalp and entire body and periorbital edema. This was associated with weakness and generalized malaise. The weakness and pain were worse with neck and shoulder movements and the weakness improved on exertion. Other symptoms were productive cough, rhinorrhea, nasal congestion and sore throat for a month associated with 20 pounds weight loss. Physical exam showed decreased breath sounds in the left upper and middle field.No lower extremity swelling.Skin findings were violaceous edematous periorbital erythema(heliotrope rash), erythema of neck, upper chest, and entire back, erythematous slightly scaly plaques overlying MCPs and PIPs(Gottron's papules). Labs showed CPK of 868, Aldolase of 8.9, serum sodium of 115(Serum osmolality 248, Urine osmolality 497, TSH 1.7, random cortisol 20.7)and ANA(1:320)was positive. Chest x-ray showed left upper lobe mass. CT chest showed malignant process arising from the left upper lobe and to the mediastinum. She underwent bronchoscopy with FNA and endobronchial biopsy which showed SCLC. DISCUSSION: Paraneoplastic syndromes occur in approximately 10% of patients with lung cancer(1). SIADH occurs in 15% of SCLC patients. LEMS is present in approximately 1% to 3% of patients with SCLC. Off all cases of DM only 5.9% have lung cancer. In some cases these syndromes can be presenting sign of cancer. The early recognition of paraneoplastic syndromes may contribute to the detection of a highly treatable, early-stage tumor. At other times, the syndromes may occur late in the course of disease or may appear as the first sign of recurrence DM is associated with poor prognosis and improved SCLC tumor survival are seen in patients with LEMS(2). Management of hyponatremia is very important as it is associated with a poor prognosis regardless of an extensive or limited stage. SCLC patients with serum sodium less than 129 mmol/l had a median survival of 8.63 months compared to 13.6 months in patients with normal sodium, and the degree of hyponatremia is a significant predictor for prognosis(3). CONCLUSIONS: SCLC is associated with large number of paraneoplastic syndromes which can also be the initial presentation in some cases. Ability to identify these syndromes especially in high risk population can lead to early diagnosis and might carry a better prognosis in some patients. Reference #1: Kanaji N, Watanabe N, Kita N, et al. Paraneoplastic syndromes associated with lung cancer. World J Clin Oncol. 2014;5(3):197-223 Reference #2: Maddison P, Gozzard P, Grainge MJ, Lang B. Long-term survival in paraneoplastic Lambert-Eaton myasthenic syndrome. Neurology. 2017 Apr 4. 88 (14):1334-1339 Reference #3: Wang W., Song Z., Zhang Y. Hyponatremia in small cell lung cancer is associated with a poorer prognosis. Translational Cancer Research. 2016;5(1):36–43. https://doi.org/10.3978/j.issn.2218-676X.2016.01.04 DISCLOSURES: No relevant relationships by alaa abu-sayf, source=Web Response No relevant relationships by Nitesh Gandhi, source=Web Response No relevant relationships by Sam Tirgari, source=Web Response

Dermatomiosite como Primeiro Sinal de Neoplasia

Os autores apresentam o caso de um homem de 69 anos, que dá entrada no serviço de urgência por diminuição da força muscular proximal com um mês de evolução. Apresentava ainda lesões cutâneas pruriginosas disseminadas no tronco, com o sinal V, rash heliotrópico e erupção cutânea no dorso, face e membros superiores, tendo sido feito o diagnóstico de dermatomiosite. Do estudo analítico efetuado verificou-se anticorpos anti-nucleares positivos, elevação de creatina quinase, lactato desidrogenase e aldolase. A biópsia cutânea confirmou o diagnóstico. Do exame físico foi constatada uma massa no quadrante superior do abdómen e considerando poder tratar-se de síndrome araneoplásica realizou ecografia, tomografia computorizada abdominal e ressonância magnética abdominal com identificação de massa única hepática com 12x8x7 cm. A biópsia desta lesão foi compatível com colangiocarcinoma. O doente faleceu de hemorragia intraparenquimatosa na região lenticocapsular externa, sem causa identificável. Com este caso pretende-se chamar a atenção da associação entre a dermatopolimiosite e tumores sólidos.