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1.
Medicine (Baltimore) ; 99(37): e21943, 2020 Sep 11.
Artigo em Inglês | MEDLINE | ID: mdl-32925726

RESUMO

RATIONALE: Antimelanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab)-positive clinically amyopathic dermatomyositis (cADM) is frequently complicated with interstitial lung disease (ILD) and has a poor prognosis. Although the short-term prognosis of anti-MDA5 Ab-positive cADM is poor, it has been suggested that the recurrence rate is not higher than that of anti-MDA5 Ab-negative dermatomyositis. Combination therapy with corticosteroids, calcineurin inhibitors, and cyclophosphamide is the gold standard for the remission induction therapy at the onset. Recently, it has been reported that tofacitinib (TOF) could be effective for refractory anti-MDA5 Ab-positive cADM with ILD. Although initial remission induction therapy has been established, therapeutic strategies for relapse cases have not yet been established. PATIENT CONCERNS: A 57-year-old woman who was diagnosed with anti-MDA5 Ab-positive cADM complicated with ILD. In October 2016, she was treated with prednisolone (PSL), tacrolimus (TAC), and cyclophosphamide (CY). These treatments were successful, and PSL could be tapered. However, she developed strong nausea and general fatigue as adverse events of CY. In April 2018, PSL was discontinued, and maintenance therapy was given with TAC. In July 2018, Gottron's sign and ILD recurred. Skin lesions on the finger were partially ulcerated and ILD was also worsening. We proposed a remission reinduction therapy including CY. However, she was rejected CY from experience with past adverse event of CY. DIAGNOSIS: Based on skin lesions and chest computed tomography (CT) findings, the diagnosis was a recurrence of anti-MDA5 Ab-positive cADM with ILD. INTERVENTIONS: Treatment by TOF 10 mg and PSL 22.5 mg (0.5 mg/kg equivalent) was introduced in November 2018. OUTCOMES: After introducing TOF and PSL, her skin lesions and chest CT findings of ILD gradually improved. Six months after the induction of TOF, the skin ulcer was epithelialized. One year after the introduction of TOF, PSL was decreased to 9 mg, and the disease activity did not re-exacerbate. LESSONS: This case report is the first report suggesting the effectiveness of TOF for recurrent case of anti-MDA5 Ab-positive cADM with ILD. TOF might be an effective therapeutic option for treating recurrent case of anti-MDA5 Ab-positive cADM.


Assuntos
Autoanticorpos/sangue , Dermatomiosite/tratamento farmacológico , Helicase IFIH1 Induzida por Interferon/imunologia , Piperidinas/uso terapêutico , Inibidores de Proteínas Quinases/uso terapêutico , Pirimidinas/uso terapêutico , Pirróis/uso terapêutico , Dermatomiosite/sangue , Dermatomiosite/imunologia , Feminino , Humanos , Pessoa de Meia-Idade , Recidiva
2.
Clin Rheumatol ; 39(11): 3195-3204, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32852623

RESUMO

INTRODUCTION: Covid-19 infection poses a serious challenge for immune-compromised patients with inflammatory autoimmune systemic diseases. We investigated the clinical-epidemiological findings of 1641 autoimmune systemic disease Italian patients during the Covid-19 pandemic. METHOD: This observational multicenter study included 1641 unselected patients with autoimmune systemic diseases from three Italian geographical areas with different prevalence of Covid-19 [high in north (Emilia Romagna), medium in central (Tuscany), and low in south (Calabria)] by means of telephone 6-week survey. Covid-19 was classified as (1) definite diagnosis of Covid-19 disease: presence of symptomatic Covid-19 infection, confirmed by positive oral/nasopharyngeal swabs; (2) highly suspected Covid-19 disease: presence of highly suggestive symptoms, in absence of a swab test. RESULTS: A significantly higher prevalence of patients with definite diagnosis of Covid-19 disease, or with highly suspected Covid-19 disease, or both the conditions together, was observed in the whole autoimmune systemic disease series, compared to "Italian general population" (p = .030, p = .001, p = .000, respectively); and for definite + highly suspected diagnosis of Covid-19 disease, in patients with autoimmune systemic diseases of the three regions (p = .000, for all comparisons with the respective regional general population). Moreover, significantly higher prevalence of definite + highly suspected diagnosis of Covid-19 disease was found either in patients with various "connective tissue diseases" compared to "inflammatory arthritis group" (p < .000), or in patients without ongoing conventional synthetic disease-modifying anti-rheumatic drugs treatments (p = .011). CONCLUSIONS: The finding of a higher prevalence of Covid-19 in patients with autoimmune systemic diseases is particularly important, suggesting the need to develop valuable prevention/management strategies, and stimulates in-depth investigations to verify the possible interactions between Covid-19 infection and impaired immune-system of autoimmune systemic diseases. Key Points • Significantly higher prevalence of Covid-19 is observed in a large series of patients with autoimmune systemic diseases compared to the Italian general population, mainly due to patients' increased susceptibility to infections and favored by the high exposure to the virus at medical facilities before the restriction measures on individual movement. • The actual prevalence of Covid-19 in autoimmune systemic diseases may be underestimated, possibly due to the wide clinical overlapping between the two conditions, the generally mild Covid-19 disease manifestations, and the limited availability of virological testing. • Patients with "connective tissue diseases" show a significantly higher prevalence of Covid-19, possibly due to deeper immune-system impairment, with respect to "inflammatory arthritis group". • Covid-19 is more frequent in the subgroup of autoimmune systemic diseases patients without ongoing conventional synthetic disease-modifying anti-rheumatic drugs, mainly hydroxyl-chloroquine and methotrexate, which might play some protective role against the most harmful manifestations of Covid-19.


Assuntos
Doenças Autoimunes/epidemiologia , Infecções por Coronavirus/epidemiologia , Pneumonia Viral/epidemiologia , Doenças Reumáticas/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Antirreumáticos/uso terapêutico , Artrite Psoriásica/tratamento farmacológico , Artrite Psoriásica/epidemiologia , Artrite Psoriásica/fisiopatologia , Artrite Reumatoide/tratamento farmacológico , Artrite Reumatoide/epidemiologia , Artrite Reumatoide/fisiopatologia , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/fisiopatologia , Betacoronavirus , Infecções por Coronavirus/fisiopatologia , Dermatomiosite/tratamento farmacológico , Dermatomiosite/epidemiologia , Dermatomiosite/fisiopatologia , Feminino , Glucocorticoides/uso terapêutico , Humanos , Itália/epidemiologia , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/fisiopatologia , Masculino , Pessoa de Meia-Idade , Pandemias , Pneumonia Viral/fisiopatologia , Doenças Reumáticas/tratamento farmacológico , Doenças Reumáticas/fisiopatologia , Escleroderma Sistêmico/tratamento farmacológico , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/fisiopatologia , Síndrome de Sjogren/tratamento farmacológico , Síndrome de Sjogren/epidemiologia , Síndrome de Sjogren/fisiopatologia , Espondilite Anquilosante/tratamento farmacológico , Espondilite Anquilosante/epidemiologia , Espondilite Anquilosante/fisiopatologia , Doenças do Tecido Conjuntivo Indiferenciado/tratamento farmacológico , Doenças do Tecido Conjuntivo Indiferenciado/epidemiologia , Doenças do Tecido Conjuntivo Indiferenciado/fisiopatologia
3.
Mol Immunol ; 126: 95-100, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32795664

RESUMO

Although T cells are considered as the central component in immune-mediated diseases, supportive evidence has demonstrated that B cells also contribute to the progression of these diseases. B cells are divided into various subsets according to their secreted cytokines. Different B cell subsets play diverse roles in immune-mediated dermatoses. Regulatory B cells (Bregs) are defined functionally by their ability to secrete IL-10, which has been revealed to contribute to immunological tolerance. Drugs that deplete B cells, such as rituximab, are now used for the treatment of several immune-mediated dermatoses. In this review, we present and discuss the current knowledge on the roles of B cells in several immune-mediate dermatoses including psoriasis, pemphigus, bullous pemphigoid, and dermatomyositis, atopic dermatitis.


Assuntos
Linfócitos B Reguladores/imunologia , Dermatite Atópica/imunologia , Dermatomiosite/imunologia , Penfigoide Bolhoso/imunologia , Pênfigo/imunologia , Psoríase/imunologia , Animais , Linfócitos B Reguladores/efeitos dos fármacos , Linfócitos B Reguladores/metabolismo , Dermatite Atópica/sangue , Dermatite Atópica/tratamento farmacológico , Dermatite Atópica/patologia , Dermatomiosite/sangue , Dermatomiosite/tratamento farmacológico , Dermatomiosite/patologia , Desmogleína 3/metabolismo , Modelos Animais de Doenças , Humanos , Fatores Imunológicos/farmacologia , Fatores Imunológicos/uso terapêutico , Interleucina-10/metabolismo , Depleção Linfocítica/métodos , Penfigoide Bolhoso/sangue , Penfigoide Bolhoso/tratamento farmacológico , Penfigoide Bolhoso/patologia , Pênfigo/sangue , Pênfigo/tratamento farmacológico , Pênfigo/patologia , Psoríase/sangue , Psoríase/tratamento farmacológico , Psoríase/patologia , Pele/imunologia , Pele/patologia
4.
FP Essent ; 494: 25-29, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32640151

RESUMO

Polymyalgia rheumatica (PMR) is a chronic systemic inflammatory disease that is common in individuals older than 70 years. Classic symptoms of PMR include pain in the neck, pelvic girdle, and shoulders. Morning stiffness that lasts at least 30 minutes is typical. Glucocorticoids are the mainstay of PMR management, and prednisone 12.5 to 25 mg/day or equivalent is recommended. Giant cell arteritis is a comorbidity of PMR. Dermatomyositis is a rare, idiopathic inflammatory myopathy characterized by erythematous skin lesions and inflammation of skeletal muscles. Dermatomyositis manifests as proximal muscle weakness and fatigue that occurs when patients rise from a seated position, walk, climb stairs, or lift objects. It is a systemic condition and also may affect joints, the esophagus, and lungs. Prednisone is started at a dose of 60 mg/day and then tapered slowly, based on response, to prevent recurrence. Dermatomyositis may be associated with malignancy.


Assuntos
Dermatomiosite , Arterite de Células Gigantes , Polimialgia Reumática , Dermatomiosite/diagnóstico , Dermatomiosite/tratamento farmacológico , Diagnóstico Diferencial , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/tratamento farmacológico , Glucocorticoides , Humanos , Polimialgia Reumática/diagnóstico , Polimialgia Reumática/tratamento farmacológico , Prednisona
5.
Am J Med Sci ; 360(4): 402-405, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32591093

RESUMO

Dermatomyositis is an inflammatory disorder involving muscle and skin. Similar to many other autoimmune diseases, environmental factors appear to trigger the onset of disease in some cases. Many drugs have been reported to be associated with dermatomyositis, and rarely infections have been described as potential triggering agents. Here we are describing a case of dermatomyositis that developed after doxycycline and levofloxacin use, who also had recent Epstein-Barr virus infection. Dermatomyositis associated with doxycycline or levofloxacin use has not yet been described in the literature, while reports of dermatomyositis after Epstein-Barr virus infection have been rare and limited to juvenile dermatomyositis or in association with cancer. It is important for clinicians to be aware of this rare association so that the diagnosis and treatment can be exercised promptly.


Assuntos
Antibacterianos/efeitos adversos , Dermatomiosite/induzido quimicamente , Dermatomiosite/virologia , Doxiciclina/efeitos adversos , Infecções por Vírus Epstein-Barr/complicações , Levofloxacino/efeitos adversos , Antígenos Virais/sangue , Proteínas do Capsídeo/sangue , Dermatomiosite/sangue , Dermatomiosite/tratamento farmacológico , Infecções por Vírus Epstein-Barr/sangue , Antígenos Nucleares do Vírus Epstein-Barr/sangue , Humanos , Resultado do Tratamento
6.
BMJ Case Rep ; 13(4)2020 Apr 23.
Artigo em Inglês | MEDLINE | ID: mdl-32332045

RESUMO

An 80-year-old woman presented with a several-year history of progressive hair loss and scalp pruritus. No other rashes or muscle weakness were noted on examination. Scalp biopsy showed interface dermatitis, dense perivascular and periadnexal lymphocytic infiltrate, mucin and scarring alopecia. Laboratory analysis did not show evidence of myositis. The patient was started on hydroxychloroquine for possible cutaneous lupus erythematosus. On follow-up, she presented with a new violaceous rash on the superior eyelids and a well-defined oval patch on the mid-hard palate suspicious for dermatomyositis. Myositis-specific autoantibodies revealed presence of anti-transcriptional intermediary factor-1γ (anti-TIF1γ) in the serum. Anti-TIF1γ autoantibody-positive dermatomyositis is a newly recognised subtype of dermatomyositis that is highly associated with amyopathic disease and has an increased risk of malignancy, making prompt diagnosis crucial. This case highlights the utility of a thorough oral exam in patients suspected to have connective tissue disease as the distinctive ovoid palatal patch is nearly pathognomonic for anti-TIF1γ dermatomyositis.


Assuntos
Autoanticorpos/sangue , Dermatomiosite/diagnóstico , Eritema/patologia , Exantema/patologia , Palato Duro/patologia , Idoso de 80 Anos ou mais , Fármacos Dermatológicos/uso terapêutico , Dermatomiosite/tratamento farmacológico , Diagnóstico Diferencial , Feminino , Humanos , Metotrexato/uso terapêutico , Fatores de Transcrição/imunologia
7.
BMJ Case Rep ; 13(2)2020 Feb 28.
Artigo em Inglês | MEDLINE | ID: mdl-32111708

RESUMO

Dermatomyositis (DM) belongs to the spectrum of inflammatory myopathies which are a heterogeneous group of acquired disorders in which dysregulated immune system is thought to play a major pathologic role. Patients with DM generally present with characteristic skin manifestations with or without muscle weakness and pain. Although subclinical myocardial involvement in DM is well reported in literature, heart failure (HF) as its principal manifestation is extremely rare. Recognition of DM, a known paraneoplastic syndrome, should prompt clinicians to search for underlying malignancy. While gynaecological malignancies are common in association with DM, cervical cancer has rarely been heard of as an aetiology of paraneoplastic DM. We are reporting a case of DM in a postmenopausal woman presenting with advanced decompensated congestive HF with hidden squamous cell carcinoma of cervix.


Assuntos
Dermatomiosite/complicações , Dermatomiosite/tratamento farmacológico , Insuficiência Cardíaca/tratamento farmacológico , Insuficiência Cardíaca/etiologia , Neoplasias do Colo do Útero/etiologia , Neoplasias do Colo do Útero/cirurgia , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Debilidade Muscular , Síndromes Paraneoplásicas
8.
BMJ Case Rep ; 13(1)2020 Feb 02.
Artigo em Inglês | MEDLINE | ID: mdl-32014990

RESUMO

Mesenteric panniculitis (MP), part of the spectrum of sclerosing mesenteritis, is an often asymptomatic disorder that is characterised by chronic inflammation of abdominal mesentery. We present a case of an 83-year-old woman who presented with proximal muscle weakness and erythematous, photosensitive rash of the face and upper torso and was subsequently diagnosed with dermatomyositis based on skin biopsy, electromyography and muscle biopsy. She had radiographic evidence of panniculitis on CT scan of the abdomen and pelvis for malignancy surveillance, which improved on serial CT scan 3 months after beginning treatment for her underlying dermatomyositis with prednisone and mycophenolate mofetil. Our case highlights that MP can be associated with underlying autoimmune disease. Connective tissue disease could be considered in the differential of MP when other etiologies such as surgery, trauma and malignancy are ruled out.


Assuntos
Dermatomiosite/complicações , Paniculite Peritoneal/complicações , Idoso de 80 Anos ou mais , Anti-Inflamatórios/uso terapêutico , Dermatomiosite/diagnóstico por imagem , Dermatomiosite/tratamento farmacológico , Dermatomiosite/patologia , Feminino , Humanos , Ácido Micofenólico/uso terapêutico , Paniculite Peritoneal/diagnóstico por imagem , Paniculite Peritoneal/tratamento farmacológico , Prednisona/uso terapêutico , Radiografia , Tomografia Computadorizada por Raios X , Resultado do Tratamento
9.
Medicine (Baltimore) ; 99(3): e18600, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32011440

RESUMO

INTRODUCTION: Anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) is an autoantigen associated with dermatomyositis (DM). Anti-MDA5 Ab-positive DM patients frequently exhibit clinically amyopathic dermatomyositis (CADM), and develop rapidly progressive interstitial lung disease (RPILD). Even with early detection and potent combination immunosuppressive therapy, anti-MDA5 Ab-positive DM patients have a poor prognosis. In the present case report, we present a rare autopsy case of a patient with anti-MDA5 Ab DM with RPILD who exhibited diffuse alveolar damage (DAD) patterning in lung specimens, and extensive hemorrhages in multiple organs. PATIENT CONCERNS: An 82-year-old Japanese man admitted with bacterial pneumonia was subsequently diagnosed with anti-MDA5 Ab-positive DM based on skin manifestations (mechanic's hand, ulcerated palmar papules, and flagellate erythema), myositis, interstitial pneumonia, and elevation of anti-MDA5 Ab titer. DIAGNOSIS: The patient was diagnosed with anti-MDA5 Ab DM, complicated with RPILD. INTERVENTIONS: The patient received potent immunosuppressive therapy consisting of pulse methylpredonisolone at a dose of 1000 mg for 3 days, followed by prednisolone at 60 mg/d, a 1000 mg pulse of intravenous cyclophosphamide (IVCY), and oral tacrolimus at 6 mg/d. Intravenous immunoglobulin (IVIG) at a dose of 400 mg/kg/d for 5 days was subsequently administered. OUTCOMES: Despite triple immunosuppressive therapy and IVIG, the patients' respiratory status deteriorated, and the patient died of respiratory failure on the twelfth day after admission. An autopsy revealed pulmonary DAD and multiorgan hemorrhages, including the left iliopsoas muscle, gastric and bowl mucosa, spleen, and left adrenal gland. LESSONS: Multiorgan hemorrhages may be a fatal complication in anti-MDA5 Ab DM patients.


Assuntos
Dermatomiosite/complicações , Dermatomiosite/imunologia , Hemorragia/etiologia , Helicase IFIH1 Induzida por Interferon/imunologia , Idoso de 80 Anos ou mais , Autoanticorpos/imunologia , Autopsia , Dermatomiosite/tratamento farmacológico , Humanos , Imunossupressores/uso terapêutico , Pulmão/fisiopatologia , Masculino
10.
Medicine (Baltimore) ; 99(3): e18761, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32011462

RESUMO

INTRODUCTION: Corynebacterium striatum is common contaminant in clinical specimens. Here, we report a rare case of pyogenic tenosynovitis of the wrist caused by C striatum in a dermatomyositis patient taking oral immunosuppressants. PATIENT CONCERNS: A 67-year-old Japanese woman with dermatomyositis had a history of multiple intraarticular injections of corticosteroids to the right wrist joint for the treatment of osteoarthritis. She was admitted to our hospital with a painful lump on the right dorsal wrist lasting for three months. MRI revealed cellulitis of the dorsum of the right wrist and hand and fluid collection in the extensor tendon sheath. C striatum was detected in the cultures of three samples of synovial fluid taken from the dorsal hand. DIAGNOSIS: Pyogenic tenosynovitis of the wrist due to C striatum. INTERVENTIONS: The infection was successfully controlled with synovectomy and adjuvant antibiotic therapy. OUTCOMES: There has been no sign of recurrence for 12-months after the surgical treatment. LESSONS: This is the first reported case of pyogenic tenosynovitis due to C striatum in a patient with dermatomyositis. Clinicians should be aware that patients undergoing immunosuppressive therapy have a risk of C striatum infection.


Assuntos
Infecções por Corynebacterium/microbiologia , Corynebacterium , Dermatomiosite/microbiologia , Tenossinovite/microbiologia , Idoso , Terapia Combinada , Infecções por Corynebacterium/terapia , Dermatomiosite/tratamento farmacológico , Feminino , Humanos , Imunossupressores/uso terapêutico , Tenossinovite/terapia
11.
Medicine (Baltimore) ; 99(7): e19012, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32049796

RESUMO

Immunoglobulins are 2nd or 3rd-line treatments in dermatomyositis (DM) or polymyositis (PM) refractory to high-dose corticosteroids and immunosuppressants. Immunoglobulins (2 g/kg/mo) are usually administered intravenously (IVIg) once a month and the patients stay at hospital for a few days. Recently, subcutaneous injections (SCIg) were proposed 2 to 3 times per week, in some dysimmune diseases. SCIg are administered at home preferably by the patient or by a nurse. We investigated the needs and attitudes of DM and PM patients with experience of IVIg and SCIg.Seven patients (6 PM and 1 DM) from a single center participated in a focus group (N = 6) or underwent in-depth interview (N = 1). Six had the experience of both IVIg at hospital and SCIg at home; 1 has received only IVIg at hospital. Verbatim was recorded and transcribed for further content analysis and computer-aided textual analysis.Clinical profiles and stories were heterogeneous. At diagnosis, muscle weakness, severe pain, and fatigue were at the forefront of patients' complaints impairing daily life. Patients reported considerable improvement with immunoglobulins. SCIg were described as easy, less disruptive for daily life, well tolerated, and less time-consuming. SCIg self-administration at home restored the feeling of autonomy and control.Interviews of DM and PM patients revealed that recovering autonomy and control was a central advantage of home-based SCIg that were efficient, well tolerated, and perceived as a good compromise between treatment burden and efficacy.


Assuntos
Dermatomiosite/tratamento farmacológico , Imunização Passiva/métodos , Imunoglobulinas/administração & dosagem , Polimiosite/tratamento farmacológico , Adulto , Idoso , Idoso de 80 Anos ou mais , Esquema de Medicação , Feminino , Grupos Focais , Humanos , Imunoglobulinas/uso terapêutico , Injeções Subcutâneas/enfermagem , Masculino , Pessoa de Meia-Idade , Pesquisa Qualitativa , Autoadministração , Resultado do Tratamento
12.
Arthritis Rheumatol ; 72(7): 1214-1226, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32103637

RESUMO

OBJECTIVE: Juvenile dermatomyositis (DM) is a heterogeneous systemic immune-mediated vasculopathy. This study was undertaken to 1) identify inflammation/endothelial dysfunction-related biomarker profiles reflecting disease severity at diagnosis, and 2) establish whether such biomarker profiles could be used for predicting the response to treatment in patients with juvenile DM. METHODS: In total, 39 biomarkers related to activation of endothelial cells, endothelial dysfunction, and inflammation were measured using multiplex technology in serum samples from treatment-naive patients with juvenile DM from 2 independent cohorts (n = 30 and n = 29). Data were analyzed by unsupervised hierarchical clustering, nonparametric tests with correction for multiple comparisons, and Kaplan-Meier tests with Cox proportional hazards models for analysis of treatment duration. Myositis-specific antibodies (MSAs) were measured in the patients' serum using line blot assays. RESULTS: Severe vasculopathy in patients with juvenile DM was associated with low serum levels of intercellular adhesion molecule 1 (Spearman's rho [rs ] = 0.465, P = 0.0111) and high serum levels of endoglin (rs = -0.67, P < 0.0001). In the discovery cohort, unsupervised hierarchical clustering analysis of the biomarker profiles yielded 2 distinct patient clusters, of which the smaller cluster (cluster 1; n = 8) exhibited high serum levels of CXCL13, CCL19, galectin-9, CXCL10, tumor necrosis factor receptor type II (TNFRII), and galectin-1 (false discovery rate <0.0001), and this cluster had greater severity of muscle disease and global disease activity (each P < 0.05 versus cluster 2). In the validation cohort, correlations between the serum levels of galectin-9, CXCL10, TNFRII, and galectin-1 and the severity of global disease activity were confirmed (rs = 0.40-0.52, P < 0.05). Stratification of patients according to the 4 confirmed biomarkers identified a cluster of patients with severe symptoms (comprising 64.7% of patients) who were considered at high risk of requiring more intensive treatment in the first 3 months after diagnosis (P = 0.0437 versus other cluster). Moreover, high serum levels of galectin-9, CXCL10, and TNFRII were predictive of a longer total treatment duration (P < 0.05). The biomarker-based clusters were not evidently correlated with patients' MSA serotypes. CONCLUSION: Results of this study confirm the heterogeneity of new-onset juvenile DM based on serum biomarker profiles. Patients with high serum levels of galectin-9, CXCL10, TNFRII, and galectin-1 may respond suboptimally to conventional treatment, and may therefore benefit from more intensive monitoring and/or treatment.


Assuntos
Dermatomiosite/tratamento farmacológico , Dermatomiosite/metabolismo , Imunossupressores/uso terapêutico , Biomarcadores , Quimiocina CCL19/imunologia , Quimiocina CXCL10/imunologia , Quimiocina CXCL13/imunologia , Criança , Pré-Escolar , Estudos de Coortes , Dermatomiosite/imunologia , Duração da Terapia , Endoglina/metabolismo , Células Endoteliais/metabolismo , Endotélio Vascular/metabolismo , Endotélio Vascular/fisiopatologia , Feminino , Galectina 1/metabolismo , Galectinas/metabolismo , Humanos , Inflamação/imunologia , Molécula 1 de Adesão Intercelular/metabolismo , Masculino , Prognóstico , Modelos de Riscos Proporcionais , Receptores Tipo II do Fator de Necrose Tumoral/imunologia
14.
J Zoo Wildl Med ; 50(4): 1008-1011, 2020 Jan 09.
Artigo em Inglês | MEDLINE | ID: mdl-31926538

RESUMO

Curvularia spp. are globally distributed saprophytic fungi, classified in the literature as dematiaceous, or darkly pigmented fungi. These fungi have been increasingly recognized as causing cutaneous, ocular, respiratory, and central nervous system infections in humans, but have been infrequently documented as pathogens in the veterinary literature. A 5-yr-old male Chinese goral (Naemorhedus griseus) presented with bilateral fungal dermatitis of the pinnae, and subsequent pyogranulomatous rhinosinusitis. Clinical signs included epistaxis, mucosanguineous nasal discharge, and dyspnea. Sequential histologic examinations of cutaneous and nasal lesions revealed pyogranulomatous inflammation with extracellular and phagocytized nonpigmented yeasts. Fungal culture and polymerase chain reaction identified Curvularia sp. The absence of pigmentation in tissue in this case suggests that pigmentation may not be a consistent histologic finding for this fungus, emphasizing the importance of molecular identification to prevent misidentification. Despite intensive interventions in this goral, the disease progressed, and was ultimately fatal.


Assuntos
Dermatomiosite/veterinária , Rinite/veterinária , Sinusite/veterinária , Animais , Animais de Zoológico , Antifúngicos/uso terapêutico , Clotrimazol/uso terapêutico , Dermatomiosite/tratamento farmacológico , Dermatomiosite/microbiologia , Griseofulvina/uso terapêutico , Masculino , Rinite/tratamento farmacológico , Rinite/microbiologia , Ruminantes , Sinusite/tratamento farmacológico , Sinusite/microbiologia
15.
Mod Rheumatol ; 30(3): 411-423, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-31955618

RESUMO

Juvenile dermatomyositis is the most common type of juvenile idiopathic inflammatory myopathy mainly affecting the skin and proximal muscles. We have published the Japanese version of 'Clinical practice guidance for juvenile dermatomyositis (JDM) 2018 'consisting of a review of articles in the field and evidence-informed consensus-based experts' opinion on the treatment strategy in collaboration with The Pediatric Rheumatology Association of Japan and The Japan College of Rheumatology under the financial support by 'Research on rare and intractable diseases, Health and Labor Sciences Research Grants'. This article is a digest version of the Japanese guidance.


Assuntos
Dermatomiosite/diagnóstico , Guias de Prática Clínica como Assunto , Adolescente , Criança , Consenso , Dermatomiosite/tratamento farmacológico , Humanos , Japão , Reumatologia/organização & administração , Sociedades Médicas/normas
18.
Pediatr Rheumatol Online J ; 17(1): 56, 2019 Aug 20.
Artigo em Inglês | MEDLINE | ID: mdl-31429786

RESUMO

The idiopathic inflammatory myopathies (IIM) until recently have been considered a heterogeneous broad group of six autoimmune muscle diseases. Initially, autoantibodies in IIM (including JDM) and CD8+ T cell-induced cytotoxicity (PM and IBM) were the predominant recognized etiopathology mechanisms used to classify myopathies. In the early late 1990's to 2000's, evolving understanding of the molecules such as interleukin (IL), tumor necrosis factor (TNF), interferon (IFN), and other cytokines as well as differences in response to therapies, has led IIM researchers to look beyond previous disease mechanisms. For decades the overexpression of Th1- associated cytokines (TNF-α, IFN-γ and IL-12) in the areas of inflammation in skin and muscle in IIM pointed to Th1 as the primary pathway for inflammation in myositis.However, in the last decade overexpression and elevated level of Th17-associated cytokines (IL-17, IL-22, and IL-6) were identified in the blood and the inflamed muscles of myositis patients. We also do not know how Th1 and Th2 cytokines work differently in diverse hosts, in different concentrations, in different inflammatory milieus, and in the presence or absence of each other or other adhesion/co-stimulatory molecules such as NF-κB. Also, several autoantibodies to intracellular organelles have been identified in myositis.In this review, we will discuss the most recent advances in IIM research and how that might bring new biologic therapies to market in the next 5-15 years to assist in the care of our most difficult IIM and JDM patients.


Assuntos
Produtos Biológicos/uso terapêutico , Miosite/tratamento farmacológico , Adulto , Anticorpos Monoclonais Humanizados/uso terapêutico , Criança , Doença Crônica , Ensaios Clínicos como Assunto , Dermatomiosite/tratamento farmacológico , Previsões , Humanos , Imunossupressores/uso terapêutico , Terapias em Estudo/tendências
19.
Pathol Int ; 69(9): 536-540, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31273873

RESUMO

Upper-lobe predominance of elastofibrosis is agreed upon for the diagnosis of clinical pleuroparenchymal fibroelastosis (PPFE). We herein describe a patient with dermatomyositis-related interstitial pneumonia with a histology of lower-lobe predominant PPFE. A 71-year-old woman who had been diagnosed with dermatomyositis-related interstitial pneumonia died of respiratory failure. The computed tomography patterns of the lower lobes showed reticular and ground-glass opacities with traction bronchiectasis. An autopsy revealed that the bilateral lower lobes were sclerotic with decreased air volume. A microscopic examination of the lower lobes showed pleural fibrosis and subpleural elastofibrosis without the structural destruction, indicative of histological PPFE. PPFE histology was also evident in the upper lobes but relatively modest compared to that of the lower lobes. In addition, because the computed tomography images of the patient were suggestive of non-PPFE-type fibrosis, lower-lobe dominant PPFE might be overlooked in daily practice.


Assuntos
Dermatomiosite/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pleurais/diagnóstico por imagem , Fibrose Pulmonar/diagnóstico por imagem , Corticosteroides/uso terapêutico , Idoso , Autopsia , Dermatomiosite/complicações , Dermatomiosite/tratamento farmacológico , Dermatomiosite/patologia , Evolução Fatal , Feminino , Humanos , Imunossupressores/uso terapêutico , Pulmão/diagnóstico por imagem , Pulmão/patologia , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/patologia , Tecido Parenquimatoso/diagnóstico por imagem , Tecido Parenquimatoso/patologia , Inibidores da Fosfodiesterase 5/uso terapêutico , Pleura/diagnóstico por imagem , Pleura/patologia , Doenças Pleurais/complicações , Doenças Pleurais/tratamento farmacológico , Doenças Pleurais/patologia , Fibrose Pulmonar/complicações , Fibrose Pulmonar/tratamento farmacológico , Fibrose Pulmonar/patologia , Tadalafila/uso terapêutico , Tomografia Computadorizada por Raios X
20.
Dermatol Online J ; 25(6)2019 Jun 15.
Artigo em Inglês | MEDLINE | ID: mdl-31329393

RESUMO

Gottron papules, a heliotrope rash, scalp and extremity erythema, pruritus, and fatigue are the characteristic signs and symptoms of amyopathic dermatomyositis (ADM). Amyopathic dermatomyositis is considered a distinct entity from dermatomyositis (DM) because the characteristic muscle weakness and muscle enzyme elevations of DM are absent in ADM. With respects to treatment, ADM treatments have traditionally included topical corticosteroids and/or systemic immunosuppressants and immunomodulators. Herein we present a patient with refractory ADM that was responsive to low-dose naltrexone therapy.


Assuntos
Dermatomiosite/tratamento farmacológico , Naltrexona/administração & dosagem , Antagonistas de Entorpecentes/administração & dosagem , Humanos , Masculino , Pessoa de Meia-Idade , Naltrexona/uso terapêutico , Antagonistas de Entorpecentes/uso terapêutico
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