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1.
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
2.
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
3.
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
4.
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
5.
Arthritis Care Res (Hoboken) ; 72(2): 265-273, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31507092

RESUMO

OBJECTIVE: To study growth and puberty in a multinational longitudinal prospective cohort of children with juvenile dermatomyositis (DM). METHODS: Children from 31 countries who were ages <18 years and had juvenile DM in active phase were studied, and analyses of height, weight, and pubertal development were conducted in those who had follow-up visits during a 2-year period and for whom anthropometric data was available. RESULTS: A total of 196 of 275 children (71%) were included. We found a significant reduction in parent-adjusted height Z score over time in female patients (P < 0.0001) and male patients (P = 0.001), but with catch-up growth at the final study visit. Median body mass index Z score peaked at 6 months (P < 0.0001) and was still significantly above baseline at the final study visit, which was at a median of 26 months after baseline (P = 0.007), with no difference between sexes. Female patients with a disease duration ≥12 months after onset had significantly lower parent-adjusted height Z score (P = 0.002) and no 2-year catch-up growth. At the final study visit, growth failure was seen in 20 of 97 female patients (21%) and in 11 of 73 male patients (15%). Height deflection (∆height Z score less than -0.25/year) was observed in 29 of 116 female patients (25%) and 25 of 80 male patients (31.3%). Delayed puberty was seen in 20 of 55 female patients (36.4%) and in 11 of 31 male patients (35.5%). Children in early pubertal stage at baseline had the highest risk of growth failure. CONCLUSION: Juvenile DM in the active phase and/or its treatment has a significant impact on growth and puberty in affected children. Children with recent onset of puberty or previous growth failure have the highest risk of delayed pubertal development and further growth retardation.


Assuntos
Dermatomiosite/diagnóstico , Dermatomiosite/fisiopatologia , Puberdade/fisiologia , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Seguimentos , Humanos , Estudos Longitudinais , Masculino
9.
F1000Res ; 82019.
Artigo em Inglês | MEDLINE | ID: mdl-31824645

RESUMO

Dermatomyositis (DM) is an inflammatory myopathy with characteristic skin manifestations, the pathologies of which are considered autoimmune diseases. DM is a heterogeneous disorder with various phenotypes, including myositis, dermatitis, and interstitial lung disease (ILD). Recently identified myositis-specific autoantibodies have been associated with distinct clinical features. For example, anti-melanoma differentiation-associated protein 5 antibodies have a high specificity for clinically amyopathic DM presenting rapidly progressive ILD. Furthermore, anti-transcriptional intermediary factor 1γ antibodies found in patients with juvenile and adult DM are closely correlated with malignancies, especially in elderly patients. Finally, patients with anti-aminoacyl-transfer RNA synthetase antibodies share characteristic clinical symptoms, including myositis, ILD, arthritis/arthralgia, Raynaud's phenomenon, and fever; thus, the term "anti-synthetase syndrome" is also used. With a focus on the characteristic cutaneous manifestations in each subgroup classified according to myositis-specific autoantibodies, we introduce the findings of previous reports, including our recent analysis indicating that skin eruptions can be histopathologically classified into myositis-specific autoantibody-associated subgroups and used to determine the systemic pathologies of the different types of antibody-associated DM.


Assuntos
Dermatomiosite , Doenças Pulmonares Intersticiais , Miosite , Aminoacil-tRNA Sintetases , Autoanticorpos , Humanos
10.
Zhonghua Er Ke Za Zhi ; 57(12): 928-933, 2019 Dec 02.
Artigo em Chinês | MEDLINE | ID: mdl-31795559

RESUMO

Objective: To report the clinical features of anti-MDA5 antibody positive juvenile dermatomyositis (JDM) complicated with severe interstitial lung disease (ILD). Methods: The clinical data of three patients, who was admitted to the Department of Rheumatology and Immunology, Children's Hospital of the Capital Institute of Pediatrics from September 2016 to July 2017, with anti-melanoma differentiation associated gene 5 (MDA5) antibody positive JDM complicated with ILD were retrospectively extracted and analyzed. Meanwhile, PubMed database, CNKI, Wanfang database and China Biology Medicine disc (from their establishment to February 2019) with the key words "juvenile dermatomyositis" "interstitial lung disease" , and "anti-MAD5 antibody" both in English and Chinese were searched. Results: There were 2 females and 1 male (P1-P3), aged from 10 years 3 months to13 years 4 months, the time from onset to diagnosis were 2 months, 4 months and 10 months. All presented with rash. One of them had decreased muscle strength, and two had decreased activity tolerance. Creatine kinase was 588, 915 and 74 U/L, and serum ferritin were 1 792, >2 000 and 195.4 µg/L. All three patients had positive anti-MDA5 antibodies. At the time of diagnosis, all of them had ILD, pneumothorax and mediastinal emphysema, but had no respiratory symptoms. All three patients received oral methylprednisolone and cyclophosphamide pulse therapy, while human immunoglobulin was given only to P1 and P2. P1 developed rapid progressive pulmonary interstitial disease (RPILD) and died of respiratory failure after 2 months. While P2 and P3 were followed up for 1 to 2 years, who had complete remission, as anti-MDA5 antibody turned to negative and ILD improved significantly. Ten related reports in literature were retrieved, without reported Chinese cases, and most cases initiated with rash and very likely complicated with arthritis. Some of them were more likely to have ILD rather than muscle weakness. It also showed that Japanese JDM children had higher rate of positive anti-MDA5 antibody than patients from the U.S. and U.K., and are more susceptible to ILD and RPILD. The mortality rate of patients with RPILD is extremely high. Conclusions: The cases of JDM with positive anti-MDA5 antibody mainly presented with rash and mild muscle weakness, and could be complicated with ILD, pneumothorax and mediastinal emphysema without respiratory symptoms at early stage. Anti-MDA5 antibody titer is related to disease activity and can turn to negative after treatment.


Assuntos
Autoanticorpos/imunologia , Dermatomiosite/diagnóstico , Helicase IFIH1 Induzida por Interferon/imunologia , Doenças Pulmonares Intersticiais/diagnóstico , Adolescente , Criança , China , Dermatomiosite/imunologia , Feminino , Humanos , Doenças Pulmonares Intersticiais/imunologia , Masculino , Prognóstico , Estudos Retrospectivos
11.
Beijing Da Xue Xue Bao Yi Xue Ban ; 51(6): 1173-1177, 2019 Dec 18.
Artigo em Chinês | MEDLINE | ID: mdl-31848525

RESUMO

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.


Assuntos
Dermatomiosite , Doenças Pulmonares Intersticiais , Vasculite , Feminino , Humanos , Imunoglobulina A , Pele
12.
Zhong Nan Da Xue Xue Bao Yi Xue Ban ; 44(10): 1157-1162, 2019 Oct 28.
Artigo em Chinês | MEDLINE | ID: mdl-31857510

RESUMO

OBJECTIVE: To investigate the clinical characteristics of dermatomyositis, to investigate the types and clinical features of dermatomyositis complicated with malignant tumor, and to provide evidence for clinical diagnosis, treatment and prognostic evaluation.
 Methods: The clinical manifestations and laboratory test results for 108 cases of dermatomyositis with complications in the Second Xiangya Hospital of Central South University were analyzed.
 Results: Patients aged from 14 to 60 years accounted for 62.96%. The first symptom was single skin rash (54.63%), and the most characteristic cutaneous features were asymmetrical proximal myositis with various degrees (97.22%). The visceral involvement was as follows: the digestive tract (31.48%), the heart (19.44%), the lung (26.85%), and the thyroid damage (12.96%). Twelve (11.11%) patients were combined with malignant tumor. The positive rates for albumin (ALB), glutamic oxalacetic transaminase (AST), glutamic-pyruvic transaminase (ALT), lactate dehydrogenase (LDH), creatine kinase (CK), creatine kinase isoenzyme (CK-MB), erythrocyte sedimentation rate (ESR), anti Jo-1 antibody, anti ribonucleoprotein (RNP) antibody, and anti-topoisomerasel (Scl) antibody were 25.93%, 46.30%, 28.70%, 87.04%%, 51.85%, 26.85%, 55.56%, 2.27%, 8.99%, and 2.27%, respectively. The patients were divided into a tumor group and a non-tumor group. The chi-square test results from clinical symptoms and laboratory tests suggested that increase of ESR was a risk factor for dermatomyositis combining tumor. The main strategy of therapy was corticosteroids.
 Conclusion: Dermatomyositis possesses typical skin lesions and dermatitis is the most common initial symptom of dermatomyositis. In clinic, diagnosis of dermatomyositis should be timely combined with muscle enzymes test, electromyography and muscle biopsy. Dermatomyositis can easily involve many organs. Thus relevant examinations (such as chest X-ray and CT) should be done preventively. Rapid ESR is a risk factor for dermatomyositis complicated with malignant tumor and it can be used as an index to guide clinical diagnosis.


Assuntos
Dermatomiosite , Adolescente , Adulto , Creatina Quinase , Eletromiografia , Humanos , Pessoa de Meia-Idade , Pele , Adulto Jovem
14.
Best Pract Res Clin Rheumatol ; 33(4): 101440, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31585842

RESUMO

Skin lesions occur, often at very early stages, in many of the most frequent inflammatory rheumatic diseases such as in systemic lupus erythematosus (SLE), dermatomyositis (DM), systemic sclerosis (SSc), Sjögren's syndrome, rheumatoid arthritis (RA), and psoriatic arthritis. It is important to recognize the different specific cutaneous lesions in SLE (e.g., "butterfly" rash in acute, annular or psoriasiform photosensitive lesions in the subacute form, and discoid lesions in the chronic form) for an early diagnosis and to estimate the associated risks of internal disease, whereas nonspecific lesions (exanthema, vasculitis, and alopecia) can be part of SLE flares. Cutaneous lesions in DM (Gottron's papules and sign, heliotrope rash, dystrophic cuticles, and nailfold capillary abnormalities) may occur before any clinically evident muscular or systemic organ involvement and are of utmost importance for early diagnosis. The pattern of cutaneous lesions and associated autoantibodies also allow the distinction of different phenotypes, either more prone to life-threatening interstitial lung disease (MDA-5) or with higher risk for neoplasia (TIF1-γ). Many other skin lesions, although not specific, require further investigation to look for a possible underlying inflammatory rheumatic disease: non-pruritic urticarial lesions in anti-C1q-associated urticarial vasculitis, Still's disease or hereditary auto-inflammatory syndromes, transient macular purpura of vasculitis in Sjögren's syndrome, Behçet's disease, or RA, Raynaud's phenomenon in SSc and mixed connective tissue disease, erythema nodosum or other panniculitis in RA, Behçet's disease and SLE, pustular eruptions in Behçet's disease, psoriasis, and hereditary auto-inflammatory syndromes. After reviewing in detail the cutaneous manifestations of the most frequent inflammatory rheumatic diseases, we describe a topographic and morphological approach to skin rashes, calling attention to facial rashes, hand involvement, scalp, nail, or leg lesions or to some morphological aspects of skin lesions (annular, pustular, urticarial, or exanthematous) that may be the initial manifestations of inflammatory rheumatic diseases. The importance of skin lesions is confirmed by their presence as part of the classification criteria of many inflammatory rheumatic diseases. They also contribute to early diagnosis, to characterize disease phenotypes, to aid in effective patient management, and, ultimately, to impact on disease prognosis.


Assuntos
Artrite Reumatoide , Dermatomiosite , Exantema , Lúpus Eritematoso Sistêmico , Doenças Reumáticas , Síndrome de Sjogren , Artrite Reumatoide/complicações , Artrite Reumatoide/diagnóstico , Dermatomiosite/complicações , Dermatomiosite/diagnóstico , Exantema/etiologia , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Doenças Reumáticas/complicações , Doenças Reumáticas/diagnóstico , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico , Dermatopatias
15.
Zhonghua Jie He He Hu Xi Za Zhi ; 42(10): 765-770, 2019 Oct 12.
Artigo em Chinês | MEDLINE | ID: mdl-31594111

RESUMO

Objective: To investigate the clinical significance of detection of myositis-specific antibodies (MSAs) and myositis-associated antibodies (MAAs) in patients with connective tissue disease-associated interstitial lung diseases (CTD-ILD). Methods: Serum samples of 120 patients with CTD-ILD admitted to the Department of Respiratory, Affiliated Drum Tower Hospital of Nanjing University Medical College from December 2016 to April 2018 were collected for analysis. The patients included 45 with polymyositis/dermatomyositis (PM/DM), 36 with Sjogren's syndrome (SS) and 39 with undifferentiated connective tissue disease (UCTD). There were 37 males and 83 females with an average age of (56±11) years. Thirty-two patients with non-CTD-ILD, 10 males and 22 females with an average age of (42±17) years, were enrolled as the control group. Euroline Autoimmune Inflammatory Myopathies 16 Ag kit was used for detecting MSAs and MAAs, and the positive rates of serum MSAs and MAAs were calculated. The antibody distribution and clinical characteristics of different groups were analyzed and compared. Results: Eighty-nine of the 120 patients with CTD-ILD were positive for MSA and/or MAA (74.2%), and the detection rates of MSAs and MAAs were 52.5% (63/120) and 61.7% (74/120) respectively. No myositis antibody was detected in the non-CTD-ILD group. The detection rates of MSAs in PM/DM-ILD group, SS-ILD group and UCTD-ILD group were 75.6% (34/45), 33.3%(12/36) and 43.6%(17/39) respectively. The total detection rate of MSAs in PM/DM group was significantly higher than that in SS group and UCTD group (χ(2)=14.53, 8.95, 0.01). The anti-ARS was the most frequent (50/120, 41.7%). The positive rates of MAAs in the three groups were 64.4%(29/45), 77.8%(28/36), 43.6%(17/39) respectively, and anti-Ro-52 accounted for 60%(72/120), and were highly correlated with MSAs such as anti-Jo-1 antibodies. Conclusion: Myositis antibody profiling should be performed in patients with ILD who were negative for conventional autoimmune antibody testing and had no CTD. In patients with SS-ILD and UCTD-ILD, the myositis antibody spectrum could detect the presence of myositis-specific antibodies and myositis-related antibodies in some patients, and its role in clinical diagnosis and treatment needed further observation.


Assuntos
Autoanticorpos/sangue , Autoantígenos/imunologia , Doenças Pulmonares Intersticiais/imunologia , Miosite/imunologia , Polimiosite/imunologia , Adulto , Idoso , China/epidemiologia , Doenças do Tecido Conjuntivo/sangue , Dermatomiosite/sangue , Dermatomiosite/complicações , Dermatomiosite/epidemiologia , Dermatomiosite/imunologia , Feminino , Humanos , Doenças Pulmonares Intersticiais/sangue , Doenças Pulmonares Intersticiais/etiologia , Masculino , Pessoa de Meia-Idade , Miosite/sangue , Miosite/diagnóstico , Miosite/epidemiologia , Polimiosite/complicações , Polimiosite/epidemiologia , Testes Sorológicos
16.
Zhonghua Yi Xue Za Zhi ; 99(38): 2976-2981, 2019 Oct 15.
Artigo em Chinês | MEDLINE | ID: mdl-31607028

RESUMO

Objective: To explore the expression and clinical significance of chemokine ligand 18 (CCL18) in Bronchoalveolar Lavage Fluid (BALF) of patients with connective tissue disease-associated interstitial lung disease (CTD-ILD). Methods: From January 2016 to June 2017, BALF of patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD group), patients with dermatomyositis-associated interstitial lung disease (DM-ILD group), and patients with primary Sjögren syndrome-associated interstitial lung disease (pSS-ILD group) of Peking University People's Hospital were collected. According to the prognosis of each group of patients during hospitalization, they were divided into the discharged and the died. Besides, 30 patients without ILD served as a control group. Levels of CCL18 in BALF of all patients were tested by enzyme linked immunosorbent assay (ELISA). Cells in BALF of RA-ILD group, DM-ILD group and pSS-ILD group were collected and analyzed by absolute different cell counts. Results of high-resolution CT (HRCT) of these three groups were scored. In addition, the area under the curve (AUC) of CCL18 in predicting mortality during hospitalization was calculated. Results: A total of 38 patients with RA-ILD, 54 patients with DM-ILD, and 35 patients with pSS-ILD were enrolled. Levels of CCL18 of those discharged patients of RA-ILD, DM-ILD, and pSS-ILD groups were 8.27(3.62, 14.36), 11.04 (5.86, 17.38), 5.25(2.68, 8.21) µg/L, respectively, which were all significantly higher than that of the control group [2.54(1.26, 3.66) µg/L, all P<0.05]. Furthermore, levels of CCL18 of those deceased patients of RA-ILD and DM-ILD groups were 18.28 (13.82, 22.39), 18.81 (16.29, 22.90) µg/L, which were significantly higher than that of the discharged patients of same group (all P<0.05). Levels of CCL18 were positively correlated with lymphocyte percentage in BALF of RA-ILD, DM-ILD and pSS-ILD groups (r=0.4356, 0.4029, 0.3939, respectively, all P<0.05). Besides, levels of CCL18 were significantly correlated with HRCT scores of RA-ILD and DM-ILD groups (r=0.4242, 0.3319, respectively, both P<0.05). Areas under the curve (AUCs) of CCL18 to predict mortality during hospitalization of RA-ILD and DM-ILD groups were 0.860, 0.851, respectively. Conclusions: Levels of CCL18 are elevated in BALF of CTD-ILD patients, and may be correlated with the severity and prognosis during hospitalization. CCL18 might be served as an indicator of the severity and prognosis of CTD-ILD.


Assuntos
Doenças do Tecido Conjuntivo , Dermatomiosite , Doenças Pulmonares Intersticiais , Líquido da Lavagem Broncoalveolar , Quimiocinas , Quimiocinas CC , Doenças do Tecido Conjuntivo/metabolismo , Humanos , Doenças Pulmonares Intersticiais/etiologia
19.
J Drugs Dermatol ; 18(10): 995-998, 2019 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-31584777

RESUMO

Background: Autoimmune connective tissue diseases (ACTDs) are a broad range of diseases featured by immune dysregulation, and often have multisystem involvement with prominent skin manifestations. Pruritus is one of the most common symptoms in these diseases, with significant impact on the quality of life of patients. Objective: To characterize the frequency, location, severity, and timing relative to disease onset of pruritus in different ACTDs. Methods: A chart review of all patients seen in the Rheumatology-Dermatology clinic at Massachusetts General Hospital. Results: Itch was a troubling symptom in 83% of dermatomyositis (DM), 61% of systemic lupus erythematosus (SLE), 59% of Sjogren syndrome (SJO), 22% of systemic sclerosis (SSc), and 60% of mixed connective tissue disease. In DM and SLE, itch paralleled the course of inflammatory skin manifestations in 83% and 45%, respectively. Itch in DM is more intense and more treatment resistant in 12% vs 1% in SLE. In contrast, itch in SSc and SJO tended to occur later in the disease course, 86% vs 42%, respectively. Conclusion: Itch is common in all ACTDs and often under-evaluated and under treated. Pruritus is more common and more severe in DM than in SLE. Treatment of pruritus in ACTDs can be challenging, and sometimes multi-modal therapy is warranted. J Drugs Dermatol. 2019;18(10):995-998.


Assuntos
Dermatomiosite/complicações , Lúpus Eritematoso Sistêmico/complicações , Prurido/diagnóstico , Escleroderma Sistêmico/complicações , Síndrome de Sjogren/complicações , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Terapia Combinada/métodos , Dermatomiosite/imunologia , Feminino , Humanos , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Pessoa de Meia-Idade , Prurido/imunologia , Prurido/terapia , Qualidade de Vida , Estudos Retrospectivos , Escleroderma Sistêmico/imunologia , Índice de Gravidade de Doença , Síndrome de Sjogren/imunologia , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
20.
Neurology ; 93(19): e1768-e1777, 2019 11 05.
Artigo em Inglês | MEDLINE | ID: mdl-31594859

RESUMO

OBJECTIVE: To define the clinical phenotype of dermatomyositis (DM) with anti-Mi2 autoantibodies. METHODS: In this longitudinal cohort study, the prevalence and severity of clinical features at disease onset and during follow-up in patients with anti-Mi2-positive DM were compared to patients with anti-Mi2-negative DM, antisynthetase syndrome (AS), and immune-mediated necrotizing myopathy (IMNM). Longitudinal anti-Mi2 autoantibody titers were assessed. RESULTS: A total of 58 patients with anti-Mi2-positive DM, 143 patients with anti-Mi2-negative DM, 162 patients with AS, and 170 patients with IMNM were included. Among patients with anti-Mi2-positive DM, muscle weakness was present in 60% at disease onset and occurred in 98% during longitudinal follow-up; fewer patients with anti-Mi2-negative DM developed weakness (85%; p = 0.008). Patients with anti-Mi2-positive DM were weaker and had higher creatine kinase (CK) levels than patients with anti-Mi2-negative DM or patients with AS. Muscle biopsies from patients with anti-Mi2-positive DM had prominent necrosis. Anti-Mi2 autoantibody levels correlated with CK levels and strength (p < 0.001). With treatment, most patients with anti-Mi2-positive DM had improved strength and CK levels; among 10 with multiple serum samples collected over 4 or more years, anti-Mi2 autoantibody titers declined in all and normalized in 3, 2 of whom stopped immunosuppressant treatment and never relapsed. Patients with anti-Mi2-positive DM had less calcinosis (9% vs 28%; p = 0.003), interstitial lung disease (5% vs 16%; p = 0.04), and fever (7% vs 21%; p = 0.02) than did patients with anti-Mi2-negative DM. CONCLUSIONS: Patients with anti-Mi2-positive DM have more severe muscle disease than patients with anti-Mi2-negative DM or patients with AS. Anti-Mi2 autoantibody levels correlate with disease severity and may normalize in patients who enter remission.


Assuntos
Autoanticorpos/imunologia , Calcinose/epidemiologia , Dermatomiosite/imunologia , Febre/epidemiologia , Doenças Pulmonares Intersticiais/epidemiologia , Complexo Mi-2 de Remodelação de Nucleossomo e Desacetilase/imunologia , Debilidade Muscular/epidemiologia , Adulto , Idoso , Calcinose/fisiopatologia , Estudos de Casos e Controles , Estudos de Coortes , Creatina Quinase/sangue , Dermatomiosite/sangue , Dermatomiosite/epidemiologia , Dermatomiosite/fisiopatologia , Feminino , Humanos , Estudos Longitudinais , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/fisiopatologia , Miosite/imunologia , Miosite/fisiopatologia , Necrose , Fenótipo , Prevalência , Índice de Gravidade de Doença
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