RESUMO
OBJECTIVES: In addition to various immunosuppressive agents, belimumab and anifrolumab became available in Japan. We aimed to investigate glucocorticoid-free clinical remission in a single-centre retrospective cohort in October 2023. METHODS: Our cohort included patients with SLE who needed to start or increase glucocorticoids for disease activity and were followed up for more than 1 year. We investigated the rate of achievement of clinical remission off corticosteroids (CR off C), defined as no clinical score on the SLEDAI-2K without glucocorticoids, baseline predictors of CR off C, medications used when CR off C was achieved, and flare rates following CR off C. RESULTS: Out of the 60 patients followed for an average of 5.4 (±2.6) years, 17 (28.3%) achieved CR off C in 3.6 (±1.2) years after enrolment. Use of belimumab and anifrolumab accounted for eight (47.1%) of the achievers. Among the baseline data, male sex, recent enrolment, high glucocorticoid dose, and detection of immune complex (IC) significantly predicted CR off C, while lupus nephritis (LN) and a low C3 level tended to predict it. In the multivariate analysis, IC detection was the only predictor of CR off C. Clinical flares were observed in 5.9% of the achievers during a median 1.2 years after achievement of CR off C. CONCLUSION: In the era of biologics, CR off C was achieved in 28.3% of the patient cohort requiring the start or increase of glucocorticoids for disease activity, with a relatively low rate of flares, suggesting that glucocorticoid-free clinical remission is an achievable target in SLE. IC disease, represented by male sex or nephritis, is likely to benefit from currently available medications.
Assuntos
Produtos Biológicos , Doenças do Complexo Imune , Lúpus Eritematoso Sistêmico , Humanos , Masculino , Glucocorticoides/uso terapêutico , Estudos Retrospectivos , Produtos Biológicos/uso terapêutico , Índice de Gravidade de Doença , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Imunossupressores/uso terapêutico , Doenças do Complexo Imune/tratamento farmacológico , Complexo Antígeno-AnticorpoRESUMO
Polyarteritis nodosa (PAN) is a potentially life-threatening systemic vasculitis, which predominantly involves medium arteries. However, it may be difficult to diagnose PAN in its early stage. The aim of our study was to investigate the sensitivity and specificity of the American College of Rheumatology (ACR) and the Japanese Ministry of Health, Labour and Welfare (MHLW) criteria for the diagnosis of PAN in a single-centre retrospective cohort in Japan and to develop simplified criteria with favourable diagnostic performance. All patients with "PAN" or "suspicion of PAN," as indicated on insurance forms, were included. The patient population was classified into PAN and non-PAN groups based on a retrospective chart review. The sensitivity and specificity of the ACR and MHLW criteria were calculated. Items that favourably discriminated the PAN group from the non-PAN group were determined and used as items for our provisional criteria. Thirteen cases of PAN and 24 cases without PAN were included in this study. The sensitivities of the ACR and MHLW criteria were 61.5% (8/13) and 30.8% (4/13), respectively, whereas the specificities were 79.2% (19/24) and 87.5% (21/24), respectively. We developed provisional criteria consisting of seven items, and found that a cut-off of ≥ 4 items had a sensitivity of 92.3% (12/13) and specificity of 91.7% (22/24) (p < 0.000001). The provisional seven-item criteria, developed in our real-world cohort of patients suspected of having PAN, had a high sensitivity and specificity and may be useful in the diagnosis of PAN, although it should be validated in additional patient populations.
Assuntos
Poliarterite Nodosa/diagnóstico , Adulto , Idoso , Estudos de Casos e Controles , Bases de Dados Factuais , Técnicas de Apoio para a Decisão , Feminino , Humanos , Japão , Masculino , Pessoa de Meia-Idade , Poliarterite Nodosa/fisiopatologia , Estudos Retrospectivos , Sensibilidade e EspecificidadeAssuntos
Dermatomiosite , Doenças Pulmonares Intersticiais , Autoanticorpos , Dermatomiosite/diagnóstico , Dermatomiosite/tratamento farmacológico , Humanos , Helicase IFIH1 Induzida por Interferon , Doenças Pulmonares Intersticiais/induzido quimicamente , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Piperidinas , PirimidinasRESUMO
A 76-year-old man with bronchial asthma was admitted for respiratory failure and bloody sputum. A significant drop in haemoglobin and multiple consolidations supported clinical diagnosis of diffuse alveolar haemorrhage (AH). Myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) was positive and urinalysis suggested glomerulonephritis. Based on eosinophilia, sinusitis, peripheral nerve involvement, and leukocytoclastic vasculitis, he was diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA) associated with AH. Our case-based review suggested that male predominance (65%), high positivity for ANCA (88%), and a high frequency of renal involvement (45%) may be characteristic of AH in EGPA. Although AH is rare in EGPA, we should be aware of this life-threatening complication.
Assuntos
Anticorpos Anticitoplasma de Neutrófilos , Hemorragia , Alvéolos Pulmonares , Humanos , Masculino , Idoso , Hemorragia/etiologia , Hemorragia/diagnóstico , Anticorpos Anticitoplasma de Neutrófilos/sangue , Alvéolos Pulmonares/patologia , Pneumopatias/etiologia , Pneumopatias/diagnóstico , Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/complicações , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/diagnóstico , Peroxidase/imunologiaRESUMO
A 79-year-old woman was evaluated for weakness, dysphagia, and elevated levels of creatinine kinase. Her medical history included stage IIIB cervical cancer eight years previously, which improved after undergoing radiotherapy. Two years later, cancer recurred in the right hilar and mediastinal lymph nodes, and the patient was successfully treated with chemotherapy. Physical examinations showed Gottron's sign, the V sign, the Holster sign, and nailfold erythema. Computed tomography revealed left supraclavicular lymphadenopathy, compatible with cancer recurrence. Anti-nuclear matrix protein 2 (NXP2) antibody was found in her sera and a diagnosis of cancer-associated dermatomyositis was thus made. This case suggests that cancer progression over a period of years may have triggered the onset of autoimmunity against NXP2.
Assuntos
Dermatomiosite , Neoplasias do Colo do Útero , Feminino , Humanos , Idoso , Dermatomiosite/tratamento farmacológico , Neoplasias do Colo do Útero/complicações , Recidiva Local de NeoplasiaRESUMO
Among the myositis-specific antibodies (MSA), anti-transcriptional intermediary factor 1 (TIF1)-γ and anti-nuclear matrix protein 2 (NXP2) antibodies are reportedly associated with cancer-associated myositis (CAM). We aimed to investigate patient characteristics of CAM and the clinical role of cancer-associated MSA (caMSA) in a retrospective cohort from a city hospital. All patients visiting our department between April 2014 and October 2021 with newly diagnosed dermatomyositis, polymyositis, and clinically amyopathic dermatomyositis were included. Anti-TIF1-γ and anti-NXP2 antibodies were collectively considered as caMSA. First, we compared clinical characteristics in CAM, defined as cases showing onset or recurrence of malignancy within 5 years, versus non-CAM. Second, we investigated independent risk factors for CAM. Third, we compared clinical characteristics with and without caMSA within CAM. Finally, we investigated whether caMSA was predictive of poor prognosis. The cohort of 39 patients included 12 (30.7%) CAM cases. Compared with non-CAM, CAM had significantly more dermatomyositis and higher frequencies of dysphagia, anti-TIF1-γ antibody, and caMSA. Using logistic regression analysis, caMSA was an independent risk factor for CAM. In a comparison between caMSA and non-caMSA within CAM, caMSA was associated with higher frequencies of stage ≥ II. However, caMSA did not necessarily indicate a poor prognosis. Only caMSA represented an independent risk factor for CAM and showed a significant association with advanced cancer. Key Points ⢠Cancer-associated MSA was an independent risk factor for cancer-associated myositis. ⢠Cancer-associated MSA was associated with advanced cancer.
Assuntos
Dermatomiosite , Miosite , Neoplasias , Polimiosite , Anticorpos Anti-Idiotípicos , Autoanticorpos , Dermatomiosite/complicações , Hospitais Comunitários , Humanos , Japão , Análise de Mediação , Neoplasias/complicações , Polimiosite/complicações , Estudos RetrospectivosRESUMO
Giant cell arteritis (GCA) occasionally presents with ischaemic stroke. Generally, symptoms related to GCA or elevated levels of inflammation markers would be a clue for the diagnosis of GCA. However, we encountered a rare case of GCA that presented with recurrent cerebellar infarctions without symptoms related to GCA (headache, fever, or jaw claudication). Furthermore, C-reactive protein levels, measured at the time of two of the stroke attacks, were within the normal range. On physical examination, the temporal arteries were prominent and weakly pulsatile. Temporal artery ultrasonography showed halo signs, and temporal artery biopsy revealed GCA. To our knowledge, this is the first case of GCA presenting with recurrent ischaemic stroke lacking GCA features but diagnosed before death. Considering this case-based review, we suggest that GCA may have been missed in elderly patients with ischaemic stroke, especially in those with posterior circulation infarction. Therefore, physical examination of the temporal arteries, temporal artery ultrasonography, and vessel wall magnetic resonance imaging may be useful in those patients.
Assuntos
Isquemia Encefálica , Arterite de Células Gigantes , AVC Isquêmico , Acidente Vascular Cerebral , Idoso , Isquemia Encefálica/complicações , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/patologia , Humanos , Infarto/complicaçõesRESUMO
To relate the clinical findings of parvovirus B19 infection to the phase of the disease, we performed a retrospective chart review of 30 adult patients who tested positive for IgM antibody against parvovirus B19 at our hospital from March 2003 to November 2008. Median patient age was 38 years, with 86.7% aged between 26 and 45 years. The male-to-female ratio was 4:26 (86.7% female). Symptoms in the first phase were mainly flu-like, including fever, headache, or myalgia. Symptoms in the second phase were arthralgia in 24 (85.7%) and rash in 23 (82.1%). Fever was observed in 21 (70.0%), and 22 (75.9%) were found to be lymphopenic. The onsets in 73.3% of cases were concentrated within 10.1% of the study period, an observation nearly consistent with an outbreak of erythema infectiosum. Three patients had symmetrical swelling of joints, all of whom also had rash. Most patients visited the hospital within a week of onset and prognosis was favorable. In the parvovirus B19 infection, flu-like symptoms were frequent in the first phase, while rash and arthralgia were common in the second. Female sex, age between 26 and 45, and presence of rash, arthralgia, fever, and lymphopenia were clinical findings with a high frequency (≥70%), and these factors may contribute to diagnosis. In an era when early diagnosis and therapy is required in rheumatoid arthritis, it is important to recognize the parvovirus B19 infection with a presentation of acute arthritis and a favorable prognosis.
Assuntos
Artralgia/patologia , Edema/patologia , Eritema Infeccioso/patologia , Parvovirus B19 Humano/patogenicidade , Adulto , Idoso , Artralgia/fisiopatologia , Artralgia/virologia , Surtos de Doenças , Edema/fisiopatologia , Edema/virologia , Eritema Infeccioso/complicações , Eritema Infeccioso/fisiopatologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
A 79-year-old woman was admitted for suspected giant cell arteritis (GCA). She had suffered from dizziness, headache, jaw claudication and visual disturbance. Her medical history included bronchial asthma and parasinusitis. Her superficial temporal arteries were markedly enlarged with tenderness. Laboratory data showed eosinophilia (6968/µL) and a positive result of myeloperoxidase-ANCA. A histological examination of the biopsied artery revealed granulomatous inflammation consisting of lymphocytes and eosinophils with a multinucleated giant cell. Her conditions met both the criteria for GCA and eosinophilic granulomatosis with polyangiitis (EGPA). We finally considered that she had temporal arteritis as an initial manifestation of EGPA after a comprehensive literature review. To our knowledge, this is the first case in which temporal arteritis with a giant cell developed as an initial and sole manifestation of EGPA.
Assuntos
Síndrome de Churg-Strauss , Arterite de Células Gigantes , Granulomatose com Poliangiite , Idoso , Síndrome de Churg-Strauss/diagnóstico , Feminino , Arterite de Células Gigantes/etiologia , Granulomatose com Poliangiite/diagnóstico , HumanosRESUMO
Objective We investigated the continuation rate, safety and efficacy of treatment with hydroxychloroquine (HCQ) in a retrospective cohort of systemic lupus erythematosus (SLE) in a Japanese municipal hospital. Methods All of the patients with SLE who started treatment with HCQ were included in this study. A retrospective chart review was performed. Our primary outcomes were the continuation rate of HCQ treatment for 1 year and adverse events (AEs) during the treatment. We also investigated the efficacy of HCQ treatment in cases in which treatment with immunosuppressive therapies remained unchanged for the preceding six months. Results Forty-seven patients with SLE were included in this study. Twenty-five patients (53.2%) had AEs. Eleven (64.7%) of the 17 patients who tried the readministration of HCQ could continue HCQ treatment. The continuation rate of HCQ for a period of 1 year was 78.3% (36 of 46 patients). The development of cutaneous lesions was the most frequent adverse event (25.5%) followed by gastrointestinal symptoms (8.5%). In the 16 cases in which the immunosuppressive therapies remained unchanged for at least six months prior to starting HCQ treatment, the SLE disease activity index, anti-DNA antibody, immune complex, and serum complement activity significantly decreased over a period of 1 year, while the prednisolone dose significantly decreased. Conclusion The continuation rate of HCQ treatment was high in an SLE cohort of a Japanese municipal hospital. Although more than half of the patients experienced AEs, the readministration of HCQ was often successful. HCQ treatment provided benefits regarding the clinical and immunological findings in Japanese patients with SLE, which would likely lead to glucocorticoid tapering.
Assuntos
Antirreumáticos/uso terapêutico , Hidroxicloroquina/efeitos adversos , Hidroxicloroquina/uso terapêutico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/fisiopatologia , Adulto , Estudos de Coortes , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos , Feminino , Hospitais Municipais/estatística & dados numéricos , Humanos , Japão/epidemiologia , Lúpus Eritematoso Sistêmico/epidemiologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do TratamentoRESUMO
Sjögren syndrome (SS) is one of several collagen vascular diseases that occasionally involve the central nervous system. We report two cases of SS involving young patients who initially presented with aseptic meningitis. A male with recurrent AM was found to have anti-Ro/SSA and La/SSB antibodies in a screening test for autoimmune process. A minor salivary gland biopsy revealed lymphocytic infiltrations compatible with SS, although the patient did not exhibit sicca symptoms. A female presenting with AM and polyarthritis also reported xerophthalmia. Anti-Ro/SSA antibody testing and a positive result in a minor salivary gland biopsy led to the diagnosis of SS. In the literature review, we found that AM or aseptic meningoencephalitis (AME) preceded or had a concomitant onset with SS in approximately 70% of cases. Screening for anti-Ro/SSA antibody, as well as systemic assessment for rheumatic symptoms, may be useful for diagnosing AM/AME of unknown etiology.
Assuntos
Meningite Asséptica/diagnóstico por imagem , Meningite Asséptica/diagnóstico , Síndrome de Sjogren/diagnóstico por imagem , Síndrome de Sjogren/diagnóstico , Adulto , Humanos , Imageamento por Ressonância Magnética , Masculino , Meningoencefalite/diagnóstico , Meningoencefalite/diagnóstico por imagem , Adulto JovemRESUMO
A 62-year-old female patient with systemic lupus erythematosus (SLE) was admitted for cerebral infarction. The magnetic resonance angiography showed focal narrowing of the cerebral arteries that was initially considered as atherosclerosis due to her cardiovascular risk factors. Ten weeks later, she was again admitted for multiple cerebral infarctions. Vessel wall magnetic resonance imaging revealed gadolinium enhancement of the arterial walls of the narrowing lesions, leading to a diagnosis of cerebral arteritis. Based on a literature review, cerebral medium-sized arteritis in SLE likely progresses insidiously during the active phase of SLE, which may later result in occlusion irrespective of disease activity.
Assuntos
Arterite , Infarto Cerebral , Ciclofosfamida/uso terapêutico , Lúpus Eritematoso Sistêmico , Vasculite Associada ao Lúpus do Sistema Nervoso Central , Angiografia por Ressonância Magnética/métodos , Paresia , Antirreumáticos/uso terapêutico , Arterite/diagnóstico por imagem , Arterite/etiologia , Infarto Cerebral/diagnóstico , Infarto Cerebral/etiologia , Infarto Cerebral/fisiopatologia , Infarto Cerebral/terapia , Imagem de Difusão por Ressonância Magnética/métodos , Progressão da Doença , Feminino , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/terapia , Vasculite Associada ao Lúpus do Sistema Nervoso Central/complicações , Vasculite Associada ao Lúpus do Sistema Nervoso Central/diagnóstico , Pessoa de Meia-Idade , Paresia/diagnóstico , Paresia/etiologia , Paresia/terapia , Gravidade do Paciente , Prognóstico , RecidivaRESUMO
AL amyloidosis (AL) is a systemic disorder due to extracellular tissue deposition of amyloid fibrils, composed of immunoglobulin light chains. Since the description of AL involving temporal arteries in 1986, this disorder has been known as one of the differential diagnoses of giant cell arteritis (GCA). We encountered a case of an elderly female presenting with headache and tender and enlarged temporal arteries, that was pathologically diagnosed with temporal artery involvement of AL due to Bence-Jones-type MM. To our knowledge, this was the first case of AL with temporal artery involvement in Japan, that presented with GCA-like features. Literature review of AL cases with temporal artery involvement showed close similarity between these disorders, but suggested that vasculature involvement (extremity claudication, kidney or heart), macroglossia, carpal tunnel syndrome and normal or low (<0.5 mg/dL) CRP levels may predict AL rather than GCA. Physicians should keep in mind that AL involving temporal arteries can be a pitfall in the diagnosis of GCA, as seen in our and previous cases.
Assuntos
Arterite de Células Gigantes/diagnóstico , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico , Artérias Temporais/patologia , Amiloide , Proteína de Bence Jones , Biomarcadores , Biópsia , Diagnóstico Diferencial , Arterite de Células Gigantes/etiologia , Arterite de Células Gigantes/metabolismo , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/etiologia , Amiloidose de Cadeia Leve de Imunoglobulina/metabolismoRESUMO
A 45-year-old man was admitted with acute abdominal pain and eosinophilia. Abdominal computed tomography revealed thickness of the ascending and transverse colon with decreased contrast enhancement and a small amount of ascites. In an emergency operation, the necrotic colon was resected. Histopathology showed subserous medium-sized arteritis with abundant eosinophil infiltrates and thrombosis in the portal vein branches. He was diagnosed with polyarteritis nodosa (PAN), and immunosuppressive therapy improved his condition. Two years later, the disease recurred with ischemic cutaneous lesions and marked eosinophilia. Our experience suggests that marked eosinophilia in PAN may imply severe organ involvement, including gastrointestinal necrosis, as well as the association of venous thrombosis.
Assuntos
Eosinofilia/diagnóstico , Gastroenteropatias/etiologia , Poliarterite Nodosa/diagnóstico , Trombose Venosa/etiologia , Eosinofilia/complicações , Gastroenteropatias/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Poliarterite Nodosa/complicações , Recidiva , Tomografia Computadorizada por Raios X , Trombose Venosa/diagnósticoRESUMO
Objectives To investigate the sensitivity and specificity of a temporal artery biopsy (TAB) in the diagnosis of giant cell arteritis (GCA) in a single-center retrospective cohort in Japan. Methods A retrospective chart review was performed on consecutive patients who visited our hospital between April 2009 and October 2018 and underwent a TAB. The sensitivity and specificity were calculated for the three pathological standards for a TAB, predetermined according to the pathological criterion of the 1990 American College of Rheumatology (ACR) criteria: A) vasculitis characterized by predominant mononuclear cell infiltration; B) vasculitis with granulomatous inflammation; and C) vasculitis with multinucleated giant cells. We also analyzed the clinical parameters predicting the diagnosis of GCA and the impact of a diagnostic delay of ≥3 months on cardiovascular complications of GCA. Results Our study population was 16 cases in the GCA group and 13 in the non-GCA group. The sensitivity and specificity for Standard A of a TAB were 81% and 85%, respectively, while those for stricter Standards B or C were identical, at 75% and 100%, respectively. These pathological standards, but not any other parameters, significantly predicted the diagnosis. A diagnostic delay tended to cause cardiovascular complications (p=0.057). Conclusion The sensitivity and specificity of the pathological standards of a TAB were favorable in our cohort and were the only predictors for the diagnosis of GCA. Considering the possible impact of a diagnostic delay on cardiovascular complications, the early recognition and prompt initiation of glucocorticoid therapy is needed, even in Japan, where GCA is uncommon.
Assuntos
Doenças Cardiovasculares/etiologia , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/diagnóstico , Artérias Temporais/patologia , Idoso , Idoso de 80 Anos ou mais , Biópsia , Doenças Cardiovasculares/patologia , Estudos de Coortes , Diagnóstico Tardio/efeitos adversos , Feminino , Humanos , Japão , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Sensibilidade e Especificidade , Vasculite/patologiaAssuntos
Células Dendríticas/patologia , Neoplasias Hematológicas/complicações , Doenças Pulmonares Intersticiais/etiologia , Pulmão/patologia , Idoso de 80 Anos ou mais , Anti-Inflamatórios/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Diagnóstico Tardio , Evolução Fatal , Feminino , Neoplasias Hematológicas/diagnóstico por imagem , Neoplasias Hematológicas/tratamento farmacológico , Neoplasias Hematológicas/patologia , Humanos , Doenças Pulmonares Intersticiais/tratamento farmacológico , Imagem Multimodal , Especificidade de Órgãos , Tomografia por Emissão de Pósitrons , Prednisolona/uso terapêutico , Pele/patologia , Tomografia Computadorizada por Raios XRESUMO
Muscle weakness is rarely a presenting symptom of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), although the disease frequently involves the lungs, skin, neurons, and kidneys. Here we describe a case of AAV presenting with muscle weakness in which only muscle biopsy could confirm the diagnosis. The literature review, including three similar cases, suggested that patients with ANCA-associated muscle vasculitis likely had myalgia, normal levels of creatine kinase, pulmonary fibrosis, rheumatoid factor, and muscle edema on MRI.