RESUMO
BACKGROUND AND OBJECTIVE: Systemic sclerosis (SSc) is a highly heterogeneous disease whose treatment is based mainly on immunosuppressants, antifibrotics, and vasodilators. Intravenous immunoglobulin (IVIG) have proved effective in other autoimmune diseases. The objective of this study is to evaluate the efficacy and safety of IVIG in SSc. METHODS: The systematic review was conducted according to the PRISMA Statement. Medline, Embase and Cochrane Library databases were searched until March 2024. We assessed the quality of included studies using the Cochrane Risk of Bias 2.0 tool (RoB 2) for randomised clinical trials and the Cochrane Risk in non-randomized studies (ROBINS-I) tool for observational studies. RESULTS: From 1242 studies identified, 15 studies were included, of which 14 were observational studies. In total, 361 patients with SSc were included, and 295 received treatment with IVIG. Most of the studies used a dose of 2 g/kg IVIG. Ten studies, including the clinical trial, showed high risk of bias, and five had a critical risk. Skin involvement was assessed using modified Rodnan skin score, in 11 studies and the authors reported cutaneous efficacy in 9 of them. The 6 studies that assessed muscle involvement reported an improvement. Six studies reported data on gastrointestinal efficacy. Other domains such as lung and joint involvement and steroid-sparing effect were evaluated. The most frequent adverse events were mild, including headache, abdominal pain, fever, and skin rash. CONCLUSION: Treatment with IVIG in SSc patients could be helpful and safe in patients with cutaneous, muscular, or digestive manifestations.
RESUMO
INTRODUCTION: Interstitial lung diseases associated with systemic autoimmune diseases (ILD-SAD) can progress to a fibrotic form that can benefit from antifibrotic treatment. The aim of the study is to describe a cohort of patients with ILD-SAD who manifest progressive pulmonary fibrosis treated with antifibrotics. METHODS: Single-centre retrospective observational study from a tertiary care hospital on a cohort of patients with ILD-SAD with progressive pulmonary fibrosis evaluated in a joint pulmonology and rheumatology clinic that initiated treatment with antifibrotic drugs between 01/01/2019 and 01/12/2021. Clinical characteristics were analysed. The evolution of pulmonary function test and adverse effects during treatment were described. RESULTS: 18 patients were included. The mean age was 66.7⯱â¯12.7 years, with a higher frequency of females (66.7%). Systemic sclerosis (SS) was the most frequent systemic autoimmune disease (36.8%). The majority of patients were receiving systemic glucocorticoid treatment (88.9%), 72.2% of patients were receiving treatment with disease-modifying drugs, the most frequent being mycophenolate mofetil (38.9%), and 22.2% with rituximab. Functional stability was observed after the start of antifibrotic treatment. Two patients died during follow-up, one due to progression of ILD. CONCLUSION: Our study suggests a beneficial effect of antifibrotic treatment added to immunomodulatory treatment in patients with fibrotic ILD-SAD in real life. In our cohort, patients with ILD-SAD with progressive fibrosing involvement show functional stability after starting antifibrotic treatment. Treatment tolerance was relatively good with a side effect profile similar to that described in the medical literature.
Assuntos
Doenças Autoimunes , Doenças Pulmonares Intersticiais , Fibrose Pulmonar , Escleroderma Sistêmico , Feminino , Humanos , Pessoa de Meia-Idade , Idoso , Fibrose Pulmonar/complicações , Fibrose Pulmonar/tratamento farmacológico , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/complicações , Fibrose , Escleroderma Sistêmico/complicações , Doenças Autoimunes/complicações , Doenças Autoimunes/tratamento farmacológicoRESUMO
Rheumatoid meningitis (RM) is a rare complication of rheumatoid arthritis that can manifest as stroke-like episodes. We present the case of a 63-year-old woman with a past history of overlap syndrome and clinical manifestations suggestive of amyopathic dermatomyositis, rheumatoid arthritis, and systemic lupus erythematosus. She presented to the emergency department with sudden onset right-sided clumsiness and numbness, as well as a 2-week history of left hemicranial headache. Laboratory workup revealed positive serum antinuclear antibodies, anti-Ro antibodies, anti-citrullinated peptide antibodies (ACPA), and elevated rheumatoid factor. Lymphocytic pleocytosis, positive ACPA and anti-Ro antibodies with passive diffusion pattern, and negative microbiological studies were demonstrated in the CSF. Brain magnetic resonance imaging showed predominant left fronto-parieto-occipital leptomeningeal and pachimeningeal enhancement. She was diagnosed with RM and received methylprednisolone IV mg/kg once daily. Stroke-like episodes in the setting of a patient with lymphocytic pleocytosis in the cerebrospinal fluid (CSF) and meningeal enhancement should raise suspicion of RM. In this context, serum rheumatoid factor and ACPA levels should always be measured and ACPA should also be measured in CSF. To our knowledge, this is the first reported case of RM in the context of an overlap syndrome. ACPA levels in CSF could be a relevant diagnostic clue in the setting of central nervous system disturbance and overlapping autoimmune conditions that include rheumatoid arthritis. In our case, the presence of a suggestive clinical scenario of RM reinforces the probable pathogenic role of ACPA when it is present in the central nervous system, even without intrathecal synthesis evidence.
Assuntos
Artrite Reumatoide , Meningite , Acidente Vascular Cerebral , Feminino , Humanos , Pessoa de Meia-Idade , Fator Reumatoide , Anticorpos Antiproteína Citrulinada , Leucocitose/complicações , Artrite Reumatoide/complicações , Artrite Reumatoide/diagnóstico , Meningite/diagnóstico , Meningite/etiologia , SíndromeAssuntos
Eosinofilia , Fasciite , Adulto , Eosinofilia/diagnóstico , Fasciite/diagnóstico , Feminino , HumanosRESUMO
Antisynthetase Syndrome (ASS) is a subset of idiopathic inflammatory myopathies characterised by specific clinical features such as interstitial lung disease (ILD), fever, myositis, Raynaud's phenomenon, cutaneous involvement and arthritis related to the presence of anti-aminoacyl-tRNA-synthetase (anti-ARS) autoantibodies. Moreover, Pulmonary arterial hypertension (PAH) is a life-threatening complication associated with connective tissue diseases mainly systemic sclerosis (SSc-PAH). It has been suggested that PAH can complicate ASS patients but little is known about the prevalence and risk factors to develop this complication. Here we report on two patients with ASS and PH. The first one represents a complete picture of ASS anti-Jo-1 positive, the second an amyophatic ASS anti-PL-12 positive. In one of our ASS-PAH patients, specific treatment lead to improvement of PAH. There are no specific recommendations on current guidelines regarding either PAH screening or treatment in ASS, but performing echocardiogram, ECG, pulmonary function test and prompt initiation of specific therapies seems to improve right heart catheterisation (RHC) parameters and survival.
Assuntos
Miosite/complicações , Hipertensão Arterial Pulmonar/complicações , Aminoacil-tRNA Sintetases/imunologia , Autoanticorpos/sangue , Feminino , Humanos , Pessoa de Meia-Idade , Miosite/imunologia , Hipertensão Arterial Pulmonar/imunologia , Tomografia Computadorizada por Raios XAssuntos
Granulomatose com Poliangiite , Neoplasias Pulmonares , Nódulo Pulmonar Solitário , Fluordesoxiglucose F18 , Granulomatose com Poliangiite/diagnóstico , Humanos , Neoplasias Pulmonares/diagnóstico , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Nódulo Pulmonar Solitário/diagnóstico por imagemAssuntos
Infecções por HIV , Processamento de Imagem Assistida por Computador/métodos , Fraturas por Osteoporose , Radiografia Torácica/métodos , Esterno , Tomografia Computadorizada por Raios X/métodos , Detecção Precoce de Câncer/métodos , Infecções por HIV/complicações , Infecções por HIV/diagnóstico , Humanos , Achados Incidentais , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/prevenção & controle , Masculino , Pessoa de Meia-Idade , Fraturas por Osteoporose/diagnóstico por imagem , Fraturas por Osteoporose/etiologia , Esterno/diagnóstico por imagem , Esterno/lesõesRESUMO
Sarcoidosis and tuberculosis are two common granulomatous conditions that may share clinical and radiological presentations. The galaxy sign (sarcoid galaxy sign) is a characteristic radiological sign of pulmonary sarcoidosis on thoracic computed tomography (CT). We present the case of a patient with sarcoidosis that was initially misdiagnosed as tuberculosis, in whom the galaxy sign on CT was useful as it suggested the correct diagnosis.
Assuntos
Sarcoidose Pulmonar/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Tuberculose Pulmonar/diagnóstico por imagem , Adulto , Diagnóstico Diferencial , Feminino , HumanosAssuntos
Doenças Pulmonares Intersticiais/diagnóstico por imagem , Neoplasias Pulmonares/diagnóstico por imagem , Linfoma Difuso de Grandes Células B/diagnóstico por imagem , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Ciclofosfamida/administração & dosagem , Doxorrubicina/administração & dosagem , Humanos , Doenças Pulmonares Intersticiais/complicações , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/tratamento farmacológico , Linfoma Difuso de Grandes Células B/complicações , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Masculino , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Prednisona/administração & dosagem , Rituximab/administração & dosagem , Vincristina/administração & dosagemAssuntos
Aminoacil-tRNA Sintetases/imunologia , Azatioprina/administração & dosagem , Calcinose , Doenças Pulmonares Intersticiais , Miosite , Músculos Paraespinais , Prednisona/administração & dosagem , Tomografia Computadorizada por Raios X/métodos , Autoanticorpos/sangue , Calcinose/diagnóstico por imagem , Calcinose/etiologia , Feminino , Humanos , Imunossupressores/administração & dosagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/terapia , Pessoa de Meia-Idade , Miosite/diagnóstico , Miosite/imunologia , Miosite/fisiopatologia , Miosite/terapia , Músculos Paraespinais/diagnóstico por imagem , Músculos Paraespinais/patologiaAssuntos
Abscesso/microbiologia , Nádegas/microbiologia , Sacroileíte/microbiologia , Infecções Estafilocócicas/diagnóstico , Abscesso/diagnóstico , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Sacroileíte/diagnóstico por imagem , Infecções Estafilocócicas/complicações , Tomografia Computadorizada por Raios XAssuntos
Hiperpigmentação/diagnóstico , Doenças da Unha/diagnóstico , Unhas/patologia , Escleroderma Sistêmico/diagnóstico , Dermatopatias/diagnóstico , Biópsia , Proliferação de Células , Comorbidade , Feminino , Dedos , Humanos , Hiperpigmentação/patologia , Hiperpigmentação/fisiopatologia , Melanócitos/patologia , Pessoa de Meia-Idade , Doenças da Unha/patologia , Doenças da Unha/fisiopatologia , Escleroderma Sistêmico/patologia , Escleroderma Sistêmico/fisiopatologia , Dermatopatias/patologia , Dermatopatias/fisiopatologiaRESUMO
Chondrodermatitis nodularis chronica helicis is a rare non-neoplastic inflammatory and degenerative process of the external ear, characterized by necrobiotic changes in the dermis that extend down to the perichondrium. This condition has been occasionally reported in patients with limited cutaneous systemic sclerosis but not in those with concomitant primary biliary cirrhosis; this association is known as Reynolds syndrome. We report a 70-year-old woman diagnosed with primary biliary cirrhosis at age 47 and with limited cutaneous systemic sclerosis at age 54 who developed a painful ulcerated nodule on the helical rim of the left ear shortly after the last diagnosis. The lesion was excised because of the suspicion of malignancy, but the histopathology was consistent with chondrodermatitis nodularis chronica helicis. Although this condition is infrequent, it is necessary to know, because it may occur in patients with systemic sclerosis and be mistaken for neoplasms, such as basal cell and squamous cell carcinoma, and these patients have an increased risk for the development of skin malignancies.