Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 21.305
Filtrar
1.
Rheum Dis Clin North Am ; 49(1): 45-66, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-36424026

RESUMO

Systemic autoimmune rheumatic diseases (SARDs) are defined by the potential to affect multiple organ systems, and cardiac involvement is a prevalent but often overlooked sequela. Myocardial involvement in SARDs is medicated by macrovascular disease, microvascular dysfunction, and myocarditis. Systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, eosinophilic granulomatosis with polyangiitis, and sarcoidosis are associated with the greatest risk of myocardial damage and heart failure, though myocardial involvement is also seen in other SARDs or their treatments. Management of myocardial involvement should be disease-specific. Further research is required to elucidate targetable mechanisms of myocardial involvement in SARDs.


Assuntos
Artrite Reumatoide , Síndrome de Churg-Strauss , Granulomatose com Poliangiite , Sarcoidose , Humanos , Progressão da Doença
4.
Chest ; 162(5): e245-e248, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-36344132

RESUMO

Although sarcoidosis is an established cause of multiorgan dysfunction, acute presentation with thrombotic microangiopathy resulting in severe renal and hematological sequelae has not been reported. We describe the case of a patient presenting with hypercalcemia, pancreatitis, and acute renal failure, followed by microangiopathic hemolytic anemia. Although there were no significant respiratory symptoms, thoracic radiology and mediastinal lymph node biopsy results were in keeping with sarcoidosis as the underlying cause of this multisystem presentation. Corticosteroids were commenced with clinical and biochemical improvement. This novel case highlights the need to consider sarcoidosis as part of the differential diagnosis for unusual multiorgan presentations and for early multidisciplinary involvement in such cases to permit optimal treatment.


Assuntos
Injúria Renal Aguda , Sarcoidose , Microangiopatias Trombóticas , Humanos , Microangiopatias Trombóticas/diagnóstico , Microangiopatias Trombóticas/etiologia , Rim , Injúria Renal Aguda/terapia , Biópsia/efeitos adversos , Sarcoidose/complicações , Sarcoidose/diagnóstico , Sarcoidose/patologia
7.
JACC Cardiovasc Imaging ; 15(11): 1944-1955, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-36357136

RESUMO

BACKGROUND: Patients with suspected cardiac sarcoidosis frequently undergo fluorodeoxyglucose (FDG)-positron emission tomography (PET)/computed tomography (CT) imaging to assess disease activity at baseline and after treatment initiation. OBJECTIVES: This study investigated the effect of immunosuppressive therapy and biopsy status to achieve complete treatment response (CTR), partial treatment response (PTR), or no response (NR) on myocardial FDG-PET/CT. METHODS: This study analyzed 83 patients with suspected cardiac sarcoidosis (aged 53 ± 1.8 years, 71% were male, 69% were White, 61% had a history of biopsy-confirmed sarcoidosis) who were treatment naive, had evidence of myocardial FDG at baseline, and underwent repeat PET imaging after treatment initiation. CTR was graded visually, and PTR/NR were measured both visually and quantitatively using the total glycolytic activity. Patients were also evaluated for the occurrence of death, sustained ventricular arrhythmias, and heart failure admissions. RESULTS: Overall, 59 patients (71%) achieved CTR/PTR (30%/41%) at follow-up scan (P = 0.04). Total glycolytic activity and visual estimate of PTR/NR had excellent agreement (κ = 0.86 [95% CI: 0.72-0.99]; P < 0.0001). In patients receiving prednisone only, the highest rates of CTR/PTR were observed in patients initiated on moderate or high dose (P < 0.01). In a regression model, moderate prednisone start dose (P = 0.03) was more strongly associated with achieving CTR/PTR than was high prednisone start dose. However, the latter patients were tapered faster between start dose and follow-up scan (P < 0.01). After a median follow-up of 4.7 (IQR: 3.1-7.8) years, patients who were biopsy-proven (vs non-biopsy-proven; P = 0.029) and with preserved left ventricular function (P = 002) were less likely to experience major adverse cardiac events. Outcomes based on treatment response status (CTR vs PTR vs NR; P = 0.23) were not significantly different. CONCLUSIONS: Among patients with suspected sarcoidosis and evidence of myocardial inflammation, treatment response by serial FDG-PET was variable, but a favorable response was more common when using moderate-to-high intensity prednisone dose. Biopsy-proven individuals and those with preserved systolic function were less likely to experience adverse outcomes during follow-up.


Assuntos
Cardiomiopatias , Miocardite , Sarcoidose , Humanos , Masculino , Feminino , Fluordesoxiglucose F18 , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Compostos Radiofarmacêuticos , Prednisona , Cardiomiopatias/diagnóstico por imagem , Cardiomiopatias/tratamento farmacológico , Cardiomiopatias/patologia , Valor Preditivo dos Testes , Sarcoidose/diagnóstico por imagem , Sarcoidose/tratamento farmacológico , Sarcoidose/patologia , Tomografia por Emissão de Pósitrons/métodos , Terapia de Imunossupressão
9.
BMJ Case Rep ; 15(11)2022 Nov 24.
Artigo em Inglês | MEDLINE | ID: mdl-36423948

RESUMO

Sarcoidosis is an immune-mediated inflammatory disorder with unknown aetiology that is marked by non-caseating granulomas in affected organs. Pulmonary sarcoidosis is the most common manifestation, but gastrointestinal involvement, particularly in the small bowel, is exceedingly rare. While symptom-driven treatment guidelines that are steroid based are well established for pulmonary and few extrapulmonary manifestations (ie, cardiac, neurologic, renal), gastrointestinal sarcoidosis treatment is largely extrapolation with optimal management under investigation. Additionally, few works document small bowel obstruction related to small bowel sarcoidosis. We present a case of short-interval recurrent small bowel obstruction in a man in his sixties that revealed newly diagnosed sarcoidosis with suspected small bowel involvement who never underwent steroid therapy. The patient exhibited gastrointestinal symptoms, despite asymptomatic pulmonary disease and a course of prednisone may have reduced his risk of recurrence. We also review suggested gastrointestinal sarcoidosis treatment and surveillance guidelines with focus on the small bowel.


Assuntos
Obstrução Intestinal , Sarcoidose , Masculino , Humanos , Sarcoidose/complicações , Sarcoidose/diagnóstico , Sarcoidose/tratamento farmacológico , Obstrução Intestinal/etiologia , Intestino Delgado/diagnóstico por imagem , Granuloma , Doença Crônica
11.
Medicine (Baltimore) ; 101(40): e30883, 2022 Oct 07.
Artigo em Inglês | MEDLINE | ID: mdl-36221396

RESUMO

RATIONALE: There are many causes of hypercalcemia, with hyperparathyroidism and malignancy accounting for 90% of cases. Sarcoidosis and the intake of vitamin D supplements may also cause hypercalcemia, although the occurrence rate is low if only one is involved. We herein report a sarcoidosis patient who developed hypercalcemia after taking cholecalciferol (vitamin D supplement) for a year. PATIENT CONCERN: A 62-year-old Japanese man presented with hypercalcemia and acute kidney injury along with symptoms of fatigue and appetite loss while being followed up for sarcoidosis. DIAGNOSES: We determined that a combination of cholecalciferol supplementation and sarcoidosis had led to hypercalcemia for several reasons. First, hypercalcemia had not been noted when this patient had first been admitted due to sarcoidosis-related respiratory failure several years earlier, which we presumed that was the highest sarcoidosis disease activity. Second, low serum 25-OH Vit.D3 and high 1,25-(OH)2 Vit.D3 levels were noted despite cholecalciferol supplementation for a year, suggesting that 1-α-hydroxylase overexpression caused by sarcoidosis accelerated the conversion from 25-OH Vit.D3 to 1,25-(OH)2 Vit.D3. INTERVENTIONS: Although initially resistant to preservative management, the hypercalcemia promptly improved after starting corticosteroid treatment. OUTCOMES: Hypercalcemia and acute kidney injury were normalized after corticosteroid treatment. LESSONS: We should be aware of patients' medications, especially in patients with granulomatosis disease. The concomitant measurement of 25-OH Vit.D3 and 1,25-(OH)2 Vit.D3 levels is useful for determining the cause of hypercalcemia.


Assuntos
Injúria Renal Aguda , Hipercalcemia , Sarcoidose , Injúria Renal Aguda/induzido quimicamente , Injúria Renal Aguda/complicações , Cálcio , Colecalciferol/efeitos adversos , Suplementos Nutricionais/efeitos adversos , Humanos , Hipercalcemia/induzido quimicamente , Hipercalcemia/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Oxigenases de Função Mista , Sarcoidose/complicações , Sarcoidose/tratamento farmacológico , Vitamina D/uso terapêutico
12.
Sci Rep ; 12(1): 16906, 2022 Oct 07.
Artigo em Inglês | MEDLINE | ID: mdl-36207373

RESUMO

Sarcoidosis is a systemic granulomatous disease of unknown etiology with significant heterogeneity in organ manifestations and clinical course. Subjects with sarcoidosis share several features such as, non-necrotizing granuloma, hypergammaglobulinemia, increased local and circulating inflammatory cytokines. Macrophage migration inhibitory factor (MIF) is a pluripotent chemokine modulating cellular function. Study included healthy controls (n = 28) and sarcoidosis patients (n = 65). Sera and BAL of sarcoidosis patients were collected and patients were followed longitudinally for 3 years, and demographics, stages, pulmonary function tests, and organ involvements were recorded. We evaluated MIF in the serum and bronchoalveolar lavage (BAL) fluid of sarcoidosis patients in association with clinical features and cytokines, IL-18, IL-10, IL-6, IFN-γ. We found serum MIF had a positive correlation with IL-10 and IFN-γ and % predicted total lung capacity (%TLC). Serum IL-18 had a significant positive correlation with serum lysozyme, but a negative correlation with %TLC and %DLCO. We identified two groups of sarcoidosis subjects with distinct clinical and cytokine features. A group with prominent extrapulmonary involvement, and low serum MIF, IL-10 and IFN-γ and a group with elevated serum MIF, IL-10 and IFN-γ levels. Our work provides understanding of phenotypic diversity in association with heterogeneity in cytokine landscape in sarcoidosis.


Assuntos
Fatores Inibidores da Migração de Macrófagos , Sarcoidose , Líquido da Lavagem Broncoalveolar , Citocinas , Humanos , Interleucina-10 , Interleucina-18 , Interleucina-6 , Muramidase
13.
BMC Infect Dis ; 22(1): 770, 2022 Oct 04.
Artigo em Inglês | MEDLINE | ID: mdl-36192705

RESUMO

BACKGROUND: Sarcoidosis is a systemic inflammatory disease that is characterized by non-caseating epithelioid-cell granulomas upon histology. However, similar histological findings may also be seen with certain infections. Thus, differentiation from infection is pivotal to ensure appropriate treatment. Here, we present a case of a disseminated infection with Mycobacterium genavense owing to an interleukin 12 receptor subunit beta 1 (IL-12Rß1) associated immunodeficiency in a previously healthy female who was initially misdiagnosed with sarcoidosis. M. genavense is a nontuberculous mycobacterium which can cause lymphadenopathy, gastrointestinal and bone marrow infiltration in immunocompromised patients. With this case report we aim to highlight that an infection with M. genavense on the ground of a genetic defect of mycobacterial immune control may represent a rare differential diagnosis of sarcoidosis. CASE PRESENTATION: A 31-year-old female was referred to our hospital with progressive lymphadenopathy, hepatosplenomegaly, pancytopenia and systemic inflammation. She had previously been evaluated for generalized lymphadenopathy in another hospital. At that time, lymph node biopsies had revealed sarcoid-like lesions and a systemic corticosteroid treatment was initiated based on a putative diagnosis of sarcoidosis. When her condition worsened, she was transferred to our university clinic, where the diagnosis of disseminated M. genavense infection owing to an inborn interferonopathy was made. Her family history revealed that her brother had also suffered from IL-12Rß1 deficiency and had died from a systemic infection with M. genavense at the age of 21. The patient received antimycobacterial treatment combined with subcutaneous type I interferon, which eventually led to a gradual improvement over the next months. CONCLUSIONS: Differentiating between sarcoidosis and sarcoid-like lesions secondary to infections may be challenging, especially when pathogens are difficult to detect or not expected in an apparently immunocompetent patient. Patients with IL-12Rß1-associated immunodeficiency may be asymptomatic until adulthood, and disseminated M. genavense infection on the grounds of an IL-12Rß1-associated immunodeficiency may represent a rare differential diagnosis of sarcoidosis.


Assuntos
Síndromes de Imunodeficiência , Interferon Tipo I , Linfadenopatia , Infecções por Mycobacterium não Tuberculosas , Infecções por Mycobacterium , Sarcoidose , Adulto , Feminino , Humanos , Síndromes de Imunodeficiência/complicações , Linfadenopatia/complicações , Masculino , Mycobacterium , Infecções por Mycobacterium/complicações , Infecções por Mycobacterium/diagnóstico , Infecções por Mycobacterium/tratamento farmacológico , Infecções por Mycobacterium não Tuberculosas/complicações , Infecções por Mycobacterium não Tuberculosas/diagnóstico , Infecções por Mycobacterium não Tuberculosas/tratamento farmacológico , Micobactérias não Tuberculosas/genética , Receptores de Interleucina-12/genética , Sarcoidose/diagnóstico
14.
Front Immunol ; 13: 932919, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36189202

RESUMO

Objectives: Blau syndrome (BS) is a rare autoinflammatory disease characterized by arthritis, dermatitis, and granulomatous uveitis in early childhood. The study presents the clinical experience of patients with BS at a tertiary care centre in Chandigarh, North India. Methods: Analysis of the clinical profile of patients of BS with NOD2 gene mutations under follow-up was carried out. Results: Diagnosis of BS was genetically confirmed in 11 patients (10 children and one adult; six male and five female patients) from 10 families. The median age of onset of symptoms was 12 months (range, 4 months-4 years), while the age at diagnosis ranged from 2.3 to 26 years. The classic triad of arthritis, dermatitis, and uveitis was present in 6/11 (54.5%) patients. The frequency of arthritis, dermatitis, and uveitis was 100%, 81.8%, and 72.7%, respectively. The median age at diagnosis of ocular symptoms was 4 years (range, 2-26 years). Family history was noted in six families. Renal involvement was observed in two children. All patients in our cohort had the R334W variant in NOD2 gene. An asymptomatic carrier sibling with R334W mutation was identified in one family. Methotrexate was used as a first-line agent in all children. Adalimumab, which was commenced in five patients with uveitis, resulted in significant improvement in four patients. The total follow-up duration of the present cohort is 1,063.8 patient-months. Conclusions: The possibility of BS should always be considered in patients with arthritis and early ocular involvement. Uveitis is often progressive and refractory to currently available therapies. Systemic involvement appears to remain a significant cause of morbidity and mortality.


Assuntos
Artrite , Dermatite , Uveíte , Adalimumab/uso terapêutico , Adolescente , Adulto , Artrite/diagnóstico , Artrite/tratamento farmacológico , Artrite/genética , Criança , Pré-Escolar , Dermatite/genética , Feminino , Humanos , Lactente , Masculino , Metotrexato/uso terapêutico , Proteína Adaptadora de Sinalização NOD2/genética , Sarcoidose , Sinovite , Centros de Atenção Terciária , Uveíte/diagnóstico , Uveíte/tratamento farmacológico , Uveíte/genética , Adulto Jovem
15.
Artigo em Inglês | MEDLINE | ID: mdl-36232221

RESUMO

A 44-year-old woman was admitted to hospital with end-stage renal failure, productive cough, and decreased exercise tolerance. She had owned nine cats, which resulted in long-term exposure (18 years) to silica-containing bentonite cat litter. High-resolution computed tomography of the chest showed micronodular lesions in the lungs, and mild mediastinal lymphadenopathy. A lung biopsy revealed multinucleated giant cells, some of which had birefringent material and Schaumann bodies. X-ray photoelectron spectroscopy revealed the presence of silicon in the lung biopsy specimen, as well as in the patient's cat litter. The pulmonary condition was suggestive of sarcoid-like lung disease, rather than silicosis, sarcoidosis, or hypersensitivity pneumonitis, according to the clinicopathological findings. Renal failure appeared to be a result of chronic hypercalcemia due to extrarenal calcitriol overproduction in activated alveolar macrophages. Ultimately, the patient was diagnosed with sarcoid-like lung disease complicated by end-stage renal failure from exposure to bentonite cat litter. Therapy with steroids, in addition to elimination of the bentonite cat litter exposure, resulted in a significant improvement in the health condition. At a follow-up visit after 4 months, an almost complete resolution of the lung lesions and a significant improvement in renal function were observed.


Assuntos
Falência Renal Crônica , Pneumopatias , Sarcoidose , Dermatopatias , Bentonita , Calcitriol , Feminino , Humanos , Sarcoidose/diagnóstico , Silício , Dióxido de Silício/toxicidade
16.
Rev. esp. patol. torac ; 34(3): 186-188, Oct. 2022. ilus, tab
Artigo em Espanhol | IBECS | ID: ibc-210687

RESUMO

Se trata de un varón de 34 años que tras regresar de un viaje transatlántico consulta por dolor pleurítico derecho, malestar general, sudoración y tos productiva. Presenta alteraciones radiológicas compatibles con tromboembolismo pulmonar (TEP) bilateral, patrón micronodular y adenopatías mediastínicas que podrían estar en relación con algún proceso infeccioso-inflamatorio por el que inicia estudio. Tras aplicar una amplia estrategia diagnóstica y excluir otras enfermedades, el resultado es compatible con TEP bilateral coincidente con sarcoidosis pulmonar estadio II. El paciente evoluciona posteriormente de forma favorable con tratamiento corticoideo. (AU)


This is a 34-year-old man who, after returning from a transatlantic trip, consulted for right pleuritic pain, general malaise, sweating, and a productive cough. He presented radiological alterations compatible with bilateral pulmonary thromboembolism (PTE), a micronodular pattern and mediastinal lymphadenopathies that could be related to some infectious-inflammatory process for which he began the study. After applying a broad diagnostic strategy and excluding other diseases, the result is compatible with bilateral PET coinciding with stage II pulmonary sarcoidosis. The patient subsequently evolves favorably with corticosteroid treatment. (AU)


Assuntos
Humanos , Masculino , Adulto , Embolia Pulmonar/diagnóstico por imagem , Embolia Pulmonar/diagnóstico , Embolia Pulmonar/tratamento farmacológico , Fumantes , Radiografia , Sarcoidose
17.
Am J Case Rep ; 23: e936578, 2022 Oct 03.
Artigo em Inglês | MEDLINE | ID: mdl-36190928

RESUMO

BACKGROUND Sarcoidosis is an inflammatory condition with multisystem involvement of unknown etiology that is characterized by noncaseating granulomas. Gastrointestinal (GI) involvement of sarcoidosis is not commonly seen in patients with extrapulmonary disease but can result in luminal narrowing, ulceration, and, less commonly, bleeding and obstruction. Patients that present with symptomatic gastric sarcoidosis are extremely rare. Definitive diagnosis can be challenging due to the need for endoscopic biopsy, which may not be performed if the diagnosis is not considered. Biopsy may be falsely negative due to the patchy mucosal involvement of this disease. CASE REPORT This case describes a 38-year-old mixed-race man who presented to the Emergency Department with GI symptoms including nausea, vomiting, and abdominal pain, which persisted after being recently discharged from an outside hospital. The patient had a known history of multisystem sarcoid including pulmonary and neurosarcoidosis, and was maintained on immunosuppressive therapy. The patient underwent upper endoscopy with biopsy confirming a new diagnosis of gastric sarcoidosis. CONCLUSIONS There is an important role for early endoscopy in the diagnosis of patients with symptomatic gastric sarcoidosis to facilitate early treatment initiation and escalation or titration of immunosuppressive therapy, especially in patients with a known history of sarcoidosis with extrapulmonary involvement. The described endoscopic appearance of gastric sarcoidosis is variable in the published literature; endoscopic biopsy is therefore essential to diagnosing this disease. This type of disease progression should be considered in all sarcoid patients with persistent GI symptoms that do not resolve with conservative management, including those who are already on established immunosuppressive therapy.


Assuntos
Doenças do Sistema Nervoso Central , Sarcoidose , Adulto , Biópsia , Doenças do Sistema Nervoso Central/complicações , Doenças do Sistema Nervoso Central/diagnóstico , Humanos , Masculino , Sarcoidose/complicações , Sarcoidose/diagnóstico , Estômago/patologia
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...