RESUMO
BACKGROUND: In cases with advanced glaucomatous disc changes, further changes associated with other optic neuropathies cannot be easily identified. We present a case of preexisting open-angle glaucoma and concurrent involvement of sarcoidosis-associated optic neuropathy. CASE PRESENTATION: A 53-year-old man presented with gradual visual loss in his left eye, which began 1 year ago and accelerated 3 months ago. The best-corrected visual acuity in the right eye was 20/20 and counting fingers in the left. Intraocular pressures (IOP) were 12 mmHg in the right eye and 34 mmHg in the left. We diagnosed him with advanced open-angle glaucoma in the left eye based on the advanced glaucomatous cupping of the left optic disc. The IOP in the left eye dropped to 10 mmHg and was well controlled with antiglaucomatous medication; however, his left optic disc developed pallor 3 months after the treatment. The patient was revealed to be diagnosed with sarcoidosis a month ago and had been treated with systemic corticosteroids thereafter by a pulmonologist. Orbital magnetic resonance imaging revealed sarcoidosis-associated optic neuropathy in the left eye. Subsequently, optic neuropathy occurred in his right eye. CONCLUSIONS: In eyes with advanced glaucomatous disc change, detecting the coexistence of other optic neuropathies can be difficult. This report highlights the importance of careful ophthalmic examinations and investigation for etiologies of other optic neuropathies if non-glaucomatous changes are suspected even in eyes with advanced glaucoma.
Assuntos
Glaucoma de Ângulo Aberto , Glaucoma , Disco Óptico , Doenças do Nervo Óptico , Sarcoidose , Humanos , Masculino , Pessoa de Meia-Idade , Glaucoma de Ângulo Aberto/complicações , Glaucoma de Ângulo Aberto/diagnóstico , Doenças do Nervo Óptico/complicações , Doenças do Nervo Óptico/diagnóstico , Doenças Raras , Sarcoidose/complicações , Sarcoidose/diagnósticoRESUMO
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 terapêuticoRESUMO
Ventricular arrhythmias are a life-threatening factor in cardiac sarcoidosis (CS), posing a significant therapeutic challenge. Stellate ganglion phototherapy (SGP), a non-invasive procedure for modification of the sympathetic nervous system, is an effective treatment for refractory ventricular tachycardia (RVT). However, there are limited data on the efficacy of SGP for RVT in patients with CS. In our case report, we found that SGP was effective for treating RVT in a patient with CS.We present the case of a man in his 60s with multiple cardioversions of implantable cardioverter defibrillator for ventricular tachycardia. The patient was administered prednisolone for the management of CS, which subsequently led to an increase in anti-tachycardia pacing for ventricular tachycardias. We introduced SGP to suppress RVT and anti-tachycardia pacing decreased from 371 to 25 events. Thus, SGP could be a feasible option for the management of RVT in patients with CS.
Assuntos
Desfibriladores Implantáveis , Miocardite , Sarcoidose , Taquicardia Ventricular , Desfibriladores Implantáveis/efeitos adversos , Humanos , Masculino , Miocardite/complicações , Fototerapia , Sarcoidose/complicações , Sarcoidose/terapia , Gânglio Estrelado , Taquicardia Ventricular/etiologia , Taquicardia Ventricular/terapiaRESUMO
Sarcoidosis is primarily a disease of the lungs, and extrapulmonary manifestations are rare. Herein we report a case of isolated bone marrow sarcoidosis presenting as symptomatic hypercalcemia. A 75-year-old female presented with complaints of confusion, dizziness, headaches, and tremulousness. Workup was unremarkable save for hypercalcemia and elevated serum 1,25(OH)D3. Bone marrow biopsy revealed non-caseating granulomas suggestive of sarcoidosis. She was treated with a slow prednisone taper and her symptoms resolved. This case demonstrates the diagnostic and therapeutic challenges associated with a novel presentation and endorses the use of bone marrow biopsy in the workup of sarcoidosis. The risks and benefits of calcium and vitamin D supplementation for steroid-induced bone disease prevention in this population are also discussed.
Assuntos
Hipercalcemia , Sarcoidose , Humanos , Feminino , Idoso , Hipercalcemia/etiologia , Hipercalcemia/diagnóstico , Medula Óssea/patologia , Sarcoidose/complicações , Sarcoidose/diagnóstico , Prednisona/uso terapêutico , CálcioAssuntos
Hipercalcemia/etiologia , Pancreatite/etiologia , Sarcoidose/complicações , Vitamina D/análogos & derivados , Adulto , Antirreumáticos/uso terapêutico , Aspiração por Agulha Fina Guiada por Ultrassom Endoscópico , Glucocorticoides/uso terapêutico , Humanos , Hidroxicloroquina/uso terapêutico , Hipercalcemia/terapia , Linfadenopatia/patologia , Masculino , Mediastino , Pancreatite/terapia , Prednisona/uso terapêutico , Sarcoidose/diagnóstico , Sarcoidose/tratamento farmacológico , Sarcoidose/patologia , Vitamina D/efeitos adversosRESUMO
Abnormal calcium metabolism in sarcoidosis patients can lead to hypercalcemia, hypercalciuria, and kidney stones. Hypercalcemia in sarcoidosis is usually due to increased activity of 1α-hydroxylase in macrophages of pulmonary granulomata, resulting in low levels of 25-hydroxyvitamin D and high levels of calcitriol. Vitamin D supplementation may be dangerous for some sarcoidosis patients and is recommended only for those with decreased 25-hydroxyvitamin D and reduced or normal calcitriol level. Diagnosis, treatment of osteoporosis, and maintenance of bone health are complex issues for sarcoidosis patients. An approach to diagnosis and treatment of bone fragility is presented.
Assuntos
Desmineralização Patológica Óssea/metabolismo , Cálcio/metabolismo , Suplementos Nutricionais/efeitos adversos , Sarcoidose/metabolismo , Vitamina D/farmacologia , Fatores Etários , Desmineralização Patológica Óssea/etiologia , Desmineralização Patológica Óssea/prevenção & controle , Calcitriol/sangue , Fraturas Ósseas/prevenção & controle , Humanos , Conduta do Tratamento Medicamentoso , Ensaios Clínicos Pragmáticos como Assunto , Fatores de Risco , Sarcoidose/complicações , Sarcoidose/terapia , Fatores Sexuais , Vitamina D/metabolismoRESUMO
OBJECTIVES: This study sought to investigate the value of electroanatomical voltage mapping (EAVM) to distinguish cardiac sarcoidosis (CS) from arrhythmogenic right ventricular cardiomyopathy (ARVC) in patients with ventricular tachycardia from the right ventricle (RV). BACKGROUND: CS can mimic ARVC. Because scar in ARVC is predominantly subepicardial, this study hypothesized that the relative sizes of endocardial low bipolar voltage (BV) to low unipolar voltage (UV) areas may distinguish CS from ARVC. METHODS: Patients with CS affecting the RV (n = 14), patients with gene-positive ARVC (n = 13), and a reference group of patients without structural heart disease (n = 9) who underwent RV endocardial EAVM were included. RV region-specific BV and UV cutoffs were derived from control subjects. In CS and ARVC, segmental involvement was determined and low-voltage areas were measured, using <1.5 mV for BV and <3.9 mV, <4.4 mV, and <5.5 mV for UV. The ratio between low BV and low UV area was calculated generating 3 parameters: Ratio3.9, Ratio4.4 and Ratio5.5, respectively. RESULTS: In control subjects, BV and UV varied significantly among RV regions. The basal septum was involved in 71% of CS patients and in none of ARVC patients. Ratio5.5 discriminated CS from ARVC the best. An algorithm including Ratio5.5 ≥0.45 and basal septal involvement identified CS with 93% sensitivity and 85% specificity. This was validated in a separate population (CS [n = 6], ARVC [n = 10]) with 100% sensitivity and 100% specificity. CONCLUSIONS: EAVM provides detailed information about scar characteristics and scar distribution in the RV. An algorithm combining Ratio5.5 (area BV <1.5 mV/area UV <5.5 mV) and bipolar basal septal involvement allows accurate diagnosis of (isolated) CS in patients presenting with monomorphic ventricular tachycardia from the RV.
Assuntos
Displasia Arritmogênica Ventricular Direita , Sarcoidose , Taquicardia Ventricular , Displasia Arritmogênica Ventricular Direita/diagnóstico , Eletrocardiografia , Técnicas Eletrofisiológicas Cardíacas , Humanos , Sarcoidose/complicações , Sarcoidose/diagnóstico , Taquicardia Ventricular/diagnósticoRESUMO
In patients with unexplained cardiomyopathy, electroanatomical mapping can identify abnormal tissue to target during electrophysiology-guided endomyocardial biopsy (EP-guided EMB). The objective of this study is to determine whether catheter ablation performed in the same setting as EP-guided EMB increases procedural risk. Sixty-seven patients (mean age 54.4 ± 13.8, 57% male) undergoing EP-guided EMB were included. Radiofrequency catheter ablation was performed in 17 patients (25%) for ventricular arrhythmias and in 2 (3%) for typical atrial flutter. Femoral arterial access was obtained in 90% ablation patients and 40% biopsy-only patients; vascular access complications were more common in the ablation group than in the EMB-only group (p = 0.02). There were no significant differences in rate of tricuspid regurgitation, thromboembolism, or pericardial effusion, whether procedural anticoagulation was used. In conclusion, catheter ablation and procedural anticoagulation can be combined with EP-guided EMB with an increased risk of vascular access complications, but no significant increase in intracardiac complications.
Assuntos
Arritmias Cardíacas/patologia , Biópsia/métodos , Cardiomiopatias/patologia , Ablação por Cateter/métodos , Endocárdio/patologia , Miocardite/patologia , Miocárdio/patologia , Sarcoidose/patologia , Adulto , Idoso , Arritmias Cardíacas/etiologia , Arritmias Cardíacas/cirurgia , Flutter Atrial/etiologia , Flutter Atrial/patologia , Flutter Atrial/cirurgia , Bloqueio Atrioventricular/patologia , Bloqueio Atrioventricular/cirurgia , Cardiomiopatias/complicações , Técnicas Eletrofisiológicas Cardíacas , Endocárdio/cirurgia , Estudos de Viabilidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Miocardite/complicações , Complicações Pós-Operatórias/epidemiologia , Sarcoidose/complicações , Taquicardia Ventricular/etiologia , Taquicardia Ventricular/patologia , Taquicardia Ventricular/cirurgia , Complexos Ventriculares Prematuros/etiologia , Complexos Ventriculares Prematuros/patologia , Complexos Ventriculares Prematuros/cirurgiaRESUMO
The role of vitamin D in the human body is not limited only to the regulation of calcium metabolism and secondary to the impact on bones. Recent studies have shown the influence of vitamin D level on muscles, on the risk of cancer, diabetes, hypertension and pulmonary diseases, including granulomatous diseases. Sarcoidosis is a granulomatous disease of unknown etiology. Hypercalcemia in the course of the disease occurs in up to 10% of cases in the consequence of autonomous overproduction of 1,25-dihydroxyvitamin D by macrophages of sarcoid granulomas. Hypercalciuria occurs 3-fold more frequent. On the other hand, treatment with corticosteroids increases the risk of osteoporosis. Vitamin D intake is recommended for prevention of osteoporosis. Such management, in sarcoidosis patients, is not so clear because of risk of hypercalcemia. Vitamin D supplementation, according to current recommendations for general population, is based solely on 25-hydroxyvitamin D level testing. This seems to be not safe in the group of sarcoidosis patients. This article discusses the role of vitamin D in sarcoidosis patients and current opinion on vitamin D supplementation in this group.
Assuntos
Cálcio/metabolismo , Suplementos Nutricionais , Sarcoidose/dietoterapia , Sarcoidose/metabolismo , Vitamina D/administração & dosagem , Corticosteroides/efeitos adversos , Corticosteroides/uso terapêutico , Humanos , Hipercalcemia/etiologia , Osteoporose/etiologia , Osteoporose/prevenção & controle , Guias de Prática Clínica como Assunto , Sarcoidose/complicações , Sarcoidose/tratamento farmacológicoRESUMO
PURPOSE OF REVIEW: Vitamin D supplementation is widespread used in the general population. In sarcoidosis, up to 50% of patients, especially postmenopausal women and those taking corticosteroids, show evidence of increased bone fragility. The purpose of this review is to provide an evidence-based rationale on how to treat sarcoidosis patients with bone health issues. RECENT FINDINGS: Evidence from observational studies show that decreased 25-hydroxy vitamin D is common in sarcoidosis. However, the great majority of sarcoidosis patents have normal or often elevated levels of 1,25-dihydroxy vitamin D (calcitriol), a marker associated with disease activity. High calcitriol levels may often be associated with hypercalcemia and hypercalcuria. The few interventional randomized controlled studies in the field, suggest that vitamin D supplementation may not be well tolerated because of hypercalcemia, moreover without substantial benefit on bone health and risk for fractures in these patients. SUMMARY: Vitamin D supplementation may be withheld in sarcoidosis patients with bone fragility, unless calcitriol levels are below normal limits. A treating scheme is proposed.
Assuntos
Desmineralização Patológica Óssea , Cálcio/metabolismo , Suplementos Nutricionais/efeitos adversos , Fraturas Ósseas/prevenção & controle , Sarcoidose , Vitamina D , Desmineralização Patológica Óssea/etiologia , Desmineralização Patológica Óssea/metabolismo , Desmineralização Patológica Óssea/prevenção & controle , Calcitriol/sangue , Humanos , Conduta do Tratamento Medicamentoso , Sarcoidose/complicações , Sarcoidose/metabolismo , Sarcoidose/terapia , Vitamina D/metabolismo , Vitamina D/farmacologiaRESUMO
BACKGROUND Laryngeal sarcoidosis is a rare extrapulmonary manifestation of sarcoidosis, accounting for 0.33-2.1% of cases. A life-threatening complication of laryngeal sarcoidosis is upper airway obstruction. In this report we describe our experience in the acute and chronic care of a patient who required an emergent tracheostomy, with the aim to provide further insight into this difficult to manage disease. CASE REPORT A 37-year-old African American female with a 10-year history of stage 1 sarcoidosis presented with severe dyspnea. Laryngeal sarcoidosis was diagnosed three years previously, and she remained stable on low-dose prednisone until six months prior to admission, at which time she self-discontinued her prednisone for the homeopathic treatment Nopalea cactus juice. Her physical examination was concerning for impending respiratory failure as she presented with inspiratory stridor and hoarseness. Laryngoscopy showed a retroflexed epiglottis obstructing the glottis with edematous arytenoids and aryepiglottic folds. Otolaryngology performed an emergent tracheostomy to secure her airway and obtained epiglottic biopsies, which were consistent with sarcoidosis. She was eventually discharged home on prednisone 60 mg daily. Following months of corticosteroids, a laryngoscopy showed the epiglottis continuing to obstruct the glottis. The addition of methotrexate to a tapered dosage of prednisone 10 mg daily was unsuccessful, and she remains on prednisone 20 mg daily for disease control. CONCLUSIONS Laryngeal sarcoidosis, a rare extrapulmonary manifestation of sarcoidosis, uncommonly presents as the life-threatening complication of complete upper airway obstruction. As such, laryngeal sarcoidosis is associated with significant morbidity and mortality, requiring a high index of suspicion for timely diagnosis and treatment.
Assuntos
Obstrução das Vias Respiratórias/etiologia , Obstrução das Vias Respiratórias/cirurgia , Doenças da Laringe/complicações , Doenças da Laringe/cirurgia , Sarcoidose/complicações , Sarcoidose/cirurgia , Traqueostomia , Adulto , Emergências , Feminino , Glucocorticoides/uso terapêutico , Humanos , Doenças da Laringe/tratamento farmacológico , Laringoscopia , Prednisona/uso terapêutico , Sarcoidose/diagnóstico , Sarcoidose/tratamento farmacológico , Traqueostomia/métodos , Resultado do TratamentoRESUMO
No disponible
Assuntos
Humanos , Feminino , Adulto , Psoríase/complicações , Psoríase , Sarcoidose/complicações , Sarcoidose/patologia , Sarcoidose , Sarcoidose Pulmonar/complicações , Sarcoidose Pulmonar , Broncoscopia/métodos , Broncoscopia , Terapia Biológica/métodos , Terapia Biológica , Células Th1/patologia , Células Th1RESUMO
Up to 50% patients with sarcoidosis display extra-pulmonary disease. However, initial and isolated (ie, without lung disease) acute muscular involvement associated with pseudo-malignant hypercalcemia is very uncommon. We report on 3 cases of life-threatening hypercalcemia revealing florid and isolated acute sarcoid-like myositis.All patients complained of fatigue, progressive general muscle weakness, and weight loss. Laboratory tests showed a severe life-threatening hypercalcemia (>3.4âmmol/L). Hypercalcemia was associated with increased serum level of 1,25-(OH)2 vitamin D and complicated with acute renal failure. One patient displayed acute pancreatitis due to hypercalcemia.In all cases, PET-scan, performed for malignancy screening, incidentally revealed an intense, diffuse, and isolated muscular fluorodeoxyglucose (FDG) uptake consistent with diffuse non-necrotizing giant cells granulomatous myositis demonstrated by muscle biopsy. Of note, creatine phosphokinase blood level was normal in all cases. No patients displayed the usual thoracic features of sarcoidosis.All patients were treated with high dose steroids and achieved rapid, complete, and sustained remission. A review of English and French publications in Medline revealed 5 similar published cases.Steroid-sensitive acute sarcoid-like myositis causing high calcitriol levels and life-threatening hypercalcemia should be recognized as a separate entity.
Assuntos
Hipercalcemia/etiologia , Miosite/complicações , Síndromes Paraneoplásicas/etiologia , Sarcoidose/complicações , Doença Aguda , Adulto , Idoso , Biópsia , Diagnóstico Diferencial , Feminino , Seguimentos , Humanos , Hipercalcemia/diagnóstico , Masculino , Pessoa de Meia-Idade , Miosite/diagnóstico , Síndromes Paraneoplásicas/diagnóstico , Tomografia por Emissão de Pósitrons , Sarcoidose/diagnósticoRESUMO
PURPOSE: Hypothalamic-pituitary (HP) neurosarcoidosis (NS) accounts for 0.5 % cases of sarcoidosis and 1 % of HP masses. Correlative data on endocrine and neurological outcomes is lacking. METHODS: Retrospective case series and literature review of presentation, treatment and outcome of HP NS. RESULTS: Our series includes 4 men, ages 34-59, followed for a median of 7.3 years (range 1.5-17). All had optic neuropathy, multiple pituitary hormone abnormalities (PHAs) and other organ involvement by sarcoidosis (lung, sino-nasal, brain/spine and facial nerve). Two patients had central diabetes insipidus and one impaired thirst with polydipsia. After treatment with high-dose glucocorticoids, optic neuropathy improved in one case and stabilized in the others. After treatment, HP lesions improved radiologically, but PHAs persisted in all cases. Review of four published series on HP NS in addition to ours yielded 46 patients, age 37 ± 11.8 years, 65 % male. PHAs consisted of anterior hypopituitarism (LH/FSH 88.8 %, TSH 67.4 %, GH 50.0 %, ACTH 48.8 %), hyperprolactinemia (48.8 %) and diabetes insipidus (65.2 %). PHAs were the first sign of disease in 54.3 % patients. Vision problems occurred in 28.3 % patients, but optic neuropathy was not well documented in previous series. Most patients (93.5 %) received high-dose glucocorticoids followed by taper; 50 % also received other immunomodulators, including methotrexate, mycophenolate mofetil, cyclosporine, azathioprine, infliximab and hydrochloroquine. Only 13 % patients showed improvement in PHAs. All-cause mortality was 8.7 %. CONCLUSION: HP NS is a serious disease requiring multidisciplinary treatment and lifelong follow-up. Prospective multicentric studies are needed to determine a more standardized approach to HP NS and outline predictors of disease outcome.
Assuntos
Hipopituitarismo/diagnóstico , Doenças da Hipófise/diagnóstico , Hipófise/patologia , Sarcoidose/diagnóstico , Adulto , Glucocorticoides/uso terapêutico , Humanos , Hipopituitarismo/tratamento farmacológico , Hipopituitarismo/etiologia , Hipotálamo/patologia , Fatores Imunológicos/uso terapêutico , Masculino , Pessoa de Meia-Idade , Doenças da Hipófise/complicações , Doenças da Hipófise/tratamento farmacológico , Estudos Retrospectivos , Sarcoidose/complicações , Transtornos da Visão/tratamento farmacológico , Transtornos da Visão/etiologia , Transtornos da Visão/patologiaRESUMO
BACKGROUND: Respiratory muscle weakness occurs in sarcoidosis and is related to decreased exercise capacity, greater fatigue, dyspnea, and lower quality of life in sarcoidosis patients. The effects of inspiratory muscle training in this population have not been comprehensively investigated so far. This study was planned to investigate the effects of inspiratory muscle training on exercise capacity, respiratory and peripheral muscle strength, pulmonary function and diffusing capacity, fatigue, dyspnea, depression, and quality of life in subjects with sarcoidosis. METHODS: This was a prospective, randomized, controlled, and double blind study. Fifteen sarcoidosis subjects (treatment group) received inspiratory muscle training at 40% of maximal inspiratory pressure (P(Imax)), and 15 subjects (control group) received sham therapy (5% of P(Imax)) for 6 weeks. Functional and maximal exercise capacity, respiratory and peripheral muscle strength, pulmonary function and diffusing capacity, fatigue, dyspnea, depression, and quality of life were evaluated. RESULTS: Functional (P < .001) and maximal exercise capacity (P = .038), respiratory muscle strength (P(Imax) [P < .001] and P(Emax) [P = .001]), severe fatigue (P = .002), and dyspnea perception (P = .02) were statistically significantly improved in the treatment group compared with controls; no significant improvements were observed in pulmonary function and diffusing capacity, peripheral muscle strength, fatigue, depression, and quality of life between groups after inspiratory muscle training. CONCLUSIONS: Inspiratory muscle training improves functional and maximal exercise capacity and respiratory muscle strength and decreases severe fatigue and dyspnea perception in subjects with early stages of sarcoidosis. Inspiratory muscle training can be safely and effectively included in rehabilitation programs. (ClinicalTrials.gov registration NCT02270333.).
Assuntos
Exercícios Respiratórios/métodos , Força Muscular , Músculos Respiratórios/fisiopatologia , Sarcoidose/terapia , Adulto , Depressão/etiologia , Método Duplo-Cego , Dispneia/etiologia , Tolerância ao Exercício/fisiologia , Fadiga/etiologia , Feminino , Humanos , Inalação/fisiologia , Capacidade Inspiratória/fisiologia , Pulmão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Capacidade de Difusão Pulmonar/fisiologia , Qualidade de Vida , Testes de Função Respiratória , Sarcoidose/complicações , Sarcoidose/fisiopatologiaRESUMO
Sarcoidosis is an idiopathic inflammatory disorder characterized by noncaseating granulomas, which can affect any organ system. The lungs are most commonly affected but extrapulmonary sites may cause the initial and/or sole symptoms. In this review, the disease manifestations and treatment are described, with particular emphasis on the management of each affected organ system. Diagnosis and management can be difficult and greatly affect quality of life, but despite these challenges, it is possible to successfully manage patients with sarcoidosis in the primary care setting.
Assuntos
Terapia Biológica/métodos , Cálcio , Glucocorticoides/uso terapêutico , Sarcoidose , Cálcio/sangue , Cálcio/urina , Gerenciamento Clínico , Humanos , Atenção Primária à Saúde/métodos , Sarcoidose/complicações , Sarcoidose/diagnóstico , Sarcoidose/metabolismo , Sarcoidose/fisiopatologia , Sarcoidose/terapiaRESUMO
We report a case of a 53-year-old man presenting with depressed alertness and severe excessive sleepiness in the setting of neurosarcoidosis. Neuroimaging demonstrated hypothalamic destruction due to sarcoidosis with a CSF hypocretin level of 0 pg/mL. The patient also experienced respiratory depression that presumably resulted from hypocretin-mediated hypothalamic dysfunction as a result of extensive diencephalic injury. This is a novel case, demonstrating both hypocretin deficiency syndrome, as well as respiratory dysfunction from destruction of hypocretin neurons and extensive destruction of key diencephalic structures secondary to the underlying neurosarcoidosis.
Assuntos
Doenças do Sistema Nervoso Central/complicações , Doenças Hipotalâmicas/complicações , Hipoventilação/congênito , Narcolepsia/complicações , Orexinas/deficiência , Sarcoidose/complicações , Apneia do Sono Tipo Central/complicações , Doenças do Sistema Nervoso Central/líquido cefalorraquidiano , Humanos , Doenças Hipotalâmicas/líquido cefalorraquidiano , Doenças Hipotalâmicas/fisiopatologia , Hipotálamo/fisiopatologia , Hipoventilação/líquido cefalorraquidiano , Hipoventilação/complicações , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Narcolepsia/líquido cefalorraquidiano , Orexinas/líquido cefalorraquidiano , Sarcoidose/líquido cefalorraquidiano , Apneia do Sono Tipo Central/líquido cefalorraquidianoRESUMO
BACKGROUND: Cardiac sarcoid-related ventricular tachycardia (VT) is a rare disorder; the underlying substrate and response to ablation are poorly understood. We sought to examine the ventricular substrate and outcomes of catheter ablation in this population. METHODS AND RESULTS: Of 435 patients with nonischemic cardiomyopathy referred for VT ablation, 21 patients (5%) had cardiac sarcoidosis. Multiple inducible VTs were observed with mechanism consistent with scar-mediated re-entry in all VTs. Voltage maps showed widespread and confluent right ventricular scarring. Left ventricular scarring was patchy with a predilection for the basal septum, anterior wall, and perivalvular regions. Epicardial right ventricular scar overlay and exceeded the region of corresponding endocardial scar. After ≥1 procedures, ablation abolished ≥1 inducible VT in 90% and eliminated VT storm in 78% of patients; however, multiple residual VTs remained inducible. Failure to abolish all inducible VTs was because of septal intramural circuits or extensive right ventricular scarring. Multiple procedure VT-free survival was 37% at 1 year, but VT control was achievable in the majority of patients with fewer antiarrhythmic drugs compared with preablation (2.1±0.8 versus 1.1±0.8; P<0.001). CONCLUSIONS: Patients with cardiac sarcoidosis and VT exhibit ventricular substrate characterized by confluent right ventricular scarring and patchy left ventricular scarring capable of sustaining a large number of re-entrant circuits. Catheter ablation is effective in terminating VT storm and eliminating ≥1 inducible VT in the majority of patients, but recurrences are common. Ablation in conjunction with antiarrhythmic drugs can help palliate VT in this high-risk population.
Assuntos
Cardiomiopatias/complicações , Ablação por Cateter , Sistema de Condução Cardíaco/cirurgia , Ventrículos do Coração/cirurgia , Sarcoidose/complicações , Taquicardia Ventricular/cirurgia , Adulto , Idoso , Antiarrítmicos/uso terapêutico , Cardiomiopatias/diagnóstico , Ablação por Cateter/efeitos adversos , Terapia Combinada , Intervalo Livre de Doença , Técnicas Eletrofisiológicas Cardíacas , Feminino , Sistema de Condução Cardíaco/fisiopatologia , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva , Retratamento , Sarcoidose/diagnóstico , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/etiologia , Taquicardia Ventricular/fisiopatologia , Fatores de Tempo , Resultado do TratamentoRESUMO
AGEs are permanently modified macromolecule derivatives that form through nonenzymatic glycation of amino groups of proteins. Glycer-AGEs are highly toxic and play an important role in the pathogenesis of chronic inflammatory diseases. However, the contribution of glycer-AGEs to the pathogenesis of uveitis is unclear. In this study, we measured serum levels of glycer-AGEs in 100 patients with endogenous uveitis (22 with HLA-B27-associated uveitis, 20 with VKH disease, 14 with Behçet's disease, and 44 with sarcoidosis) and 33 healthy volunteers. We then examined the effect of the AGE inhibitor in a mouse model of human endogenous uveitis (EAU) by continuous oral administration of pyridoxamine at 200 or 400 mg/kg/day. Regardless of the etiology, serum glycer-AGE levels were significantly higher in patients with uveitis than in healthy subjects. Treatment with 400 mg/kg pyridoxamine significantly reduced the clinical and histological severity of EAU and was accompanied by a significant decrease in serum and retinal glycer-AGE levels and suppression of translocation of NF-κB p65 into the nucleus of retinal cells. Serum glycer-AGE levels may therefore serve as a biomarker of human uveitis, as well as systemic inflammation, and may contribute to the progression of uveitis, including diabetic iritis, via the activation of NF-κB.
Assuntos
Doenças Autoimunes/tratamento farmacológico , Produtos Finais de Glicação Avançada/antagonistas & inibidores , Piridoxamina/uso terapêutico , Retinite/tratamento farmacológico , Uveíte/tratamento farmacológico , Administração Oral , Adulto , Sequência de Aminoácidos , Animais , Doenças Autoimunes/sangue , Doenças Autoimunes/patologia , Síndrome de Behçet/sangue , Síndrome de Behçet/complicações , Modelos Animais de Doenças , Avaliação Pré-Clínica de Medicamentos , Proteínas do Olho/imunologia , Proteínas do Olho/metabolismo , Proteínas do Olho/toxicidade , Feminino , Antígeno HLA-B27/imunologia , Humanos , Masculino , Camundongos , Pessoa de Meia-Idade , Dados de Sequência Molecular , Fragmentos de Peptídeos/imunologia , Fragmentos de Peptídeos/toxicidade , Transporte Proteico/efeitos dos fármacos , Piridoxamina/administração & dosagem , Piridoxamina/farmacologia , Retina/metabolismo , Retinite/sangue , Retinite/etiologia , Retinite/patologia , Proteínas de Ligação ao Retinol/imunologia , Proteínas de Ligação ao Retinol/toxicidade , Sarcoidose/sangue , Sarcoidose/complicações , Uveíte/sangue , Uveíte/etiologia , Uveíte/patologia , Síndrome Uveomeningoencefálica/sangue , Síndrome Uveomeningoencefálica/complicaçõesRESUMO
While low levels of vitamin D can increase the risk for osteoporosis, excessive amounts of vitamin D may also be problematic. Hypercalcemia and hypercalcuria due to increased vitamin D activity occur in a significant proportion of sarcoidosis patients. Saidenberg-Kermanac'h and colleagues compared vitamin D levels with bone fragility fractures in their sarcoidosis clinic.They found that a 25-(OH) vitamin D level between 10 and 20 ng/ml was associated with the lowest risk of bone fractures and paradoxically higher levels increased the risk of bone fractures. Using less vitamin D supplementation may simultaneously lower the risk for bone fracture and hypercalcemia in sarcoidosis.