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1.
Brain Nerve ; 76(5): 598-604, 2024 May.
Artigo em Japonês | MEDLINE | ID: mdl-38741502

RESUMO

Sarcoidosis is an idiopathic granulomatous multi-organ disease, primarily affecting the respiratory system, eyes, and skin, with less involvement in peripheral neurons and muscles. Sarcoid peripheral neuropathy encompasses cranial and spinal nerve impairment. Muscle involvement is often asymptomatic and revealed through imaging. Symptomatic muscle involvement is categorized into three clinical types: nodular myopathy, acute myopathy, and chronic myopathy. The identification of noncaseating granulomas in peripheral nerves or muscles, coupled with the exclusion of other diseases, is essential for establishing a definitive diagnosis of sarcoid peripheral neuropathy and myopathy. Sarcoid neuropathy and myopathy are typically managed with high-dose corticosteroids, immunosuppressants, or a combination of both. In recent times, the use of TNF-alpha inhibitors has notably increased. However, these conditions often exhibit resistance to treatment and may necessitate prolonged therapeutic interventions. Therefore, comprehensive examinations should be conducted before considering immunotherapy. Due to the rarity of these conditions, research on manifestation-specific treatments is lacking, and standard treatments for sarcoid neuropathy and myopathy have not been established. Additional treatment options for sarcoid neuropathy and myopathy are expected to become available in the future.


Assuntos
Doenças Musculares , Doenças do Sistema Nervoso Periférico , Sarcoidose , Humanos , Doenças do Sistema Nervoso Periférico/diagnóstico , Doenças do Sistema Nervoso Periférico/terapia , Doenças Musculares/diagnóstico , Doenças Musculares/terapia , Sarcoidose/diagnóstico , Sarcoidose/terapia , Sarcoidose/tratamento farmacológico
2.
BMJ Case Rep ; 17(4)2024 Apr 25.
Artigo em Inglês | MEDLINE | ID: mdl-38670568

RESUMO

We describe a patient who had failed renal transplant after 13 years, eventually requiring a graft nephrectomy and discontinuation of immunosuppressive therapy, including antithymocyte globulin, tacrolimus and mycophenolate while on steroid avoidance protocol. Within a few months of complete discontinuation of the immunosuppressive medications, she developed lower back pain associated with numbness in her right anterolateral thigh. The radiological imaging demonstrated multiple bony lesions throughout her axial and appendicular skeleton with normal pulmonary findings. A computerised tomography-guided bone biopsy from the left iliac crest revealed fragments of bone with granulomatous inflammation, thus making the diagnosis of extrapulmonary sarcoidosis. Initiating treatment with prednisone resulted in near-complete resolution of symptoms. Long-term immunosuppressive therapy is administered to all renal transplant recipients to help prevent acute rejection and loss of renal allograft. This case highlights that immunosuppressants can conceal the presence of underlying conditions in transplant patients.


Assuntos
Imunossupressores , Transplante de Rim , Sarcoidose , Humanos , Feminino , Sarcoidose/tratamento farmacológico , Imunossupressores/efeitos adversos , Imunossupressores/uso terapêutico , Doenças Ósseas/diagnóstico por imagem , Doenças Ósseas/etiologia , Doenças Ósseas/induzido quimicamente , Tomografia Computadorizada por Raios X , Pessoa de Meia-Idade , Prednisona/uso terapêutico , Prednisona/administração & dosagem
3.
J Neurol Sci ; 460: 123018, 2024 May 15.
Artigo em Inglês | MEDLINE | ID: mdl-38640580

RESUMO

Sarcoidosis is a disease characterized by non-caseating granulomas that can involve the central nervous system as neurosarcoidosis. This challenging disease is currently managed with high dose steroids, and sometimes the addition of infliximab. Other TNA-alpha inhibitors have not been studied as rigorously. We discovered ten neurosarcoidosis patients who were on an alternative TNA-alpha inhibitor, adalimumab. Eight patients had a positive response clinically and radiographically to adalimumab.


Assuntos
Adalimumab , Doenças do Sistema Nervoso Central , Sarcoidose , Humanos , Sarcoidose/tratamento farmacológico , Sarcoidose/diagnóstico por imagem , Adalimumab/uso terapêutico , Doenças do Sistema Nervoso Central/tratamento farmacológico , Doenças do Sistema Nervoso Central/diagnóstico por imagem , Feminino , Masculino , Pessoa de Meia-Idade , Adulto , Anti-Inflamatórios/uso terapêutico , Resultado do Tratamento , Idoso
4.
Clin Exp Ophthalmol ; 52(3): 294-316, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38385625

RESUMO

Sarcoidosis is a leading cause of non-infectious uveitis that commonly affects middle-aged individuals and has a female preponderance. The disease demonstrates age, sex and ethnic differences in clinical manifestations. A diagnosis of sarcoidosis is made based on a compatible clinical presentation, supporting investigations and histologic evidence of non-caseating granulomas, although biopsy is not always possible. Multimodal imaging with widefield fundus photography, optical coherence tomography and angiography can help in the diagnosis of sarcoid uveitis and in the monitoring of treatment response. Corticosteroid remains the mainstay of treatment; chronic inflammation requires steroid-sparing immunosuppression. Features on multimodal imaging such as vascular leakage may provide prognostic indicators of outcome. Female gender, prolonged and severe uveitis, and posterior involving uveitis are associated with poorer visual outcomes.


Assuntos
Sarcoidose , Uveíte , Pessoa de Meia-Idade , Humanos , Feminino , Uveíte/diagnóstico , Uveíte/tratamento farmacológico , Sarcoidose/complicações , Sarcoidose/diagnóstico , Sarcoidose/tratamento farmacológico , Prognóstico , Técnicas de Diagnóstico Oftalmológico , Inflamação
5.
Clin J Gastroenterol ; 17(2): 327-333, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38300407

RESUMO

We report the case of a 48-year-old male with a history of pulmonary and ocular sarcoidosis. Non-caseating granulomas, identified histologically, are the most characteristic manifestation of sarcoidosis. Hepatic sarcoidosis is difficult to diagnose using radiological imaging. In the patient reported in this study, ultrasound and contrast-enhanced computed tomography scans identified multiple intra-abdominal lymphadenopathies, with evidence of liver and splenic infiltrations. The first liver biopsy revealed non-caseating granulomatous hepatitis consistent with hepatic sarcoidosis. The patient was treated with ursodeoxycholic acid (UDCA), but his laboratory parameters did not improve. Prednisone was initiated at a dose of 30 mg daily and slowly tapered. At a dose of 12.5 mg daily, marked improvements in the fibrotic and sarcoid-like lesions were noted at the second biopsy. A third biopsy was performed, with the patient on a prednisone taper of 5 mg/day showed mild fibrous expansion in the portal tracts and mild parenchymal necro-inflammatory lesions. However, overall, fibrosis marker levels remained stable over the course of treatment. A fourth biopsy was performed after a 5-year course of 5 mg/day prednisone. This revealed minimal lobular inflammation without fibrosis. Thus, treatment of this patient with corticosteroids and UDCA resulted in marked improvements in his biochemical and histological parameters.


Assuntos
Hepatopatias , Sarcoidose , Masculino , Humanos , Pessoa de Meia-Idade , Prednisona/uso terapêutico , Ácido Ursodesoxicólico/uso terapêutico , Hepatopatias/diagnóstico por imagem , Hepatopatias/tratamento farmacológico , Hepatopatias/etiologia , Sarcoidose/complicações , Sarcoidose/tratamento farmacológico , Sarcoidose/diagnóstico , Corticosteroides/uso terapêutico , Fibrose
6.
Probl Endokrinol (Mosk) ; 69(6): 47-53, 2024 Jan 24.
Artigo em Russo | MEDLINE | ID: mdl-38311994

RESUMO

Cushing's disease is a rare severe neuroendocrine disorder caused by chronic overproduction of adrenocorticotropic hormone by a pituitary tumor. Supraphysiological concentrations of cortisol in endogenous hypercortisolism have an immunosuppressive and anti-inflammatory effect similar to therapy with systemic glucocorticosteroids. This may reduce the activity of the patient's concomitant autoimmune inflammatory diseases. On the other hand, a decrease in cortisol levels during treatment for Cushing's disease may be associated with a reactivation of the immune system that pose a risk of onset or recurrence of an autoimmune disorder. We present our own clinical case demonstrating the development of sarcoidosis after surgical treatment of Cushing's disease.


Assuntos
Doenças Autoimunes , Hipersecreção Hipofisária de ACTH , Neoplasias Hipofisárias , Sarcoidose , Humanos , Hidrocortisona/uso terapêutico , Hipersecreção Hipofisária de ACTH/complicações , Hipersecreção Hipofisária de ACTH/tratamento farmacológico , Hipersecreção Hipofisária de ACTH/cirurgia , Hormônio Adrenocorticotrópico/uso terapêutico , Sarcoidose/complicações , Sarcoidose/tratamento farmacológico
7.
Clin Chest Med ; 45(1): 131-157, 2024 03.
Artigo em Inglês | MEDLINE | ID: mdl-38245362

RESUMO

Over 50% of patients with sarcoidosis will require anti-inflammatory therapy at some point in their disease course. Indications for therapy are to improve health-related quality of life, prevent or arrest organ dysfunction (or organ failure) or avoid death. Recently published treatment guidelines recommended a stepwise approach to therapy however there are some patients for whom up front combination or more intense therapy maybe reasonable. The last decade has seen an explosion of studies and trials evaluating novel therapeutic agents and treatment strategies. Currently available anti-inflammatory therapies and several novel therapies are discussed here.


Assuntos
Anticorpos Monoclonais , Sarcoidose , Humanos , Anticorpos Monoclonais/uso terapêutico , Infliximab/uso terapêutico , Qualidade de Vida , Anti-Inflamatórios/farmacologia , Anti-Inflamatórios/uso terapêutico , Sarcoidose/tratamento farmacológico , Fator de Necrose Tumoral alfa/uso terapêutico
9.
Am Fam Physician ; 109(1): 19-29, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38227868

RESUMO

Sarcoidosis is a multisystem granulomatous inflammatory disease of unknown etiology that can involve any organ. Ongoing dyspnea and dry cough in a young to middle-aged adult should increase the suspicion for sarcoidosis. Symptoms can present at any age and affect any organ system; however, pulmonary sarcoidosis is the most common. Extrapulmonary manifestations often involve cardiac, neurologic, ocular, and cutaneous systems. Patients with sarcoidosis can exhibit constitutional symptoms such as fever, unintentional weight loss, and fatigue. The early recognition and diagnosis of sarcoidosis are challenging because there is no diagnostic standard for testing, initial symptoms vary, and patients may be asymptomatic. Consensus guidelines recommend a holistic approach when diagnosing sarcoidosis that focuses on clinical presentation and radiographic findings, biopsy with evidence of noncaseating granulomas, involvement of more than one organ system, and elimination of other etiologies of granulomatous disease. Corticosteroids are the initial treatment for active disease, with refractory cases often requiring immunosuppressive or biologic therapies. Transplantation can be considered for advanced and end-stage disease depending on organ involvement.


Assuntos
Sarcoidose Pulmonar , Sarcoidose , Adulto , Pessoa de Meia-Idade , Humanos , Sarcoidose/terapia , Sarcoidose/tratamento farmacológico , Sarcoidose Pulmonar/diagnóstico , Sarcoidose Pulmonar/tratamento farmacológico , Corticosteroides/uso terapêutico , Dispneia/diagnóstico , Diagnóstico Diferencial
10.
Lancet Rheumatol ; 6(2): e81-e91, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38267106

RESUMO

BACKGROUND: Sarcoidosis is an inflammatory condition that can affect various organs and tissues, causing the formation of granulomas and subsequent functional impairment. The origin of sarcoidosis remains unknown and there are few treatment options. Mechanistic target of rapamycin (mTOR) activation is commonly seen in granulomas of patients across different tissues and has been shown to induce sarcoidosis-like granulomas in a mouse model. This study aimed to examine the efficacy and safety of the mTOR inhibitor sirolimus as a treatment for cutaneous sarcoidosis. METHODS: We did a single-centre, randomised study treating patients with persistent and glucocorticoid-refractory cutaneous sarcoidosis with sirolimus at the Vienna General Hospital, Medical University of Vienna (Vienna, Austria). We recruited participants who had persistent, active, and histologically proven cutaneous sarcoidosis. We used an n-of-1 crossover design in a placebo-controlled, double-blind topical treatment period and a subsequent single-arm systemic treatment phase for 4 months in the same participants. Participants initially received either 0·1% topical sirolimus in Vaseline or placebo (Vaseline alone), twice daily. After a washout period, all participants were subsequently administered a 6 mg loading dose followed by 2 mg sirolimus solution orally once daily, aiming to achieve serum concentrations of 6 ng/mL. The primary endpoint was change in the Cutaneous Sarcoidosis Activity and Morphology Index (CSAMI) after topical or systemic treatment. All participants were included in the safety analyses, and patients having completed the respective treatment period (topical treatment or systemic treatment) were included in the primary analyses. Adverse events were assessed at each study visit by clinicians and were categorised according to their correlation with the study drug, severity, seriousness, and expectedness. This study is registered with EudraCT (2017-004930-27) and is now closed. FINDINGS: 16 participants with persistent cutaneous sarcoidosis were enrolled in the study between Sept 3, 2019, and June 15, 2021. Six (37%) of 16 participants were men, ten (63%) were women, and 15 (94%) were White. The median age of participants was 54 years (IQR 48-58). 14 participants were randomly assigned in the topical phase and 2 entered the systemic treatment phase directly. Daily topical treatment did not improve cutaneous lesions (effect estimate -1·213 [95% CI -2·505 to 0·079], p=0·066). Systemic treatment targeting trough serum concentrations of 6 ng/mL resulted in clinical and histological improvement of skin lesions in seven (70%) of ten participants (median -7·0 [95% CI -16·5 to -3·0], p=0·018). Various morphologies of cutaneous sarcoidosis, including papular, nodular, plaque, scar, and tattoo-associated sarcoidosis, responded to systemic sirolimus therapy with a long-lasting effect for more than 1 year after treatment had been stopped. There were no serious adverse events and no deaths. INTERPRETATION: Short-term treatment with systemic sirolimus might be an effective and safe treatment option for patients with persistent glucocorticoid-refractory sarcoidosis with a long-lasting disease-modulating effect. The effect of sirolimus in granulomatous inflammation should be investigated further in large, multi-centre, randomised clinical trials. FUNDING: Vienna Science and Technology Fund, Austrian Science Fund.


Assuntos
Butilaminas , Sarcoidose , Sirolimo , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Glucocorticoides/farmacologia , Granuloma , Vaselina , Sarcoidose/tratamento farmacológico , Sirolimo/efeitos adversos
11.
Ocul Immunol Inflamm ; 32(2): 178-180, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-36637975

RESUMO

We read with great interest the article by Oyeniran E et al. on "Isolated optic disc granuloma as a presenting sign of sarcoidosis." We would like to share our experience with a similar optic nerve head granuloma secondary to sarcoidosis in the absence of any systemic symptoms and no evidence of signs of periocular/intraocular inflammation. However, our case was refractory to oral steroids and methotrexate and required intravitreal dexamethasone implants and mycophenolate mofetil.


Assuntos
Disco Óptico , Sarcoidose , Uveíte , Humanos , Sarcoidose/complicações , Sarcoidose/diagnóstico , Sarcoidose/tratamento farmacológico , Granuloma/diagnóstico , Granuloma/tratamento farmacológico , Granuloma/complicações , Uveíte/complicações , Transtornos da Visão
12.
Ocul Immunol Inflamm ; 32(2): 181-183, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37467487

RESUMO

We report a case of intermediate uveitis in the setting of both systemic sarcoidosis and multiple sclerosis. A 68-year-old female was diagnosed with bilateral granulomatous intermediate uveitis and cystoid macular edema. Initial systemic work-up was unrevealing. The uveitis was treated successfully with local corticosteroid injections. Eighteen months after presentation, the patient developed new systemic symptoms. Additional testing revealed systemic lymphadenopathy, with biopsy showing non-caseating granulomas, leading to a diagnosis of sarcoidosis. However, MRI of the brain and spinal cord along with cerebrospinal fluid analysis was consistent with MS. The management of the uveitis and systemic inflammation was co-managed by ophthalmology, neurology, and rheumatology, and eventually controlled with leflunomide and rituximab. Patients can rarely have co-existing systemic sarcoidosis and multiple sclerosis. Although challenging to diagnose, radiographic findings and cerebrospinal fluid analysis can be helpful to differentiate multiple sclerosis and neurosarcoidosis. Management of these patients requires coordination between multiple specialties.


Assuntos
Esclerose Múltipla , Sarcoidose , Uveíte Intermediária , Uveíte , Feminino , Humanos , Idoso , Esclerose Múltipla/complicações , Esclerose Múltipla/diagnóstico , Uveíte/complicações , Uveíte/diagnóstico , Uveíte/tratamento farmacológico , Sarcoidose/complicações , Sarcoidose/diagnóstico , Sarcoidose/tratamento farmacológico , Uveíte Intermediária/complicações , Granuloma/complicações
13.
Osteoporos Int ; 35(4): 733-736, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38102330

RESUMO

A 70-year-old female patient was admitted for close examination and treatment of hypercalcemia (corrected serum calcium levels: 3.04 mmol/L) and renal dysfunction (serum creatinine levels: 254.59 µmol/L). The patient had a history of sarcoidosis, diagnosed based on epithelioid cell granulomas in subcutaneous nodule biopsies, uveitis, and bilateral hilar lymphadenopathy, which had spontaneously remitted 10 years before admission. Because the patient was diagnosed with hypercalcemia associated with recurrent sarcoidosis, prednisone (20 mg/day) was initiated, and its dose was tapered following the decrease in serum calcium and creatinine levels. However, the levels of these parameters increased again when the prednisone dose was reduced to ≤ 4 mg/day. We were concerned about glucocorticoid-induced osteoporosis in the patient but hesitated to use first-line bisphosphonates because of renal dysfunction. Therefore, denosumab was initiated to reduce the risk of hypercalcemia, renal dysfunction, and glucocorticoid-induced osteoporosis. Serum creatinine and corrected serum calcium levels subsequently decreased. The prednisone dose could be reduced following repeated denosumab administration.Thus, denosumab can be a multifaceted, beneficial option for sarcoidosis-induced hypercalcemia, as it alleviates renal dysfunction indirectly by normalizing serum calcium levels, facilitates reduction of the glucocorticoid dose, and ameliorates glucocorticoid-induced osteoporosis.


Assuntos
Hipercalcemia , Nefropatias , Osteoporose , Sarcoidose , Idoso , Feminino , Humanos , Cálcio , Creatinina , Denosumab/uso terapêutico , Glucocorticoides/efeitos adversos , Granuloma/complicações , Hipercalcemia/induzido quimicamente , Hipercalcemia/tratamento farmacológico , Osteoporose/induzido quimicamente , Osteoporose/complicações , Osteoporose/tratamento farmacológico , Prednisona/efeitos adversos , Sarcoidose/complicações , Sarcoidose/tratamento farmacológico
14.
Front Immunol ; 14: 1286684, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38077350

RESUMO

Objective: Immunosuppressive therapy for cardiac sarcoidosis (CS) still largely consists of corticosteroid monotherapy. However, high relapse rates after tapering and insufficient efficacy are significant problems. The objective of this study was to investigate the efficacy and safety of non-biological and biological disease-modifying anti-rheumatic drugs (nb/bDMARDs) considering control of myocardial inflammation assessed by 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) of the heart. Methods: We conducted a retrospective analysis of treatment response to nb/bDMARDs of all CS patients seen in the sarcoidosis center of the University Hospital Zurich between January 2016 and December 2020. Results: We identified 50 patients with CS. Forty-five patients with at least one follow-up PET/CT scan were followed up for a mean of 20.5 ± 12.8 months. Most of the patients were treated with prednisone and concomitant nb/bDMARDs. At the first follow-up PET/CT scan after approximately 6.7 ± 3 months, only adalimumab showed a significant reduction in cardiac metabolic activity. Furthermore, comparing all serial follow-up PET/CT scans (143), tumor necrosis factor inhibitor (TNFi)-based therapies showed statistically significant better suppression of myocardial 18F-FDG uptake compared to other treatment regimens. On the last follow-up, most adalimumab-treated patients were inactive (n = 15, 48%) or remitting (n = 11, 35%), and only five patients (16%) were progressive. TNFi was safe even in patients with severely reduced left ventricular ejection fraction (LVEF), and a significant improvement in LVEF under TNFi treatment was observed. Conclusion: TNFi shows better control of myocardial inflammation compared to nbDMARDs and corticosteroid monotherapies in patients with CS. TNFi was efficient and safe even in patients with severely reduced LVEF.


Assuntos
Miocardite , Sarcoidose , Humanos , Fluordesoxiglucose F18 , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Estudos Retrospectivos , Compostos Radiofarmacêuticos , Volume Sistólico , Adalimumab/uso terapêutico , Função Ventricular Esquerda , Sarcoidose/diagnóstico por imagem , Sarcoidose/tratamento farmacológico , Sarcoidose/complicações , Miocardite/tratamento farmacológico , Corticosteroides/uso terapêutico , Inflamação/tratamento farmacológico
15.
BMJ Case Rep ; 16(10)2023 Oct 17.
Artigo em Inglês | MEDLINE | ID: mdl-37848272

RESUMO

The case report describes the details of a man in his 40s admitted for evaluation of renal failure. Biochemical testing revealed parathyroid hormone (PTH) -independent hypercalcaemia. The evaluations for the usual causes such as malignancies, granulomatous diseases, multiple myeloma and vitamin D toxicity were negative. Fluorodeoxyglucose Positron Emission Tomography (FDG-PET) scan identified diffuse uptake in the muscles, and the subsequent muscle biopsy showed non-caseating granulomas suggestive of granulomatous myositis, possibly sarcoidosis, in view of raised ACE levels and the absence of other causes. The patient showed a dramatic response to glucocorticoids, with prompt relief of symptoms and normalisation of serum calcium and creatinine. The case highlights the importance of considering sarcoid myositis in the evaluation of hypercalcaemia and the need for prompt initiation of glucocorticoid therapy to achieve favourable outcomes. The successful use of FDG-PET in diagnosing PTH-independent hypercalcaemia suggests its potential as a valuable tool in the diagnostic algorithm for this condition.


Assuntos
Hipercalcemia , Miosite , Insuficiência Renal , Sarcoidose , Masculino , Humanos , Hipercalcemia/diagnóstico , Hipercalcemia/etiologia , Fluordesoxiglucose F18 , Sarcoidose/complicações , Sarcoidose/diagnóstico , Sarcoidose/tratamento farmacológico , Miosite/diagnóstico , Granuloma/complicações , Insuficiência Renal/complicações
16.
Front Immunol ; 14: 1150128, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37781378

RESUMO

Nowadays, immune checkpoint inhibitors (ICI) have become the cornerstone of treatment for many tumors, either as monotherapy or in combination with other therapies. However, these drugs are associated with several new side effects that need early detection. We present the case of a 41-year-old male patient who has been diagnosed with advanced hepatocellular carcinoma (HCC) with metastatic retroperitoneal lymph nodes and a subdiaphragmatic metastatic lesion, undergoing second-line treatment with a combination of nivolumab and ipilimumab. After completing four cycles, the patient was admitted to the hospital due to intermittent fever and profuse sweating. A CT scan showed multiple pathologically enlarged lymph nodes in several locations, raising suspicion of disease progression. The patient's clinical progress was favorable after symptomatic treatment (antipyretics) and was discharged one week after admission. Several days later, the patient complained about painful bilateral ocular redness and was diagnosed with bilateral anterior uveitis. Further blood tests showed elevated angiotensin-converting enzyme (ACE) levels of 67 U/L (normal range: 8 - 52) and decreasing alpha-fetoprotein (AFP) levels of 698 ng/mL (previously 1210 ng/mL), indicative of non-progression of the oncological disease. Finally, an excisional biopsy confirmed the presence of non-necrotizing granulomatous lymphadenitis, leading to the diagnosis of sarcoidosis-like reaction (SLR) induced by immunotherapy as the etiology of the polyadenopathy syndrome. SLR, although uncommon, is an adverse effect of ICI treatment resulting from immune system dysregulation, which can mimic disease progression. It is crucial to be aware of this adverse event and to understand the optimal management approach.


Assuntos
Antineoplásicos Imunológicos , Carcinoma Hepatocelular , Neoplasias Hepáticas , Sarcoidose , Masculino , Humanos , Adulto , Inibidores de Checkpoint Imunológico/efeitos adversos , Carcinoma Hepatocelular/tratamento farmacológico , Antineoplásicos Imunológicos/efeitos adversos , Neoplasias Hepáticas/tratamento farmacológico , Sarcoidose/induzido quimicamente , Sarcoidose/diagnóstico , Sarcoidose/tratamento farmacológico , Progressão da Doença
17.
Medicina (B Aires) ; 83(5): 816-820, 2023.
Artigo em Espanhol | MEDLINE | ID: mdl-37870342

RESUMO

We present the case of a healthy young woman who consulted for left peripheral facial palsy associated with fever, dry cough, dyspnea, and asthenia of two weeks' evolution. Physical examination revealed hypoesthesia in left T6 to T12 dermatomes and bilateral galactorrhea. In the laboratory, she presented negative viral serology, elevated erythrocyte sedimentation rate, antinuclear antibody titers, prolactin and thyroid-stimulating hormone, with positive antiperoxidase antibodies. Computed tomography showed multiple bilateral cervical, mediastinal, and hilar adenopathies, without involvement of lung parenchyma. Cerebrospinal fluid culture was negative for common germs, mycobacteria, and Xpert MTB/RIF, and cytology did not show atypia. Contrast-enhanced magnetic resonance was performed on the brain without pathological findings and on the spine with alteration of the centromedullary signal from T6 to T9 of almost the entire thickness of the cord, with posterior enhancement with gadolinium. During hospitalization, she recovered sensitivity in the left trunk and did not repeat febrile or cough episodes. She was referred to another center for mediastinoscopy with lymph node biopsy revealing the presence of numerous non-caseating granulomas compatible with sarcoidosis. It was classified as probable neurosarcoidosis and started treatment with corticosteroids with improvement of the remaining neurological symptoms. A magnetic resonance was performed three months later where the signal alteration was limited from T7 to T8. Our objective is to highlight the florid neurological presentation that made it necessary to rule out other more frequent entities and the favorable evolution even before starting a first-line scheme of treatment.


Presentamos el caso de una mujer joven sana, que consultó por parálisis facial periférica izquierda asociada a fiebre, tos seca, disnea y astenia de dos semanas de evolución. Al examen físico se evidenció hipoestesia en dermatomas D6 a D12 izquierdos y galactorrea bilateral. En el laboratorio presentaba serologías virales negativas, eritrosedimentación, títulos de anticuerpos antinucleares, prolactina y hormona tiroestimulante elevados, con anticuerpos antiperoxidasa positivos. La tomografía computarizada mostró múltiples adenopatías cervicales, mediastinales e hiliares bilaterales, sin compromiso del parénquima pulmonar. El cultivo de líquido cefalorraquídeo fue negativo para gérmenes comunes, micobacterias (Xpert MTB/RIF), y la citología no mostró atipia. Se realizó una resonancia magnética con contraste endovenoso de cerebro sin hallazgos patológicos y de columna con alteración de la señal centromedular de D6 a D9 de casi la totalidad del espesor del cordón, con refuerzo con contraste endovenoso. Durante la internación recuperó la sensibilidad en tronco izquierdo y no repitió episodios febriles o tusígenos. Se realizó mediastinoscopía con biopsia ganglionar con anatomía patológica con presencia de numerosos granulomas no caseificantes compatibles con sarcoidosis. Se clasificó como neurosarcoidosis probable e inició tratamiento con corticoides con mejoría de los síntomas neurológicos restantes, realizándose una resonancia magnética a los tres meses, donde la alteración de la señal se limitaba desde D7 a D8. Nuestro objetivo es destacar la presentación neurológica en múltiples sitios que obligó a descartar otras entidades más frecuentes, así como la evolución favorable incluso previo al inicio de un esquema de tratamiento de primera línea.


Assuntos
Doenças do Sistema Nervoso Central , Sarcoidose , Feminino , Gravidez , Humanos , Tosse , Sarcoidose/diagnóstico , Sarcoidose/tratamento farmacológico , Sarcoidose/complicações , Doenças do Sistema Nervoso Central/diagnóstico por imagem , Doenças do Sistema Nervoso Central/tratamento farmacológico , Pulmão
18.
Am J Case Rep ; 24: e940919, 2023 Oct 16.
Artigo em Inglês | MEDLINE | ID: mdl-37844027

RESUMO

BACKGROUND Sarcoidosis is a benign systemic granulomatous disorder of unknown etiology that affects multiple organs. Patients diagnosed with sarcoidosis usually present with nonspecific symptoms: fatigue, fever, weight loss, and respiratory symptoms such as cough and dyspnea; 50% of the patients are asymptomatic at the time of diagnosis. In 90% of patients, sarcoidosis targets hilar and mediastinal lymph nodes. In rare cases, it presents solely in the breast with no other symptoms. The diagnosis is established based on compatible clinical and radiological findings and supported by histological evidence in 1 or more organs of non-caseating epithelioid cell granulomas in the absence of organisms or particles. CASE REPORT We herein present a unique case of a 27-year-old woman who presented with pain and swelling in her left breast. On examination, the left breast revealed multiple, firm-hard, and tender masses. Breast ultrasound showed large loculated focally dilated ducts with significant periductal vascularity and inflammation. Fine-needle aspiration (FNA) showed an inflammatory process with granulomatous formation. An ultrasound-guided core biopsy, which was histologically consistent with granulomatous mastitis, and elevated angiotensin-converting enzyme (ACE) levels confirmed a diagnosis of sarcoidosis. The patient was started on IV steroids followed by oral prednisolone and azathioprine. During treatment, the mass size decreased and the pain substantially improved. CONCLUSIONS It is important to consider sarcoidosis in a patient who presents with only pain and swelling in the breast to ensure early diagnosis and initiate treatment, improving the patient's overall prognosis.


Assuntos
Sarcoidose , Feminino , Humanos , Adulto , Sarcoidose/complicações , Sarcoidose/diagnóstico , Sarcoidose/tratamento farmacológico , Granuloma , Biópsia por Agulha Fina , Dispneia , Dor
19.
Respir Med ; 219: 107422, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37827293

RESUMO

PURPOSE: To investigate the response to Acthar Gel® in patients with moderate to severe sarcoidosis uveitis. METHODS: This is a prospective open-label study that enrolled patients with moderate to severe sarcoidosis uveitis to receive 80 units daily of Acthar Gel for ten days followed by maintenance treatment with 80 units twice weekly. The primary outcome was the proportion of patients meeting at least one of the following variables 1) improved visual acuity, 2) resolution of intraocular inflammation, 3) ability to taper ocular or oral steroids by at least 50% or 4) reduction of cystoid macular edema, with no worsening of any single measure and no need for additional sarcoidosis therapies at 24 weeks. RESULTS: A total of nine patients were enrolled in the study. Four patients completed the full 24-week course of Acthar Gel, and three of these met the primary endpoint. Among the five patients who did not complete the 24-week course of treatment, four discontinued the treatment due to worsening ocular inflammation. One patient discontinued treatment due to severe adverse effects. The most common adverse effects were fluid retention (77%), insomnia (44%), hypertension (44%) and hyperglycemia (44%). CONCLUSIONS: We observed a clinical response to Acthar Gel in some patients with moderate to severe sarcoidosis uveitis, but a substantial proportion either failed to respond or did not tolerate the therapy. These observations may serve as preliminary data for controlled trials of Acthar Gel, but they do not support its role prior to failure of other agents.


Assuntos
Sarcoidose , Uveíte , Humanos , Estudos Prospectivos , Uveíte/tratamento farmacológico , Sarcoidose/complicações , Sarcoidose/tratamento farmacológico , Transtornos da Visão , Inflamação
20.
Am J Physiol Cell Physiol ; 325(5): C1336-C1353, 2023 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-37746695

RESUMO

Sarcoidosis is a complex inflammatory fibrotic disease that affects multiple organ systems. It is characterized by the infiltration of lymphocytes and mononuclear phagocytes, which form non-caseating granulomas in affected organs. The lungs and intrathoracic lymph nodes are the most commonly affected organs. The underlying cause of sarcoidosis is unknown, but it is believed to occur in genetically predisposed individuals who are exposed to pathogenic organisms, environmental contaminants, or self and non-self-antigens. Recent research has suggested that the microbiome may play a role in the development of respiratory conditions, including sarcoidosis. Additionally, metabolomic studies have identified potential biomarkers for monitoring sarcoidosis progression. This review will focus on recent microbiome and metabolomic findings in sarcoidosis, with the goal of shedding light on the pathogenesis and possible diagnostic and therapeutic approaches.


Assuntos
Sarcoidose , Humanos , Sarcoidose/diagnóstico , Sarcoidose/tratamento farmacológico , Sarcoidose/patologia , Pulmão/patologia
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