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BACKGROUND: The mechanisms driving the transition from inflammation to fibrosis in sarcoidosis patients are poorly understood; prognostic features are lacking. Immune cell profiling may provide insights into pathogenesis and prognostic factors of the disease. This study aimed to establish associations in simultaneous of lymphocyte subset profiles in the blood, bronchoalveolar lavage fluid (BALF), and lung biopsy tissue in the patients with newly diagnosed sarcoidosis. METHODS: A total of 71 sarcoid patients (SPs) and 20 healthy controls (HCs) were enrolled into the study. CD31, CD38, CD44, CD103 positive T lymphocytes in blood and BALF were analysed. Additionally, the densities of CD4, CD8, CD38, CD44, CD103 positive cells in lung tissue biopsies were estimated by digital image analysis. RESULTS: Main findings: (I) increase of percentage of CD3(+)CD4(+)CD38(+) in BALF and blood, and increase of percentage of CD3(+)CD4(+)CD44(+) in BALF in Löfgren syndrome patients comparing with patients without Löfgren syndrome, (II) increase of percentage of CD3(+)CD4(+)103(+) in BALF and in blood in patients without Löfgren syndrome (comparing with Löfgren syndrome patients) and increase of percentage of CD3(+)CD4(+)103(+) in BALF and in blood in more advanced sarcoidosis stage. (III) Increasing percentage of BALF CD3(+)CD4(+)CD31(+) in sarcoidosis patients when comparing with controls independently of presence of Löfgren syndrome, smoking status or stage of sarcoidosis. Several significant correlations were found. CONCLUSIONS: Lymphocyte subpopulations in blood, BALF, and lung tissue were substantially different in SPs at the time of diagnosis compared to HCs. CD3(+)CD4(+)CD31(+) in BALF might be a potential supporting marker for the diagnosis of sarcoidosis. CD3(+)CD4(+)CD38(+) in BALF and blood and CD3(+)CD4(+)CD44(+) in BALF may be markers of the acute immune response in sarcoidosis patients. CD4(+)CD103(+) T-cells in BALF and in blood are markers of the persistent immune response in sarcoidosis patients and are potential prognostic features of the chronic course of this disease.

Background: Sarcoidosis is a systemic inflammatory disease of unknown etiology that can affect virtually any organ. Löfgren syndrome, characterized by erythema nodosum, hilar lymphadenopathy, fever and polyarthritis, represents only 20-30% of the cases of sarcoidosis. Only 2- 10% of the cases feature hypercalcemia. Case: The case of a 42-year-old Hispanic woman with a history of erythema nodosum and three weeks of nausea, emesis, constipation, asthenia, adynamia, polydipsia, and somnolence, concomitant with hypercalcemia, but normal parathyroid hormone (PTH) and 25-hydroxyvitamin D has been presented. The initial diagnostic approach was based upon the suspicion of multiple myeloma or bone metastases; however, further findings of bilateral hilar lymphadenopathy, elevated serum angiotensin-converting enzyme (ACE) and a right inguinal lymphadenomegaly suggested an alternate diagnosis. Biopsy of the latter supported sarcoidosis as the diagnosis. She was successfully treated in the hospital with zoledronic acid and as an outpatient with immunosuppressive therapy. Persistence of a previously undisclosed symptom of oligomenorrhea led to the detection of hyperprolactinemia secondary to hypophyseal infiltration, refractory to immunosuppressive therapy but with an adequate response to cabergoline. Conclusion: This case strays from Löfgren Syndrome’s expected behavior, presenting a more progressive, multisystemic disease. This case report was written in adhThis case strays from Löfgren Syndrome’s expected behavior, presenting a more progressive, multisystemic disease. This case report was written in adherence to the CARE guidelines of 2013 to include information in it.erence to the CARE guidelines of 2013 to include information in it.

Background Sarcoidosis is a multisystemic granulomatous disease that affects the lungs in more than 90% of the patients. It is associated with a variable clinical course and considering all the different forms of disease presentation, there are an absence of reliable clinical prognostic markers that can predict the outcome at diagnosis. Objective The aim of our study was to investigate prognostic factors at diagnosis in a population of sarcoidosis patients from Northern Portugal. Methods A group of 110 patients with chronic evolution was compared with 129 patients with disease resolution regarding their clinical, radiologic and laboratorial features. Results We found a positive association between the chronic forms and lung function impairment, radiologic stage II, lower lymphocyte CD4/CD8 and extrapulmonary disease. Lofgren syndrome and asthenia instead had a protective significant association to chronicity. Our final logistic regression model found a significant independent association between age (adjusted OR = 1.06), extrapulmonary involvement (adjusted OR = 2.68), Lofgren's syndrome (adjusted OR = 0.15) with outcome toward chronicity. Conclusions In this first study searching for prognostic factors at diagnosis in a Northern Portuguese population, we found clinical prognosis factors that have been described in other populations that should be considered whenever sarcoidosis is identified.

The objective:to compare the results of follow-up over patients with a mild form of pulmonary sarcoidosis who received alpha-tocopherol and those receiving no treatment.Subjects and methods.The results of follow-up over 80 sarcoidosis patients were retrospectively analyzed – two groups of 40 patients enrolled according to the copy – pair principle (gender, age, initial X-ray stage, Löfgren syndrome, and follow-up duration). The only sign that statistically distinguished the groups was a high frequency of complaints about weakness in those who received vitamin E – 55% versus 30% (p < 0.05). The follow-up period for patients in both groups was a year or more (a control point). The database was maintained and processed using SPSS-18, Windows 10. A clear advantage of vitamin E administration was revealed when comparing the data of computed tomography, which resulted in improvement in 87.5%, and the disease progressed in 2.5%, while in the group receiving no treatment – 60 and 12.5%, respectively. The OR of improvement in computed tomography with alpha-tocopherol versus the group receiving no treatment made 4.67 (95% CI, 1.51-14.46).Conclusion.The use of vitamin E in mild forms of thoracic sarcoidosis gives a better result compared to the group receiving no treatment.

Background Sarcoidosis is a heterogeneous disease with high variability in natural history and clinical spectrum. The study aimed to reveal different clinical phenotypes of patients with similar characteristics and prognosis. Methods Cluster analysis including 26 phenotypic variables was performed in a large cohort of 694 sarcoidosis patients, collected and followed-up from 1976 to 2018 at Bellvitge University Hospital, Barcelona, Spain. Results Six homogeneous groups were identified after cluster analysis: C1 (n=47; 6.8%), C2 (n=85; 12.2%), C3 (n=153; 22%), C4 (n=29; 4.2%), C5 (n=168; 24.2%), and C6 (n=212; 30.5%). Presence of bilateral hilar lymphadenopathy (BHL) ranged from 65.5% (C4) to 97.9% (C1). Patients with Lofgren syndrome (LS) were distributed across 3 phenotypes (C1, C2, and C3). In contrast, phenotypes with pulmonary (PS) and/or extrapulmonary sarcoidosis (EPS) were represented by groups C4 (PS 100% with no EPS), C5 (PS 88.7% plus EPS), and C6 (EPS). EPS was concentrated in groups C5 (skin lesions, peripheral and abdominal lymph nodes, and hepatosplenic involvement) and C6 (skin lesions, peripheral lymph nodes, and neurological and ocular involvement). Unlike patients from LS groups, most patients with PS and/or EPS were treated with immunosuppressive therapy, and evolved to chronicity in higher proportion. Finally, the cluster model worked moderately well as a predictive model of chronicity (AUC=0.705). Conclusion Cluster analysis identified 6 different clinical patterns with similar phenotypic variables and predicted chronicity in our large cohort of patients with sarcoidosis. Classification of sarcoidosis into phenotypes with prognostic value may help physicians to improve the efficacy of clinical decisions.

BackgroundSarcoidosis most commonly affects lungs and intrathoracic lymph nodes, but any other organ can be involved. In epidemiological studies, many occupational and environmental exposures have been linked to sarcoidosis but their relationship with the disease phenotype has barely been studied.ObjectiveTo investigate how occupational and environmental exposures prior to diagnosis relate to organ involvement in patients with sarcoidosisMethodsWe retrospectively studied patients seen at a sarcoidosis clinic between 2017 and 2020. Patients were included if they had a clinical presentation consistent with sarcoidosis and histologically confirmed epithelioid granulomas or had Löfgren syndrome. In a case–case analysis using multivariable logistic regression we calculated odds ratios (OR) of prespecified exposure categories (based on expert ascertainment) for cases with a given organ involvement versus cases without this organ involvement.ResultsWe included 238 sarcoidosis patients. Sarcoidosis limited to pulmonary involvement was associated with exposure to inorganic dust prior to diagnosis (OR 2.11; 95% confidence interval [CI] 1.11–4.17). Patients with liver involvement had higher odds of contact with livestock (OR 3.68; 95% CI 0.91–12.7) or having jobs with close human contact (OR 4.33; 95% CI 1.57–11.3) than patients without liver involvement. Similar associations were found for splenic involvement (livestock: OR 4.94, 95% CI 1.46–16.1; close human contact: OR 3.78; 95% CI 1.47–9.46). Cardiac sarcoidosis was associated with exposure to reactive chemicals (OR 5.08; 95% CI 1.28–19.2) or livestock (OR 9.86; 95% CI 1.95–49.0). Active smokers had more ocular sarcoidosis (OR 3.26; 95% CI 1.33–7.79).ConclusionsOur study indicates that, in sarcoidosis patients, different exposures might be related to different organ involvements—hereby providing support for the hypothesis that sarcoidosis has more than one cause, each of which may promote a different disease phenotype.

PubMed Yılı: 2019 The relationship between sarcoidosis and malignancy is not clear yet. We retrospectively evaluated 131 sarcoidosis patients followed-up at the single Rheumatology center. The incidence of malignancies was investigated in this cohort. A total of 6 (4.6%) patients with malignancy were identified in our cohort of 131 patients with sarcoidosis. Hodgkin lymphoma (HL) was detected in three patients, followed by one patient with breast cancer, one patient with thyroid cancer and one patient with testicular cancer. All patients had chronic sarcoidosis with pulmonary involvement, and only 1 patient had acute sarcoidosis with Löfgren's syndrome. HL developed concomitantly with sarcoidosis in one patient while other two patients developed disease before and after sarcoidosis diagnosis. Two patients with solid tumors developed malignancy years before sarcoidosis diagnosis, while one patient developed thyroid cancer during sarcoidosis follow-up. All 6 sarcoidosis–malignancy patients survived after six year years follow up. We found low incidence of malignancy in patients with sarcoidosis in our small cohort. The sarcoidosis–malignancy relationship can only be a coincidence and/or can be explained by a common pathogenesis. New prospective studies involving large patients series are needed in this regard. © 2019 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología WOS:000608393600010 31153889

A 58-year-old man presented with fever, erythema nodosum, and arthralgia of knee and ankle joints. Laboratory data showed signs of inflammation, and chest x-ray revealed bilateral hilar lymphadenopathy. 18F-FDG PET/CT was acquired to analyze the underlying causes and revealed FDG accumulation in thoracic lymphadenopathy and in subcutaneous lesions and periarticular uptake. This typical triad is known as Löfgren syndrome, an acute manifestation of sarcoidosis and which, as this case shows, can be visualized on 18F-FDG PET/CT. It is important to recognize this syndrome and to discriminate it from the classic presentation of sarcoidosis because of its different diagnostic and therapeutic consequences.

A 17-year-old previously healthy man presented with a 4-week history of progressive bilateral leg swelling with discomfort and erythema, but no signs of arthritis or erythema nodosum. An incidental finding of a query pulmonary nodule on chest X-ray prompted chest CT for further evaluation, revealing bilateral hilar and mediastinal lymphadenopathy. The patient then underwent endobronchial ultrasound and transbronchial needle aspiration biopsies of mediastinal lymph nodes. Biopsies and bronchoalveolar lavage samples were negative for microbiology, including mycobacterial culture. Pathology demonstrated non-caseating granulomas consistent with a diagnosis of sarcoidosis. Weeks later, he developed arthralgias of the left metacarpophalangeal joints and erythema nodosum and was diagnosed with Löfgren syndrome, a phenomenon rarely described in the paediatric population. This case highlights an approach to lower extremity swelling as well as hilar lymphadenopathy in the paediatric population. In addition, it emphasises the importance of multidisciplinary teamwork for accurate and timely diagnoses.

Background Musculoskeletal (MSK) sarcoidosis presents with a variety of clinical phenotypes. Four subtypes of MSK sarcoidosis have been identified to date: Lofgren syndrome, chronic sarcoid arthritis, osseous sarcoidosis, sarcoid myopathy. Each subtype has been reported with varying incidence mainly due to lack of universal classification criteria. Methods We performed a retrospective chart review of patients with MSK sarcoidosis at a single academic center between January 2000 and December 2014. Descriptive statistics were used to describe the proportion of patients with sarcoidosis who had the 4 MSK syndromes of interest, demographic characteristics and therapeutic agents used. Results A cohort of 58 patients with MSK manifestations were identified among 1016 patients with sarcoidosis. Frequency of subtypes include: Lofgren syndrome 46.6%, osseous sarcoidosis 25.9%, chronic sarcoid arthritis 24.1% and sarcoid myopathy 6.9%. The cohort was predominantly female (43/58 patients, 74%) and Caucasian (48/58 patients, 82.8%). Mean age was 47.2 years. One patient had overlap of osseous sarcoidosis and chronic sarcoid arthritis, another patient initially had Lofgren syndrome and later developed chronic sarcoid arthritis. Sarcoid myopathy patients presented with myalgia more often than muscle weakness. Conclusion We identified a large cohort of MSK sarcoidosis and determined the prevalence of all 4 subtypes. In patients who do develop MSK manifestations of sarcoidosis, they are commonly a part of the initial presentation of sarcoidosis. There is an unmet need to establish standardized classification criteria for the 4 MSK sarcoidosis syndromes.

Purpose To describe the ocular manifestations in a cohort of patients with systemic sarcoidosis (SS). Recent advances in the pathophysiology, diagnosis, and therapy of SS are also discussed. Methods Data from 115 Italian patients diagnosed between 2005 and 2016 were retrospectively reviewed. All but the first 17 patients underwent a comprehensive ophthalmologic examination. The diagnosis was based on clinical features, the demonstration of non-caseating granulomas in biopsies from involved organs, and multiple imaging techniques. Data on broncho-alveolar lavage fluid analysis, calcemia, calciuria, serum angiotensin-converting enzyme levels and soluble interleukin-2 receptor levels were retrieved when available. Results Ocular involvement, detected in 33 patients (28.7%), was bilateral in 29 (87.9%) and the presenting feature in 13 (39.4%). Anterior uveitis was diagnosed in 12 patients (36.4%), Löfgren syndrome and uveoparotid fever in one patient each (3%), intermediate uveitis in 3 patients (9.1%), posterior uveitis in 7 (21.2%), and panuveitis in 9 (27.3%). First-line therapy consisted of corticosteroids, administered as eyedrops (10 patients), sub-Tenon’s injections (1 patient), intravitreal implants (9 patients), or systemically (23 patients). Second-line therapy consisted of steroid-sparing immunosuppressants, including methotrexate (10 patients) and azathioprine (10 patients). Based on pathogenetic indications that tumor necrosis factor (TNF)-α is a central mediator of granuloma formation, adalimumab, targeting TNF-α, was employed in 6 patients as a third-line agent for severe/refractory chronic sarcoidosis. Conclusion Uveitis of protean type, onset, duration, and course remains the most frequent ocular manifestation of SS. Diagnostic and therapeutic advancements have remarkably improved the overall visual prognosis. An ophthalmologist should be a constant component in the multidisciplinary approach to the treatment of this often challenging but intriguing disease.

The COVID-19 pandemic represents a challenge for rheumatologists: how activity of rheumatological diseases or disease-modifying therapies may affect the outcome of SARS-CoV-2 infection is only beginning to be understood. Gianfrancesco et al provided important insights in this regard by evaluating the hospitalisation rate of 600 patients with rheumatological diseases from 40 different countries.1 They found that glucocorticoid treatment in doses equivalent with prednisolone 10 mg/day or higher was associated with an increased hospitalisation rate. Here, we describe the case of a 50-year-old man who was diagnosed with Lofgren syndrome in January 2019 presenting with cough, bilateral ankle joint swelling and mediastinal lymphadenopathy. ACE (37.3 U/L, normal range

We report the case of a 40-year-old man presenting at the emergency department with ankle and feet edema due to acute sarcoidosis (Lofgren syndrome). The diagnosis was suggested based on an ultrasound examination showing bilateral asymmetrical tenosynovitis of the flexor tendons with hypervascular subcutaneous cellulitis without any significant ankle and foot joint effusion or synovitis. This case report highlights the crucial role of ultrasound imaging as the first-line diagnostic tool in the investigation of a non-specific clinical condition, when performed by a knowledgeable sonographer with attention to detail. Radiologists performing ultrasound should be aware of the presenting ultrasound pattern of acute soft tissue sarcoidosis, and should include this entity in the differential diagnosis of patients with distal lower extremity swelling, tenosynovitis and hypervascular subcutaneous cellulitis, in order to narrow down the differential diagnosis and facilitate the clinical workup of the patient.

La sarcoidosis se considera una enfermedad huérfana, con una prevalencia a nivel mundial de 12,5 casos por cada 100 000 personas (1). Su incidencia varía ampliamente en el mundo: en los Estados Unidos y Europa oscila entre 10 y 40 casos por cada 100 000 personas, en contraste con Japón, en donde se registra 1 caso por cada 100 000 personas (0,73 casos por 100 000 varones y 1,28 casos por 100 000 mujeres) (2). En Colombia no contamos con estudios epidemiológicos al respecto; nuestra información local se limita a informes y series de casos (3, 4). En cuanto a las múltiples presentaciones de esta enfermedad, encontramos las artropatías, que pueden ser agudas o crónicas, siendo el prototipo de la artropatía aguda el síndrome de Löfgren (SL), manifestación inicial de la enfermedad en el 10 % de los casos en Estados Unidos y hasta del 50 % de los casos en España. A continuación, presentamos 1 caso de sarcoidosis que debuta con SL, en la ESE Hospital Universitario del Caribe, Cartagena, Colombia. La sarcoidosis es una enfermedad con múltiples presentaciones clínicas y requiere de alta sospecha para aproximarse al diagnóstico, sobre todo en presentaciones clínicas de baja frecuencia.

Introducción y Objetivos La litiasis en vesículas seminales es una entidad rara, diferentes alteraciones anatómicas que obstruyen los ductos eyaculadores favorecen la precipitación y formación de cálculos. Nuestro objetivo es actualizar el enfoque diagnóstico y terapéutico a propósito de un paciente con esa patología. Materiales y Métodos Se describe el caso de un paciente de 42 años con sintomatología urinaria no específica, asociado a dolor perineal, diagnosticado posteriormente con Síndrome de Löfgren. Dada la persistencia de síntomas, se le realiza UROTAC con evidencia de calcificaciones bilaterales en vesículas seminales. Por tratarse de un hallazgo infrecuente y sintomático, realizamos una revisión de la literatura que nos permitiera orientar el tratamiento. Resultados La litiasis vesicular se presenta con sintomatología poco específica. Existen ciertos factores que favorecen la formación de cálculos en las vesículas seminales como lo son el microambiente propio de las vesículas seminales y alteraciones anatómicas que favorecen la obstrucción del tracto urinario. El método de elección para el diagnóstico es la ultrasonografía transrectal, la cual se debe realizar en todos los pacientes con sospecha clínica y síntomas urinarios no específicos. El tratamiento de elección es la vesiculoscópia transuretral con litotricia. Actualmente se está evaluando la realización de la vesiculoscópia transuretral. Conclusiones La litiasis de vesículas seminales es una entidad rara, con presentación clínica variable. Su diagnóstico y manejo se convierten en un desafío para el urólogo, debido a que no existe una alternativa de manejo estándar, el manejo dependerá de la experiencia en técnicas endourológicas y disponibilidad de equipos.

Sarcoidosis is a chronic granulomatous disease that has been linked to exposure to certain environmental antigens, including previous contact with infectious agents, and a variety of other organic and inorganic particles. There is an acute form of presentation of this disease that courses with stereotypical clinical findings known as Lofgren's syndrome. A case is presented of a female patient with all the characteristic features of this syndrome.

Background: Sarcoidosis is a systemic granulomatous disease of unknown etiology. Epidemiological studies of different populations are essential because clinical presentation, organ involvement, disease severity, and prognosis vary significantly according to region and population. The aim of this study was to assess epidemiological and clinical characteristics, staging factors, and clinical course in patients with sarcoidosis from a tertiary hospital in Oporto, Portugal. Methods: A retrospective analysis of patients with sarcoidosis and at least 2 years of follow-up evaluated at the Centro Hospitalar de São João between 2000 and 2014. Results: We identified 409 patients with sarcoidosis (females, 58.9%; mean age at diagnosis, 38.9 ± 13.4 years; smokers, 14.4%]. All the patients were diagnosed according to the ERS/ATS/WASOG consensus statement and 64.1% had evidence of noncaseating epithelioid cell granulomas in biopsy specimens. Bronchoalveolar lavage was performed as part of the diagnostic work-up in 289 patients and 90.2% had lymphocytosis (CD4/CD8 ratio â¥Â 3.5 in 60.9% of cases). Exertion dyspnea, cough, and constitutional symptoms were the most common presenting symptoms; 10.1% of patients were asymptomatic, 22.8% had Löfgren syndrome, and 50.5% had extrathoracic involvement. Radiographic stages of disease according to the Scadding criteria were as follows: stage 0 (5.2%), stage I (33.7%), stage II (47.0%), stage III (8.4%), and stage IV (5.7%). Impaired respiratory function was observed in 45.6% patients and was mostly mild. Systemic treatment was administered in 58.6% of cases. Overall, 45.3% of patients experienced disease resolution. Conclusion: The epidemiological and clinical characteristics of this cohort of patients with sarcoidosis from the Oporto region in northern Portugal revealed epidemiological and clinical characteristics that were generally similar to those described in other Western Europe populations and in the US ACCESS study. However, we found a higher proportion of patients who progressed to chronic forms. Keywords: Sarcoidosis, Oporto, Löfgren syndrome, Extrathoracic involvement, Scadding criteria

Skin manifestations of sarcoidosis occur in up to 30% of cases. This review summarizes and illustrates in detail the differences between specific and unspecific skin manifestations of sarcoidosis. Important differential diagnoses, such as tuberculosis, cutaneous lymphoma and syphilis have to be excluded. The indications for systemic treatment are primarily determined by the extent of organ involvement and also by the cosmetic impairment.