Renal manifestations of sarcoidosis: from accurate diagnosis to specific treatment

ABSTRACT Sarcoidosis is a multisystem granulomatous disease characterized by epithelioid noncaseating granulomas associated with clinical and radiologic findings. The cause of this disease is still uncertain. Sarcoidosis affects mostly lungs and lymph nodes and is not usually considered a urological disease, therefore, this etiology may be overlooked in several urological disorders, such as hypercalcemia, hypercalciuria and nephrolithiasis. It affects all races and genders. This review aims to describe the urological manifestations of sarcoidosis and to elucidate how the disease may affect the management of numerous urological conditions.

Sarcoidosis subcutánea sin compromiso sistématico / Subcutaneous sarcoidosis without compromise systemic

We present the case of a 52 year old woman with a history of bilateral ovarian cancer operated. The patient attended the dermatology unit for a sudden onset of a left plantar nodule of three weeks of evolution, painful on walking, with no other history in particular. At physical examination, the patient presented a surface nodule slightly hyperpigmented, with ill-defined edges, round shape, firm consistency, mobile and painful to compression, one cm of diameter, in the middle third of the left plant. No concomitant scars are seen. Doppler soft tissue ultrasound is requested, showing a granulomatous nodule on the surface of the plantar aponeurosis. The biopsy lesion revealed non-caseating granulomas in cell tissue, PAS and Ziehl-Neelsen staining are negative A left plantar subcutaneous sarcoidosis is diagnosed, systemic involvement is ruled out. Within the first year of follow-up, there are no new lesions or findings compatible with systemic disease. (AU)

Sarcoidose ocular: a nossa realidade nos últimos seis anos / Ocular sarcoidosis: our reality for the past six years

RESUMO Introdução: Na sarcoidose, as manifestações oculares são comuns e podem constituir a manifestação inicial da doença ou mesmo a única. O objetivo deste trabalho foi analisar os parâmetros demográficos, manifestações clínicas, terapêutica e principais complicações oculares em doentes com sarcoidose ocular. Métodos: Estudo descritivo e retrospectivo que incluiu doentes com o diagnóstico de sarcoidose ocular, observados nas consultas de Inflamação Ocular e de Doenças Auto-Imunes do Hospital Prof. Doutor Fernando Fonseca, no período entre 2009 e 2015. Resultados: Foram identificados 11 doentes com o diagnóstico de sarcoidose ocular, com predomínio do sexo feminino (54,5%) e caucasianos. A média da idade ao diagnóstico foi de 45±14 anos. A sarcoidose manifestou-se de forma exclusivamente ocular em 36% dos casos. O envolvimento ocular foi a manifestação inicial em 90,9% dos casos. Identificaram-se 9 casos de uveíte, 1 de esclerite anterior nodular e 1 de queratite intersticial. O tratamento com corticoterapia tópica foi realizado em 100% dos casos, sendo o tratamento único em apenas 1 doente. Nos restantes, foi necessário associar corticoterapia oral. Em 4 desses doentes, pela gravidade da doença e atingimento binocular, utilizou-se também corticoterapia pulsada endovenosa. O tratamento adjuvante imunossupressor mais frequentemente utilizado foi o metotrexato (45%). Um doente necessitou de terapia biológica com infliximabe para controle da doença. Conclusão: A manifestação ocular mais comum foi a uveíte, com predomínio da panuveíte. O tratamento mais utilizado e com maior taxa de controle da doença foi a corticoterapia sistêmica em associação com o metotrexato.

ABSTRACT Purpose: In sarcoidosis, ocular manifestations are common and can be the initial or even the only clinical manifestation. The aim of this study was to analyze the demographic parameters, clinical manifestations, treatment and the major ocular complications in patients with ocular sarcoidosis. Methods: We conducted a descriptive and retrospective study that included patients with the diagnosis of ocular sarcoidosis, followed by inflammatory ophthalmology and immune-mediated disease consults at the Prof. Doutor Fernando Fonseca Hospital, between 2009 and 2015. Results: Eleven patients with the diagnosis of ocular sarcoidosis were identified, with a predominance of females (54,5%) and Caucasians. The average age at diagnosis was 45 ± 14 years. Sarcoidosis was exclusively ocular in 36%. The first manifestation of sarcoidosis was eye disease in 90.9 % of cases. Nine cases of uveitis, one of nodular scleritis and one of interstitial keratitis were observed. Topical corticoid treatment was applied in 100% of cases, with only one achieving remission of the disease. Oral corticoid treatment was necessary in 10 cases, four of which needed a high dose methylprednisolone induction. Methotrexate was the adjunctive immunosuppressive treatment of choice in 45% of cases. There was one refractory case for conventional immunosuppressive therapy, having achieved remission with biologic agent infliximab. Conclusion: Uveitis was the commonest ocular manifestation, and there was a predominance of panuveitis. Systemic corticoid and methotrexate were the most used immunosuppressive treatments for maintaining the controlled stated of the disease.

Administering the Sarcoidosis Health Questionnaire to sarcoidosis patients in Serbia / Aplicação do Sarcoidosis Health Questionnaire em pacientes com sarcoidose na Sérvia

Objective: The aim of this study was to use a Serbian-language version of the disease-specific, self-report Sarcoidosis Health Questionnaire (SHQ), which was designed and originally validated in the United States, to assess health status in sarcoidosis patients in Serbia, as well as validating the instrument for use in the country. Methods: This was a cross-sectional study of 346 patients with biopsy-confirmed sarcoidosis. To evaluate the health status of the patients, we used the SHQ, which was translated into Serbian for the purposes of this study. We compared SHQ scores by patient gender and age, as well as by disease duration and treatment. Lower SHQ scores indicate poorer health status. Results: The SHQ scores demonstrated differences in health status among subgroups of the sarcoidosis patients evaluated. Health status was found to be significantly poorer among female patients and older patients, as well as among those with chronic sarcoidosis or extrapulmonary manifestations of the disease. Monotherapy with methotrexate was found to be associated with better health status than was monotherapy with prednisone or combination therapy with prednisone and methotrexate. Conclusions: The SHQ is a reliable, disease-specific, self-report instrument. Although originally designed for use in the United States, the SHQ could be a useful tool for the assessment of health status in various non-English-speaking populations of sarcoidosis patients.

Objetivo: O objetivo deste estudo foi utilizar uma versão no idioma sérvio do Sarcoidosis Health Questionnaire (SHQ), um questionário de autorrelato doença-específico, concebido e originalmente validado nos EUA, para verificar o estado de saúde de pacientes com sarcoidose na Sérvia, além de validar o instrumento para uso no país. Métodos: Estudo transversal com 346 pacientes com sarcoidose confirmada por biópsia. Para avaliar o estado de saúde dos pacientes, utilizamos o SHQ, o qual foi traduzido para o sérvio para os propósitos deste estudo. Comparamos os escores do SHQ por gênero, idade, duração da doença e tratamento. Escores do SHQ mais baixos indicam pior estado de saúde. Resultados: Os escores do SHQ demonstraram diferenças no estado de saúde entre os subgrupos de pacientes avaliados. O estado de saúde foi significativamente pior entre as mulheres e pacientes mais velhos, assim como entre aqueles com sarcoidose crônica ou com manifestações extrapulmonares da doença. A monoterapia com metotrexato associou-se com melhor estado de saúde do que a monoterapia com prednisona ou a terapia combinada com prednisona e metotrexato. Conclusões: O SHQ é um instrumento de autorrelato doença-específico confiável. Embora originalmente concebido para uso nos EUA, o SHQ pode ser uma ferramenta útil na avaliação do estado de saúde de populações de pacientes com sarcoidose em vários países de língua não inglesa.

Sarcoidosis aguda: variante de Síndrome de Lõfgren sin eritema nodoso / Acute sarcoidosis: erythema nodosum-free variant of Lõfgren’s Syndrome

El síndrome de Lõfgren, es una variante aguda de la sarcoidosis, que se caracteriza por fiebre, eritema nodoso, adenomegalias hiliares pulmonares y artritis. En general, tiene un curso benigno y autolimitado, que contrasta con las formas crónicas que requieren uso de corticoides y tienen tendencia a la recidiva. Se describe aquí el caso clínico de un paciente joven, de sexo masculino, con artritis pero sin eritema nodoso, lo que dificultó el planteo diagnóstico de síndrome de Lõfgren. Se realiza además una breve descripción comparativa entre la presentación clínica de la sarcoidosis crónica y el síndrome de Lõfgren...

Tuberculosis Lymphadenitis in a Well Managed Case of Sarcoidosis.

Differentiation between tuberculosis (TB) and sarcoidoisis is sometimes extremely difficult. Sequential occurrence of sarcoidosis and TB in the same patient is uncommon. We present the case of a young man, with a proven diagnosis of sarcoidosis who later developed TB after completion of treatment for sarcoidosis. A 32-year-old male patient presented with low-grade fever since two months. Physical examination revealed cervical lymphadenopathy. Initial fine needle aspiration cytology (FNAC) of the cervical lymph node was suggestive of granulomatous inflammation; the chest radiograph was normal. Repeat FNAC from the same lymph node was suggestive of reactive lymphoid hyperplasia. The patient was treated with antibiotics and followed-up. He again presented with persistence of fever and lymphadenopathy and blurring of vision. Ophthalmological examination revealed uveitis, possibly due to a granulomatous cause. His repeat Mantoux test again was non-reactive; serum angiotensin converting enzyme (ACE) levels were raised. This time an excision biopsy of the lymph node was done which revealed discrete, non-caseating, reticulin rich granulomatous inflammation suggestive of sarcoidosis. The patient was treated with oral prednisolone and imporved symptomatically. Subsequently, nearly nine months after completion of corticosteroid treatment, he presented with low-grade, intermittent fever and a lymph node enlargement in the right parotid region. FNAC from this lymph node showed caseating granulomatous inflammation and the stain for acid-fast bacilli was positive. He was treated with Category I DOTS under the Revised National Tuberculosis Control Programme and improved significantly. The present case highlights the need for further research into the aetiology of TB and sarcoidosis.

Sarcoidosis faringo-laríngea / Pharinx-laryngeal sarcoidosis

La sarcoidosis es una enfermedad inflamatoria granulomatosa crónica multisistémica, de etiología desconocida, que afecta principalmente el aparato respiratorio, sobre todo el inferior, y a adultos jóvenes, y es rara en la vía respiratoria superior (fosas y senos paranasales, y laringe). Se manifiesta de forma muy inespecífica, con clínica secundaria a obstrucción de la vía aérea. El diagnóstico se establece cuando los hallazgos clínico-radiológicos se apoyan en granulomas tuberculoides no caseificantes. El tratamiento de base son medidas de soporte, debido a la remisión espontánea…

The sarcoidosis is an inflammatory granulomatos multisystemic chronic disease of unknown etiology, that mainly affects the respiratory tract, mainly the lower one and in young adults; being rare the affectation of the upper respiratory tract (nose, paranasal sinus and larynx). It manifests with many unspecific form, and with symptoms secondary to the airways obstruction. The diagnosis is established when the clinical and radiological findings are supported in tuberculoid granulomas without caseum. The base of the treatment are support measurements due to the spontaneous remission…

Características epidemiológicas de pacientes com sarcoidose na cidade do Rio de Janeiro / Epidemiological characteristics of sarcoidosis patients in the city of Rio de Janeiro, Brazil

OBJETIVO: Analisar as características epidemiológicas de pacientes com sarcoidose na cidade do Rio de Janeiro. MÉTODOS: Estudo descritivo, caso-controle, envolvendo 100 pacientes com sarcoidose acompanhados no Hospital Universitário Pedro Ernesto, localizado na cidade do Rio de Janeiro, entre 2008 e 2010. O diagnóstico de sarcoidose foi baseado em critérios clínicos, radiográficos, laboratoriais e histopatológicos. RESULTADOS: A doença predominou em mulheres (65 por cento), na faixa de 35-40 anos (variação: 7-69 anos), embora houvesse um segundo pico na população de aproximadamente 55 anos. A dispneia foi o sintoma mais comum (47 por cento), assim como o achado radiográfico de comprometimento pulmonar e linfonodal (estágio II; 43 por cento), seguido por estágio III (20 por cento), estágio 1(19 por cento), estágio 0 (15 por cento) e estágio IV (3 por cento). Nenhum paciente apresentou derrame pleural ou baqueteamento digital no diagnóstico. O PPD foi não reator em 94 pacientes. Os achados espirométricos no diagnóstico foram normais em 61 pacientes; indicativos de distúrbio ventilatório obstrutivo, em 21; e indicativos de distúrbio ventilatório restritivo, em 18. Os sítios de biópsia mais comuns foram os pulmões (principalmente por broncoscopia) e a pele, que confirmaram o diagnóstico em 56 por cento e 29 por cento dos casos, respectivamente. O tratamento com prednisona foi iniciado em 75 por cento dos pacientes e mantido por mais de 2 anos em 19,7 por cento. CONCLUSÕES: Este estudo corrobora vários achados relatados em outros estudos sobre as características epidemiológicas de pacientes com sarcoidose.

OBJECTIVE: To analyze the epidemiological characteristics of sarcoidosis patients in the city of Rio de Janeiro, Brazil. METHODS: A descriptive, case-control study involving 100 sarcoidosis patients under outpatient treatment between 2008 and 2010 at the Pedro Ernesto University Hospital, located in the city of Rio de Janeiro, Brazil. The diagnosis of sarcoidosis was based on clinical, radiological, biochemical, and histopathological criteria. RESULTS: There was a predominance of females in the 35-40 year age bracket (range, 7-69 years), who accounted for 65 percent of the sample, although there was a second peak at approximately 55 years of age. The most common symptom was dyspnea (in 47 percent), and the most common radiological findings were pulmonary and lymph node involvement (stage II; in 43 percent), followed by stage III (in 20 percent), stage I (in 19 percent), stage 0 (in 15 percent), and stage IV (in 3 percent). No pleural effusion or digital clubbing was observed at diagnosis. The tuberculin skin test was negative in 94 patients. Spirometric findings at diagnosis were normal in 61 patients; indicative of obstructive lung disease in 21; and indicative of restrictive lung disease in 18. The most common biopsy sites were the lungs (principally by bronchoscopy) and the skin, the diagnosis being confirmed by biopsy in 56 percent and 29 percent of the cases, respectively. Treatment with prednisone was initiated in 75 percent of the patients and maintained for more than 2 years in 19.7 percent. CONCLUSIONS: This study corroborates the findings of previous studies regarding the epidemiological characteristics of sarcoidosis patients.

Sarcoidosis in an athlete

Sarcoidosis is a multisystem granulomatous disorder of unknown etiology, characterized by "noncaseating granulomas" in different organs. Clinical signs are variable and dependent on the organ involved. Although it is often asymptomatic in athletes, considering the high level of athletic performance and the related risks due to the potential heart involvement, a particular diagnostic flow-chart to consider some other diagnoses is required. The present case report aimed to focus on the clinical approach in case of a progressive weakness associated with a reduction in global performance of an athlete. Since October 2008 a 33-year-old Scandinavian professional soccer player has shown splitting headache, fever and impaired exercise tolerance. Despite some clinical aspects and symptoms that could address diagnosis of granulomatosis according to the current guidelines, the first hypothesis was indicative of a possible viral infection. Therefore, the athlete had received a drug-therapy resolving the headache and fever. However, because of the persisting weakness, several other clinical possibilities were evaluated following a more complete diagnostic flow-chart, blood and instrumental exams. This case report focuses on the substantial absence of symptoms during the granulomatosis disease, which makes the differential diagnosis to be often complicating. Indeed, several additional exams are required in order to establish the presence of "Non-evolutive sarcoidosis- stage I", for which the therapy is no mandatory

Insuficiência renal aguda secundária à sarcoidose / Acute renal failure secondary to sarcoidosis

A sarcoidose é uma doença sistêmica de etiologia desconhecida, caracterizada pela inflamação crônica granulomatosa, que acomete com maior frequencia os pulmões, a pele e os olhos e, muito raramente, detectamos envolvimento renal na patologia. relatamos aqui o caso de uma paciente de 47 anos, branca, internada em uma unidade de emergência de Hospital Universitário, devido a sintomas e achados radiológicos sugestivos de pneumonia. Desde o início da internação ela se apresentava com quadro laboratorial de insuficiência renal aguda, inicialmente atribuída ao histórico recente de uso abusivo de anti-inflamatórios. No entanto, devido à evolução arrastada e aparentemente desfavorável, inclusive com necessidade de terapia renal substitutiva (TRS), a paciente foi submetida a uma biópsia renal para esclarecimento diagnóstico e avaliação prognóstica. A descrição histológica mostrava um quadro de nefrite intersticial aguda granulomatosa, característico de sarcoidose renal, diagnóstico confirmado após revisão do prontuário médico, que revelava internação anterior devido a evento pulmonar da doença. Iniciado tratamento com prednisoma 1mg/kg/dia, a paciente evoluiu com melhora do quadro de insuficiência renal aguda, não mais necessitando de TRS. Atualmente, a paciente é mantida em acompanhamento ambulatorial com função renal estável.

The Sarcoidosis is a systemic disease of unknown etiology characterized by chronic granulomatous inflammation, which affects most frequently the lungs, skin and eyes and very rarely detected renal involvement in pathology. report here the case of a 47 year old white, admitted in an emergency unit of University Hospital because of symptoms and radiological findings suggestive of pneumonia. Since the beginning of hospitalization she performed with a laboratory diagnosis of acute renal failure, initially attributed to the recent history of abuse of anti-inflammatory drugs. However, due to unfavorable prolonged evolution and apparently, also in need of renal replacement therapy (RRT), the patient underwent renal biopsy for diagnosis and prognostic assessment. Histology showed a picture of acute interstitial nephritis, granulomatous characteristic of renal sarcoidosis, confirmed after review of medical records, which revealed previous hospitalization due to lung of the disease. Treatment with Prednisone 1mg/kg/day, the patient presented improvement of acute renal failure, no longer requiring RRT. Currently, the patient is maintained as an outpatient with stable renal function.

[Cutaneous sarcoidosis through a hospital series of 28 cases]

Sarcoidosis is a systemic disease, of unknown etiology, characterized by noncaseating epithelioid granulomas. It may affect many organs mainly lungs, lymph nodes and skin. The aim of our study is to evaluate retrospectively the epidemiological, clinical features and therapeutic particularities of cutaneous sarocidosis through a hospital series conducted in the dermatology department of Habib Thameur hospital. We have included 28 patients [23 females and 5 males] with a mean age of 45.5 years. The most frequent skin lesions were: papular sarcoidosis in 6 cases, nodular sarcoidosis in 8 cases and plaques in 6 cases. The other clinical types observed were: lupus pernio [2 cases], subcutaneous nodules [2 cases] and scar sarcoidosis [2 cases]. Three patients presented erythema nodosum. Tuberculin skin test was negative in 77% of cases. Lymph nodes were palpable in 4 patients. Investigative exams revealed intrathoracic involvement in 8 cases and anterior uveitis sequels in 2 cases. Cutaneous manifestations were treated by chloroquin in 13 cases, allopurinol in 3 cases and intralesional betamethasone injections in 1 case. Five patients, with systemic involvement, underwent a therapy with oral corticosteroids, Sarcoidosis is a rare affection in Tunisia that affects mainly females. Cutaneous manifestations incite physicians to carry out complete investigations and to uphold a regular follow-up in order to detect systemic involvement

Sarcoidosis cardíaca con compromiso pulmonar estadio IV / Cardiac sarcoidosis with stage IV pulmonary involvement

Se presenta el caso de un paciente de 26 años, con síndrome de falla cardíaca, síntomas respiratorios persistentes, fibrilación auricular y muerte súbita por taquicardia ventricular no sostenida; con evidencia ecocardiográfica de insuficiencia mitral severa y confirmación histológica e imaginológica pulmonar de sarcoidosis

Sarcoidosis / Sarcoidosis

La sarcoidosis es una afección sistémica, de naturaleza granulomatosa y de etiología desconocida, en cuya patogenia intervienen, probablemente mecanismos inmunológicos. La afección puede comprometer prácticamente todos los órganos y sistemas siendo los mas frecuentemente afectados el aparato respiratorio, la piel y el ojo. Las formas agudas regresan generalmente en forma espontánea; las crónicas se tratan con corticoides y/o inmunosupresores.

Sarcoidosis: a delayed or missed diagnosis in children

Sarcoidosis is an idiopathic granulomatous disease, most commonly affecting young adults and presenting with bilateral hilar lymphadenopathy and pulmonary infiltrates [1]. In children it is relatively rare and its clinical spectrum varies according to the age of onset [1-2] It is more common during adolescence and usually presents with clinical features similar to the adult type. In children under the age of 4 years it is rare and has a different presentation. Clinical features are characterized by a triad of rash, uveitis, and arthritis.[3] Sarcoidosis has a worldwide distribution, but is more frequently reported from developed countries.[4,5. To our knowledge, there are no reports of childhood Sarcoidosis from Saudi Arabia. We describe the clinical and laboratory features, treatment and outcome of 8 children with sarcoidosis seen at our hospital

[ sarcoidosis]

Sarcoidosis is a multisystemic granulomatosis seen mainly in the young adult. This is a retrospective study which evaluated 35 cases of sarcoidosis confirmed histologically, during periods from 1985 to 1999. In this study, 88.57% of patients were female with a mean age of 47.5 years old. The main complaint was often a peripheral node lesion followed by an ocular lesion. This clinical study had a very polymorphic presentation. The endothoracic lesions were largely predominant [94.28%], followed by extra thoracic lesions such as cutaneous lesions [big nodules and erythema nodosum], ocular lesions [25.7%], represented by anterior uveitis [4 cases], posterior uveitis [2 cases] and one case of panuveitis. Lymphatic node lesions were present in 63% of cases. The articular involvement was mainly made of inflammatory arthralgia. The bone lesions seen in 5 cases were made of sarcoid dactylitis confirmed histologically in 3 cases. We observed one case of sarcoidosis associated to spondylarthropathy. Prednisone was indicated in 27 patients at a dose of 0.5 to 1mg/kg/day, leading to a good recovery in 58.33 of cases. The mean follow-up was 20 months. Sarcoidosis in Morocco seems to be rare. The association of sarcoidosis and spondylarthropathy was rarely described. Posterior uveitis was present in this series

Acute myeloid leukemia following regression of sarcoidosis: a case report

This report describes Acute Myeloid Leukemia [AML] occurring in a 46 years old woman previously diagnosed to have Sarcoidosis. There was no evidence of Sarcoidosis at the time of diagnosis of AML. Although the association is well recognized, a cause and effect relationship between the two diseases is not fully established. A brief review of the literature is presented. Case Reports: A 46 years old lady presented to the emergency room with a history of high grade fever, exertional dyspnoea and generalized weakness for the past 4 weeks. She had undergone coronary artery bypass grafting [CABG] five years back. A year before the current presentation, she had presented with a history of fever, dry cough and anorexia. Examination had been unremarkable, except for an ESR of 32 mm/hr and bilateral hilar lymphadenopathy on chest X-ray. She was treated with standard anti-tuberculosis therapy [ATT] empirically. Subsequent to a lack of response to ATT and cultures for Acid Fast Bacilli remaining negative, Angiotensin Converting Enzyme [ACE] levels were found to be elevated to 59 IU/L [normal 8-52 IU/L]. A lymph node had also appeared in the left supraclavicular region by this time; excisional biopsy of which revealed non-caseating granuloma. ATT was discontinued and the patient was started on oral steroids. Within the next two months she became asymptomatic and the chest X-ray showed a complete regression of hilar Lymph nodes. During her current admission, she was found to be febrile, pale, icteric with hepatosplenomegaly, but no lymphadenopathy Examination of the cardiovascular, respiratory and the central nervous system were unremarkable. Her laboratory data revealed a haemoglobin of 6.3 g/dl, total leukocyte count of 121x 109/L with 88% blast cells, and a platelet count of 24x109/L. Bone marrow aspirate revealed Auerrod containing blast cells which constituted 90% of the total nucleated cells. 80% of the cells showed reactivity to Sudan Black. The patient was diagnosed to have AML. The chromosomal analysis revealed a 46 XX karyotype. Serum chemistries revealed a BUN of 7 mg/dl; creatinine 1mg/dl; Na 140 mEq/L; K 2mEq/L; total bilirubin 3.6 mg/dl; ALT 11 IU/L; alkaline phosphate 52 IU/L; LDH 5487 IU/L; and uric acid 7.2 mg/dl. The ACE levels were within normal limits. The chest Xray showed evidence of previous sternotomy and no lymphadenopathy. She was started on induction chemotherapy consisting of cytosine arabinoside 100 mg/m2 for 7 days and mitoxantrone 12 mg/m2 for three days. Hematological remission was documented on the 29th day of induction treatment. Bone marrow biopsy did not reveal a granuloma or fibrosis