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.

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.

Avaliação eletrofisiológica cardíaca em pacientes com sarcoidose / Cardiac electrophysiological assessment in patients with sarcoidosis / Evaluación electrofisiológica cardíaca en pacientes con sarcoidosis

FUNDAMENTO: Sarcoidose é uma doença granulomatosa multissistêmica de origem desconhecida que pode causar morte súbita. OBJETIVO: Avaliação eletrofisiológica de pacientes com sarcoidose com suspeita de comprometimento cardíaco. MÉTODOS: Foram estudados 22 pacientes com média de idade de 55,32 ±13,13 anos, com diagnóstico de sarcoidose e suspeita de comprometimento cardíaco. Foram submetidos à avaliação clínica, exames laboratoriais, eletrocardiograma, ecocardiograma, Holter de 24h, cintilografia com gálio ou tecnécio e estudo eletrofisiológico. Em casos selecionados foi realizada tomografia por emissão de pósitrons ou ressonância magnética. Os pacientes foram seguidos ambulatoriamente com consultas trimestrais. RESULTADOS: Comprometimento cardíaco foi comprovado em quatro (18,2 por cento) pacientes. Extrassístoles ventriculares com densidade > 100/24h foram documentadas no Holter de 24h em 12 (54,5 por cento) pacientes. O estudo eletrofisiológico revelou aumento do intervalo HV em sete (31,8 por cento) e ponto de Wenckebach aumentado em quatro (18,2 por cento) pacientes. Houve indução de fibrilação atrial em sete (31,8 por cento) e de taquicardia ventricular sustentada em um (4,5 por cento). Nos quatro pacientes com sarcoidose cardíaca confirmada, extrassístoles ventriculares, com densidade > 100/24h foram documentadas em todos, dois apresentavam intervalo HV prolongado e fibrilação atrial foi induzida em dois. Taquicardia ventricular sustentada não foi induzida em nenhum desses pacientes. Após período médio de acompanhamento de 20,9 ± 15,7 meses, um paciente com sarcoidose cardíaca apresentou morte súbita. CONCLUSÃO: Pacientes com sarcoidose e suspeita de envolvimento cardíaco apresentam alta prevalência de EVs e distúrbios do sistema de condução.

BACKGROUND: Sarcoidosis is a multisystem granulomatous disease of unknown origin that can cause sudden death. OBJECTIVE: Electrophysiological evaluation of patients with suspected sarcoidosis with cardiac involvement. METHODS: We studied 22 patients with mean age of 55.32 ± 13.13 years, diagnosed with sarcoidosis and suspected cardiac involvement. These patients underwent clinical evaluation, laboratory tests, electrocardiogram, echocardiogram, 24-hour Holter, technetium or gallium scintigraphy and electrophysiological study. In selected cases, we performed positron emission tomography or magnetic resonance imaging. Patients were followed up in the outpatient care service with quarterly visits. RESULTS: Cardiac involvement was confirmed in four (18.2 percent) patients. Ventricular extrasystoles with density > 100/24h were documented in 24-Holter monitoring in 12 (54.5 percent) patients. Electrophysiological studies revealed an increased HV interval in seven patients (31.8 percent) and increased Wenckebach point in four (18.2 percent) patients. There was induction of atrial fibrillation in seven patients (31.8 percent) and sustained ventricular tachycardia in one patient (4.5 percent). Four patients with confirmed cardiac sarcoidosis had documented ventricular extrasystoles with density > 100/24h. Out of these, two had prolonged HV interval and atrial fibrillation was induced in two of them. Sustained ventricular tachycardia was not induced in any of these patients. After mean follow-up period of 20.9 ± 15.7 months, one patient with cardiac sarcoidosis had sudden death. CONCLUSION: Patients with sarcoidosis and suspected cardiac involvement have a high prevalence of ventricular extrasystoles (VEs) and conduction system disorders.

FUNDAMENTOS: Sarcoidosis es una enfermedad granulomatosa multisistémica de origen desconocido que puede causar la muerte súbita. OBJETIVOS:Evaluación electrofisiológica de los pacientes con sarcoidosis con sospecha de afectación cardíaca. MÉTODOS:Se estudiaron a 22 pacientes con una media de edad de 55,32 ± 13,13 años, con diagnóstico de sarcoidosis y sospecha de afectación cardíaca.Se sometieron a evaluación clínica, exámenes de laboratorio, electrocardiograma, ecocardiograma, Holter de 24 h, centellografía con galio o tecnecio y estudio electrofisiológico. En los casos seleccionados se realizó tomografía por emisión de positrones o la resonancia magnética.Los pacientes fueron seguidos en ambulatorio, en consultas trimestrales. RESULTADOS:La afectación cardíaca se comprobó en cuatro (18,2 por ciento) pacientes.Extrasístoles ventriculares con densidad > 100/24 h se documentaron en el Holter de 24 h en 12 (54,5 por ciento) pacientes.Los estudios electrofisiológicos revelaron un aumento del intervalo HV en siete (31,8 por ciento) y punto de Wenckebach aumentado en cuatro (18,2 por ciento) pacientes. No hubo inducción de fibrilación auricular en siete (31,8 por ciento) y de taquicardia ventricular sostenida en uno (4,5 por ciento). En los cuatro pacientes con sarcoidosis cardiaca confirmada, extrasístoles ventriculares (EVs), con densidad > 100/24 h se documentaron en todos, dos presentaban prolongación del intervalo HV y fibrilación auricular se indujo en dos. La taquicardia ventricular sostenida no fue inducida en cualquiera de estos pacientes. Después de un periodo medio de seguimiento de 20,9 ± 15,7 meses, un paciente con sarcoidosis cardíaca tuvo muerte súbita. CONCLUSIÓN: Los pacientes con sarcoidosis y sospecha de afectación cardíaca tienen una alta prevalencia de EVs y trastornos del sistema de conducción.

Sarcoidosis: Presentación clínica y pronóstico / Sarcoidosis: Clinical presentation and prognosis

Se analizaron las características clínicas de 26 pacientes con cuadro clínico compatible y diagnóstico histopatológico de sarcoidosis, y se identificaron variables asociadas a mortalidad en el seguimiento. Se examinaron datos clínicos y de diversos estudios complementarios. El seguimiento se realizó mediante consulta médica y encuesta telefónica. La edad media fue 42.6 ± 12.7 años. El 53.8% eran mujeres. El 88.4% presentó afección pulmonar y el 30.7% tuvo manifestaciones extratorácicas. Predominó el estadio radiológico II (34.7%). La alteración espirométrica más frecuente fue la capacidad de difusión de monóxido de carbono (DLCO) < 80% del predicho (56.5%). Se constató hipertensión pulmonar en el 34.7%. Recibieron tratamiento corticoideo el 69.2% de los pacientes. El seguimiento se realizó en el 96.1% de los casos durante 98 ± 73 meses (rango: 3 a 228). Se constató una mortalidad del 23% (n = 6). Los factores asociados en forma significativa a mayor mortalidad fueron: gasometría arterial con menor presión parcial de oxígeno (41.5 mm Hg vs. 73.3 mm Hg; p = 0.041); mayor presión parcial de dióxido de carbono (59.5 mm Hg vs. 39.6 mm Hg; p = 0.0008); presencia de hipertensión pulmonar (83.3% vs. 16.6%; p = 0.001) y mayor presión pulmonar wedge (12.5 mm Hg vs. 9.5 mm Hg; p = 0.041). Hubo una tendencia a mayor mortalidad en pacientes con estadio radiológico III/IV (66% vs. 27%; p = 0.082) y menor DLCO (33.5% vs. 51.4%; p = 0.087). Las características clínicas y evolutivas encontradas en nuestra serie difirieron en algunos aspectos con las publicadas en la literatura internacional. Los factores asociados a mortalidad estuvieron relacionados a la gravedad de la enfermedad.

We analyzed clinical characteristics of 26 patients with suggesting clinical picture and histopatological diagnosis of sarcoidosis. We identified mortality-related variables in the follow-up. We examined clinical data and several complementary tests. Follow-up was performed by clinical consultation and telephonic interview. The patients mean age was 42.6 ± 12.7 years old, and 53.8% were female. Pulmonary affection was present in 88.4% of patients and extrapulmonary manifestation were seen in 30.7%. Radiological stage II was the most frequent (34.7%). The predominant spirometric abnormality was a low carbon monoxide diffusing capacity (DLCO) in 56.5% of cases. Pulmonary hypertension was found in 34.7% of cases. Steroid therapy was performed in 69.2%. The follow-up was completed in 96.1% of patients with a mean of 98 ± 73 months (range 3 to 228). The mortality rate was 23% (n = 6). The factors significantly associated with mortality were: blood arterial gases with lower partial oxygen pressure (41.5 mm Hg vs. 73.3 mm Hg; p = 0.041); higher partial carbon dioxide pressure (59.5 mm Hg vs. 39.6 mm Hg; p = 0.0008); presence of pulmonary hypertension (83.3% vs. 16.6%; p = 0.001) and higher pulmonary capillary wedge pressure (12.5 mm Hg vs. 9.5 mm Hg; p = 0.041). There was a tendency to higher mortality in patients with radiological stage III/IV (66% vs. 27%; p = 0.082) and lower DLCO (33.5% vs. 51.4%; p = 0.087). Clinical characteristics and long-term prognosis in our serie differed from others publications in international literature. Mortality-related factors were associated with severity of disease.

Síndrome de Lõfgren / Lõfgren’s Syndrome

A sarcoidose é uma doença crônica granulomatosa, de origem desconhecida, que pode envolver praticamente qualquer órgão, mas na qual a presença de artrite é incomum. Apresentamos um caso no qual a artrite foi o achado inicial, associada ao eritema nodoso, levando à investigação e ao diagnóstico da síndrome de Lõfgren. Nessa forma de apresentação da sarcoidose, além da artrite ou artralgias e do eritema nodoso, também deve estar presente a linfadenopatia hilar bilateral como critério diagnóstico. A síndrome de Lõfgren é chamada algumas vezes de sarcoidose aguda, estando geralmente associada com um bom prognóstico e resolução espontânea.

Sarcoidosis is a chronic granulomatous disease, of unknown origin, that may involve virtually any organ, but in which the presence of arthritis is uncommon. Here we present a case in which arthritis was the first finding, associated with erythema nodosun, leading to the investigation and diagnosis of Lõfgren Syndrome. In this form of sarcoidosis presentation, in addition to arthritis or arthralgia and erythema nodosun there must be bilateral hilar lymphadenopathy as a diagnostic criterion. Lõfgren’s syndrome is sometimes called acute sarcoidosis, usually being associated with good prognosis and spontaneous resolution.

Symptomatic muscle involvement in neurosarcoidosis: a clinicopathological study of 5 cases

We report on the clinical course and histopathologic muscle alterations of five patients diagnosed with neurosarcoidosis, who underwent biopsy due to their muscle manifestations. The five patients were females and only one was less than 40 years of age. Proximal muscle weakness was presented by all and only two patients complained of myalgia. Only normal values of serum muscle enzymes were detected. Electromyography revealed diverse findings such as normal, myopathic and neuropathic patterns. Granuloma was not present in one muscle biopsy. Two patients thoroughly recovered by taking only prednisone and one patient required a methotrexate addition for 3 months before becoming asymptomatic. The other two patients received azathioprine, one due to steroid side effects but without a satisfactory evolution, and the other to strengthen the prednisone régime, with excellent results

Doenças granulomatosas da laringe / Granulomatous diseases of the larynx

Realizamos uma revisäo sobre as principais doenças granulomatosas que podem acometer a laringe, devido à grande ocorrência dessas afecçöes no Brasil e em outros países de clima tropical e subtropical. Ressaltamos os aspectos histopatológicos para o seu diagnóstico, assim como o seu tratamento.

Sarcoidose da retina e nervo óptico: relato de um caso / Ocular sarcoidosis with optic nerve and retina involvement: report of a case

Os autores descrevem um caso de sarcaidose ocular com comprometimento da retina e nervo óptico, associado à parotidite bilateral (doença de Heerfordt)

Concentration of soluble interleukin-2 receptor in bronchoalveolar lavage and serum in patients with sarcoidosis and idiopathic pulmonary fibrosis

T-Iymphocyte activation in sarcoidosis and idiopathic pulmonary fibrosis [IPF] was evaluated using an enzyme linked immunosorbent assay for soluble interleukin-2 receptor [IL-2R] in serum samples and bronchoalveolar lavage [BAL] Fluids. The study included 12 patients with active untreated sarcoidosis, 8 patient with IPF and 14 subjects as normal control. The mean serum levels were significantly higher in sarcoidosis and IPF [2562.5 +/- 1349.9 and 541.6 +/- 197.6 u/ml] compared to normal control subjects [192.3 +/- 77.3 U/ml] and being higher in sarcoidosis than IPF [p < 0.001]. In contrast, in BAL fluids the IL-2R levels were low among the three studied groups [0.04 +/- 0.05, 0.035 +/- 0.03 and 0.030 +/- 0.02 micro/mg total protein] respectively]. It is concluded that serum IL-2R may reflect disease activity in patients with active sarcoidosis

Reversibilidade do envolvimento do VIII par craniano na sarcoidose / Reversibility of involvement of the VIII cranian in sarcoidosis

O espectro do envolvimento sistêmico da sarcoidose é bastante amplo, conferindo à doença um grande polimorfismo clínico. Dentro desta diversidade destacam-se algumas formas peculiares de apresentaçäo. Descreve-se uma forma neuro-ocular onde o acometimento do VIII par associa-se à lesäo de outros pares cranianos. Pöe-se em evidência o caráter reversível da disacusia neurossensorial, comparando-se a evoluçäo com casos da literatura

Miopatía sarcoidea / Myopathic sarcoidosis

La sarcoidosis considerada como una enfermedad granulomatosa sistémica de etiología desconocida es una entidad de variable prevalencia aunque aparentemente escasa en nuestro medio. En la misma el compromiso muscular es frecuente en la forma asintomática ya que se lo encuentra en el 50 a 80% de los casos demostrables anatomo-patológicamente en el material obtenido en biopsias, mientras que las formas clínicas no manifiestan una forma usual de presentación. Se analizan en una paciente de 78 años las manifestaciones musculares y las alteraciones observadas en las respuestas inmunológicas que, junto a otros métodos complementarios y a la anatomía patológica, permitieron arribar al diagnóstico correcto de sarcoidosis. Se discuten los caracteres de estos hallazgos sentándose las correspondientes consideraciones terapéuticas