Cardiopulmonary exercise testing variables as predictors of long-term outcome in thoracic sarcoidosis

Cardiopulmonary exercise testing (CPET) plays an important role in the assessment of functional capacity in patients with interstitial lung disease. The aim of this study was to identify CPET measures that might be helpful in predicting the vital capacity and diffusion capacity outcomes of patients with thoracic sarcoidosis. A longitudinal study was conducted on 42 nonsmoking patients with thoracic sarcoidosis (median age = 46.5 years, 22 females). At the first evaluation, spirometry, the measurement of single-breath carbon monoxide diffusing capacity (D LCOsb) and CPET were performed. Five years later, the patients underwent a second evaluation consisting of spirometry and D LCOsb measurement. After 5 years, forced vital capacity (FVC) percent and D LCOsb percent had decreased significantly [95.5 (82-105) vs 87.5 (58-103) and 93.5 (79-103) vs 84.5 (44-102), respectively; P < 0.0001 for both]. In CPET, the peak oxygen uptake, maximum respiratory rate, breathing reserve, alveolar-arterial oxygen pressure gradient at peak exercise (P(A-a)O2), and Δ SpO2 values showed a strong correlation with the relative differences for FVC percent and D LCOsb percent (P < 0.0001 for all). P(A-a)O2 ≥22 mmHg and breathing reserve ≤40 percent were identified as significant independent variables for the decline in pulmonary function. Patients with thoracic sarcoidosis showed a significant reduction in FVC percent and D LCOsb percent after 5 years of follow-up. These data show that the outcome measures of CPET are predictors of the decline of pulmonary function.

Sarcoidose: associação entre aspectos clínicos, radiológicos e funcionais: experiência do Pavilhão Pereira Filho (RS) / Sarcoidosis: clinical, radiological and functional association: the experience in the Pavilhão Pereira Filho, RS

Introdução: Sarcoidose é uma doença granulomatosa multissistêmica de causa desconhecida, caracterizada pela presença de granulomas de células epitelioides, não caseosos, com apresentação clínica variando de pacientes assintomáticos, até acometimento em múltiplos orgãos e sistemas, sendo o pulmão acometido em até 90% dos casos. O objetivo deste estudo foi através de uma série de casos de sarcoidose descrever a associação entre aspectos clínicos, radiológicos e de função pulmonar. Métodos: Estudo de 103 casos com sarcoidose, atendidos em centro de referência no sul do país, entre 1990 e 2003. Resultados: Não houve diferença de frequência entre homens e mulheres (42% e 58%, respectivamente). A média de idade foi de 41,8 ± 14,1 anos. Houve predomínio em caucasianos (84%). Dados clínicos: 86% eram sintomáticos; dentre eles, 77% tinham sintomas torácicos. Os mais prevalentes foram: tosse (48%), dispneia (41%) e dor torácica (18%). Manifestações extrapulmonares foram encontrados em 80% dos casos. Sintomas sistêmicos, como emagrecimento, febre e astenia, foram encontrados em 35%, 23% e 23%, respectivamente. A espirometria foi realizada em 83 (82%) pacientes, com resultados anormais em 45%. O tipo radiológico predominante foi II (49%), seguido do tipo I (32%), tipo III (18%) e tipo 0 (1%). Realizaram radiograma de tórax e espirometria 73 pacientes, evidenciando-se associação entre a presença de distúrbio ventilatório e os tipos radiológicos I, II, III (p = 0,036). Conclusão: Nesta série de casos houve associação entre diferentes tipos radiológicos (I, II, III) e a presença de alteração funcional pulmonar. Esses achados radiográficos e funcionais não foram significativamente relacionados com os sintomas clínicos

Introduction: Sarcoidosis is a multisystem granulomatous disease of unknown cause, characterized by the presence of noncaseating epithelioid cell granulomas, with clinical presentation ranging from asymptomatic patients to involvement in multiple organs and systems, the lung being affected in up to 90% of cases. The aim of this study was to describe the association between clinical, radiological and pulmonary function aspects based on a series of sarcoidosis cases. Methods: A study of 103 cases of sarcoidosis, seen in a referral center in south Brazil between 1990 and 2003. Results: There was no difference in frequency between men and women (42% and 58%, respectively). Mean age was 41.8 ± 14.1 years. Caucasians predominated (84%). Clinical data: 86% were symptomatic, and among these, 77% had chest symptoms, the most prevalent of which were cough (48%), dyspnea (41%), and chest pain (18%). Extrapulmonary manifestations were found in 80% of cases. Systemic symptoms such as weight loss, fever, and asthenia were found in 35%, 23% and 23%, respectively. Spirometry was performed in 83 (82%) patients, with abnormal results in 45%. The predominant radiological type was II (49%), followed by type I (32%), type III (18%) and type 0 (1%). Spirometry and chest X-rays were performed in 73 patients, showing an association between the presence of respiratory disorder and radiological types I, II, and III (p = 0.036). Conclusion: In this series of cases there was an association between different radiological types (I, II, III) and presence of altered pulmonary function. These functional and radiographic findings were not significantly associated with clinical symptoms

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.

Enfermedad intersticial difusa de pulmón-neumonías intersticiales idiopáticas / Diffuse interstitial lung disease and idiopathic interstitial pneumonias

The author presents an approach to the understanding, diagnosis and management of diffuse interstitial lung disease and the idiopathic interstitial pneumonia, prevailing and evolving hypothese about its pathogenesis and implications for therapy...