What is New at Ophthalmic Epidemiology

Purpose: To compare the differences in vision and health-related quality of life (HRQOL) of individuals with ocular and non-ocular sarcoidosis; and to examine the impact of specific demographic and clinical factors on the noted differences.

Methods: A cross-sectional study using non-randomized prospective cohort was conducted at the National Eye Institute (protocol number: 06-EI-0239, NCT00379275) from August 31, 2006 until November 15, 2007. Each participant completed vision and HRQOL questionnaires, the Sarcoidosis Health Questionnaire (SHQ) and the National Eye Institute Visual Function Questionnaire (NEI-VFQ), along with a demographic/environmental exposure survey. Clinical data were collected through an ophthalmic exam as part of the research protocol.

Results: The study enrolled 75 biopsy-proven and 20 clinically presumed sarcoidosis participants which were divided into two cohorts, ocular (N?=?60) and non-ocular groups (N?=?35). The ocular group had significantly lower (P?sarcoidosis who had an annual household income of < $50,000 (P?sarcoidosis whose visual acuity was 20/100 or worse had significantly lower total NEI-VFQ scores (P?=?0.03).

Conclusions: Ocular involvement impacts both overall and vision-related quality of life among sarcoidosis patients. Lower economic status appears to have a significant impact on the quality of life of sarcoidosis patients. Assessment of visual function and general health status provide pertinent information for individuals with sarcoidosis and should be included in their care to assess burden of their disease on their quality of life.     

Sarcoidosis in residents of northern Israel of Arabic and Jewish origin: a comparative study

BACKGROUND: This study describes the features of sarcoidosis among Arab patients and compares it to Jewish patients residing in northern Israel. METHODS: All new cases of biopsy-confirmed sarcoidosis diagnosed between 1980 and 1996 in northern Israel were divided into two groups according to their ethnic origin: Jewish (n = 72) and Arabic (n = 48). Disease parameters were recorded and compared. RESULTS: Arabs and Jews had similar incidence rates that increased from 0.2/10(5) in 1980 to 2/10(5) per year in 1996. The peak incidence was in the sixth and seventh decades and the female/male ratio was 2 and 1.4 for Arabic and Jewish patients, respectively. Jewish patients had higher proportion stage II-IV pulmonary disease (78% vs. 51.2%) while their Arabic counterparts had higher proportion of stage I disease (70.8% vs. 41.7%). The proportion of extra-thoracic organ involvement was similar. Different disease phenotype indicated differed diagnostic procedures; higher proportion of mediastinoscopy for stage I disease among Arabic patients (47.9% vs. 20.8%, P = 0.015) and trans-bronchial biopsy for stage II-IV pulmonary disease among Jewish patients (25% vs. 8.35%, P = 0.05). Corticosteroids were used in a non-significantly higher proportion of Jewish patients (56.9% vs. 43.8%, P > 0.05). Of six sarcoidosis-related deaths (5%), five occurred in Jewish patients. CONCLUSIONS: This study has documented different forms of presentation, clinical manifestation, severity and prognosis of sarcoidosis present among patients of Arabic and Jewish origin residing in the area of northern Israel.     

Echocardiographic evidence of posteromedial hypokinesis of the left ventricle in relation to mitral regurgitation in cardiac sarcoidosis

We describe herein the case of a 49-year-old female patient with pulmonary sarcoidosis (stage II) with cardiac manifestation. This consisted of systolic dysfunction without dilatation of the left ventricle and severe mitral insufficiency, possibly due to thinning of the posteromedial left ventricular free wall, based on our echocardiographic observations.     

^18F-脱氧葡萄糖（FDG）符合线路显像对胸部结节病的诊断价值

目的评价^18F-脱氧葡萄糖（FDG）符合线路显像对胸部结节病的诊断和疗效监测的价值。方法对37例疑似胸部结节病患者行^18F-FDG符合线路显像。以目测法判断纵隔和肺门淋巴结放射性摄取程度,明显超过周围肺组织本底水平者为阳性。结果30例患者最终经病理等确诊为胸部结节病,另外7例为其他肺部疾病。^18F-FDG符合线路显像诊断胸部结节病的灵敏度为96.67％（29／30）、特异性为85.71％（6／7）、准确性为94.59％（35／37）、阳性预测值为96.67％（29／30）、阴性预测值为85.71％（6／7）、阳性似然比为6.76、阴性似然比为0.04。结论^18F-FDG符合线路显像对胸部结节病的诊断和疗效监测具有重要的临床应用价值。     

Fragmented QRS Complexes on 12‐Lead ECG: A Marker of Cardiac Sarcoidosis as Detected by Gadolinium Cardiac Magnetic Resonance Imaging

Background: Fragmented QRS complexes (fQRS) on a 12‐lead ECG are a marker of myocardial scar in patients with coronary artery disease. Cardiac sarcoidosis is also associated with myocardial granuloma formation and scarring. We evaluated the significance of fQRS on a 12‐lead ECG compared to Gadolinium‐delayed enhancement images (GDE) in cardiac magnetic resonance imaging (CMR). Method and results: The ECGs of patients (n = 17, mean age: 52 ± 11 years, male: 53%) with established diagnosis of sarcoidosis who underwent a CMR for evaluation of cardiac involvement were studied. ECG abnormalities included bundle branch block, Q wave, and fQRS. fQRS, Q wave, and bundle branch block were present in 9 (53%), 1 (6%), and 4 (24%) patients, respectively. The sensitivity and specificity of fQRS for detecting abnormal GDE were 100% and 80%, respectively. Sensitivity and specificity of Q waves were 11% and 100%, respectively. Conclusions: fQRS on a 12‐lead ECG in patients with suspected cardiac sarcoidosis are associated with cardiac involvement as detected by GDE on CMR.     

Antimycobacterial immune responses in patients with pulmonary sarcoidosis

Introduction: Sarcoidosis is a multisystem granulomatous disease of unknown origin. Pathogenetic involvement of Mycobacterium tuberculosis has frequently been discussed in the aetiology of sarcoidosis; however, studies still remain contradictory. Objective: We addressed the question of mycobacterial involvement in the pathogenesis of sarcoidosis by analysing cellular immune responses to mycobacterial antigens. Methods: We examined the interferon (IFN)‐γ production by enzyme‐linked immunospot in response to purified protein derivate (PPD) mycobacterial‐specific antigen early secretory antigenic target (ESAT)‐6 and culture filtrate protein (CFP)‐10 by peripheral blood mononuclear cells (PBMCs) and bronchoalveolar‐lavage mononuclear cells (BALMCs) of patients with pulmonary sarcoidosis, smear‐negative tuberculosis and controls. Results: Release of IFN‐γ in response to ex vivo contact with PPD, ESAT‐6 or CFP‐10 by BALMC and PBMC were comparable among patients with sarcoidosis and controls (PBMC P = 0.2326; BALMC P = 0.1767) and were less frequently observed in both groups compared to patients with tuberculosis (BALMC P < 0.05; PBMC P < 0.0001). Within PBMC, the immunophenotype of sarcoidosis patients differed from that of patients with tuberculosis, as well as from that of controls, while within BALMC it resembled that of patients with tuberculosis. Conclusion: In contrast to patients with tuberculosis, the frequency of mycobacteria‐specific local and systemic immune responses is not elevated in patients with sarcoidosis when compared to controls. The immunophenotype represents the local resemblance of the granulomatous reaction underlying tuberculosis and sarcoidosis while showing systemical difference. These observations do not support a role of an infection with M. tuberculosis in the pathogenesis of sarcoidosis. Please cite this paper as: Hörster R, Kirsten D, Gaede KI, Jafari C, Strassburg A, Greinert U, Kalsdorf B, Ernst M and Lange C. Antimycobacterial immune responses in patients with pulmonary sarcoidosis. The Clinical Respiratory Journal 2009; 3: 229–238.     

Sarcoidal granulomas in a cosmetic tattoo in association with pulmonary sarcoidosis

Sarcoidosis is a granulomatous disease of unknown etiology, which may affect multiple organ systems. The skin is involved in up to one‐third of cases. The development of granulomatous lesions within tattoos is a well‐recognized phenomenon in patients with sarcoidosis. The characteristic histology shows non‐caseating granulomas; however similar findings may be seen in foreign body granulomas. We report on a patient, who developed sarcoidal granulomas within the black pigment of his tattoos. This subsequently led to the diagnosis of pulmonary sarcoidosis.     

Lupus pernio with multiple bone cysts in the fingers

A 32-year-old Japanese man presented with a 3-year history of purple reddish, and painful swelling of his fingers along with indurated erythema on his nose and ears. He was diagnosed as having sarcoidosis 8 years prior because of uveitis and bilateral hilar lymphadenopathy. X-rays of the hands revealed multiple cystic lesions in the phalanges. Histological examination of the ear revealed epithelioid cell granulomas in the dermis. Oral prednisolone 20 mg/day improved his finger swelling and pain; however, his finger deformities and erythema remain unchanged. Bone involvement is sometimes seen in sarcoidosis and the hands are the most frequently affected areas. The frequency of bone involvement is higher in lupus pernio in comparison with other types of skin sarcoidosis. Systemic corticosteroids could be the first choice of treatment to relieve the symptoms.     

Focal left atrial tachycardias not associated with prior catheter ablation for atrial fibrillation: clinical and electrophysiological characteristics

Background: The clinical features and electrophysiological characteristics of patients with focal left atrial tachycardias (LATs) are not well characterized. This study reports the experience of a single center in catheter mapping and radiofrequency ablation of focal LAT not associated with prior atrial fibrillation (AF) ablation, including in cardiac sarcoidosis and transplant patients. Methods: Patients with focal LAT without a history of AF ablation were included in this retrospective analysis. Results: A total of 24 focal LATs were documented in 20 patients. Two patients were subsequently diagnosed with cardiac sarcoidosis. Two patients were status post a thoracic transplant. The mean initial cycle length of the focal LATs was 347.4 ± 96.2 ms (range 190–510 ms). Patients with a pulmonary vein (PV) ostium focus (n = 6) demonstrated a shorter cycle length than patients with other LA foci (259.2 ± 56.4 ms vs 371.9 ± 91.1 ms, P = 0.02), as well as a trend for a history of AF (67% vs 21%, P = NS). Catheter ablation was immediately successful for 19 of 22 focal LATs. Conclusions: Focal LATs not associated with prior AF ablation can originate in a variety of LA locations and clinical settings. Focal LAT arising in the PV ostia is associated with a history of AF and demonstrates a faster tachycardia rate. We also report focal LAT in cardiac sarcoidosis patients and in the donor heart of an orthotopic heart transplant recipient. Radiofrequency ablation is a successful treatment for focal LAT not associated with prior ablation, including those refractory to medical therapy. (PACE 2012; 35:17–27)     

胸内结节病的临床特征及治疗分析

目的分析胸内结节病的临床特点、诊治方法及疗效。方法收集55例胸内结节病患者的临床资料,对患者的临床特征及治疗过程和效果进行回顾性分析。结果 55例患者中女性多于男性,发病高峰在51～60岁。临床表现非特异性,以咳嗽最常见,部分患者无症状。39例患者接受糖皮质激素治疗,4例在激素减量过程中出现病情反复,给予激素加量并行缓慢减量后好转;1例停药4年后复发,重新给予激素治疗,随访6年至今未复发。接受激素治疗的患者中,常见的不良反应依次为肥胖、消化性溃疡和骨质疏松;未发生威胁生命的并发症。结论胸内结节病主要临床表现为咳嗽;患者长期接受激素治疗,应严密监测不良反应。