Incidence Of Sarcoidosis Research Articles

Cardiac sarcoidosis - a possible cause of death. Case report

The incidence of cardiac sarcoidosis is more then 5% from the patients with chronic sarcoidosis and the survival is between 2 and 5 years. The diagnosis must be precocious and the treatment aggressive for increasing life expectancy. We describe the case of a 72 years old male with pulmonary and lymph node sarcoidosis which required surgical intervention. The histological examination revealed non caseous granulomatous inflammation in the mediastinal nodes and epithelioid cardiac granulomas without myocyte necrosis. Six month later the patient developed progressive heart failure and after one year the cardiac examination revealed multiple cardiac lesions. The diagnosis of cardiac sarcoidosis is often difficult to confirm when the cardiac dysfunction is the only sign because of the lack of specific diagnosis tests. A diagnosis guide that includes histological and clinic criteria. The diagnosis must be sustained by invasive and non-invasive methods. Cardiac sarcoidosis remains a challenge by the unforeseeable evolution to death. Sudden death is common in cardiac sarcoidosis, often noiseless clinically.

Late gadolinium enhancement in cardiac sarcoidosis predicts ICD implantation and appropriate discharge

Methods 96 consecutive patients with sarcoidosis were referred to our centre for CMR between 2002 and 2007 (46 M, 50 F; mean age 57 years). Outcomes in two cohorts distinguished by evidence of LGE were retrospectively assessed (mean follow-up 3.5 years). Left ventricular (LV) volumes, ejection fraction (EF) and mass were evaluated using semi-automated analysis software and indexed to body surface area. Continuous variables were reported as mean ± standard deviation; groups (based on the presence or absence of LGE) were compared using a t-test or Fisher's exact test. Results LGE was observed in 31 out of 96 (32%) patients. No significant difference was seen in all-cause death or hospitalisation for a cardiovascular event between the LGE+ and LGEcohort. The LGE+ cohort had a significantly greater rate of ICD implantation (7 vs 1, p < 0.0005) and appropriate ICD discharge (4 vs 0, p < 0.005). Notably, only four patients had right ventricular LGE+; all required defibrillator (ICD) implantation and sustained appropriate ICD discharge. There was no difference in other secondary outcomes such as pre-ICD implantation sustained ventricular tachycardia, pacemaker implantation rate or escalation of heart failure therapy. Amongst LGE+ individuals, there was a significant reduction in biventricular EF (LVEF 50 ± 13.0% vs. 64 ± 10%, p < 0.05; RVEF 49 ± 13 vs. 58 ± 9%, p < 0.05). RVEDV was also significantly greater (173 ± 14 vs.137 ± 5 mls, p < 0.0065), although not LVEDV (105 ± 5.9 vs. 132 ± 7.9 mls, p < 0.0066). The distribution of LGE did not appear to correlate with outcome. The two groups did not differ in the incidence of biopsy-proven sarcoidosis (48/96 cases), pre-referral cardiac symptoms or duration of follow-up. Within this cohort, only five patients had STIR imaging clearly suggestive of active cardiac sarcoidosis.

Prevalence of sarcoidosis in Switzerland is associated with environmental factors

The current study aimed to investigate incidence, prevalence and regional distribution of sarcoidosis in Switzerland with respect to environmental exposures. All sarcoidosis patients hospitalised between 2002 and 2005 were identified from the Swiss hospital statistics from the Swiss Federal Office for Statistics (Neuchâtel, Switzerland). Regional exposure characteristics included the regional distribution of different industrial sectors, agriculture and air quality. Co-inertia analysis, as well as a generalised linear model, was applied. The prevalence of "ever-in-life" diagnosed sarcoidosis, currently active sarcoidosis and sarcoidosis requiring hospitalisation was 121 (95% CI 93-149), 44 (95% CI 34-54) and 16 (95% CI 10-22) per 100,000 inhabitants, respectively. The mean annual incidence of sarcoidosis was 7 (95% CI 5-11) per 100,000 inhabitants. The regional workforce in the metal industry, water supply, air transport factories and the area of potato production, artificial meadows (grassland) and bread grains were positively associated with the frequency of sarcoidosis. The prevalence of sarcoidosis was higher than assumed based on former international estimates. Higher frequency was found in regions with metal industry and intense agriculture, especially production of potatoes, bread grains and artificial meadows.

COEXISTENCE OF THORACIC PLASMACYTOMA AND SARCOIDOSIS

INTRODUCTION: An extensive debate has existed over the association of sarcoidosis with cancer. In patients with certain malignancies like lymphomas, testicular cancer, lung cancer and melanomas an increased incidence of sarcoidosis has been classically reported (1). Only a single case report of coexistence of sarcoidosis with a gastric plasmacytoma has been described in the literature. The authors present, the second reported case of a plasmacytoma presenting concomitantly with sarcoidosis, and the first reported case occurring in a thoracic medullary plasmacytoma.

Clinical features of intraocular inflammation in Hokkaido, Japan

We aimed to investigate the clinical features of intraocular inflammation/uveitis in Hokkaido, Japan. We retrospectively reviewed the medical records of 1240 uveitis patients (511 men, 729 women) who visited Hokkaido University Hospital, Sapporo, Japan between 1994 and 2003. Mean age at disease onset was 41.7 +/- 17.8 years in men and 45.7 +/- 18.3 years in women. Anterior, posterior and combined anterior and posterior segment intraocular inflammation accounted for 45.1%, 4.7% and 50.2% of cases, respectively. Sarcoidosis was the most frequent aetiology (14.9%), followed by Vogt-Koyanagi-Harada (VKH) disease (9.7%) and Behçet's disease (6.7%). Aetiologies in 49.8% patients were unknown. In sarcoidosis, women represented 72.4% of patients, and disease onset occurred at 35.1 +/- 19.0 years of age in men and 50.3 +/- 16.5 years in women. In VKH disease, 54.2% of patients were women, and disease onset took place at 45.9 +/- 15.8 years in men and 46.4 +/- 14.1 years in women. In Behçet's disease, men accounted for 56.6% of patients, and disease onset occurred at 35.5 +/- 8.5 years in men and 44.5 +/- 11.5 years in women. Women were more prone to developing sarcoidosis compared with men. By contrast, men were more prone to developing Behçet's disease. The mean age at disease onset in both sarcoidosis and Behçet's disease was significantly lower in men than in women.

Epidemiological features of Turkish patients with sarcoidosis

Epidemiological characteristics of sarcoidosis differ according to geographical distribution. The aim of our study was to disclose epidemiological characteristics in our country. The data was collected from investigators, who sent information on newly-diagnosed patients via internet. In 2 years 198 female and 95 male patients were enrolled to the study (f/m:2.08). Mean age of patients was 44+/-13 years (17-90). Mean age of male patients was 38+/-12 while mean age of female patients was 48+/-13 (p<0.001). 73.4% of patients were nonsmokers (85.4% of females; 48.4% of males; (p<0.001)). About 50% of our 293 patients were housewives. Familial sarcoidosis was found in 3 patients' first degree relatives. Estimated annual incidence of sarcoidosis for Turkey was calculated as 4 per 100,000 person. According to our study, 2/3 of sarcoidosis patients were women; mean age of patients was 45 and the disease began 10 years later in female patients. 80% of patients were nonsmokers; negative relation between sarcoidosis and smoking was evident especially in women. Familial sarcoidosis frequency was lower compared to other studies in the literature. There was no occupational exposure history in our patients. Our incidence rate, is similar with the results of other European studies.

The incidence of undiagnosed cardiac sarcoidosis in explanted hearts following heart transplantation

Background: Sarcoidosis is a multi-system disorder of unknown aetiology resulting in non-caseating granulomas. The incidence in Australia has been estimated at 4–6 per 100,000 with cardiac involvement in 25% of cases. The aim of this study was to assess the incidence of sarcoidosis in our heart transplant population.

Ocular Sarcoidosis in a Korean Population

The aim of current study was to evaluate the incidence and characteristics of ocular sarcoidosis in a Korean population. We conducted a retrospective study of 104 consecutive patients with biopsy-proven sarcoidosis seen at Asan Medical Center in Seoul, Korea, from 1993 to 2007. Medical records, photographs, and fluorescein angiograms were reviewed. Of 104 patients, 22 (21%) had intraocular involvement with female predominance (86%, M:F=3:19). Of the 39 eyes with ocular involvement, 16 (41%) eyes had isolated anterior uveitis, 12 (31%) eyes had intermediate uveitis, 6 eyes (15%) had panuveitis with retinal vasculitis, and 5 (13%) eyes had panuveitis with punched multifocal choroiditis. Mean duration of ophthalmologic follow-up was 62 months. All ocular inflammation was well managed with topical steroid and/or systemic steroid with relatively good final visual outcomes. Ocular complications such as cataract (12 eyes, 30%), glaucoma (6 eyes, 15%), vitreous opacity (1 eye, 3%), cystoid macular edema (3 eyes, 7%), neovascularization (2 eye, 5%), and epiretinal membrane (4 eye, 10%) were related to ocular sarcoidosis. In Korea, where sarcoidosis is very rare, our study indicates relatively low ocular and predominantly non posterior segment involvement with relatively good visual prognosis.

PULMONARY GRANULOMATOUS DENSITY IN SARCOIDOSIS-RACIAL DIFFERENCES

PURPOSE: African-American race has been associated with a higher disease incidence of Sarcoidosis, more severe disease at diagnosis, more extrathoracic manifestations, and chronic course. Our hypothesis is that the lung granuloma counts are higher in African-Americans compared to Caucasians.

Airway involvement and obstruction from granulomas in African–American patients with sarcoidosis

Sarcoidosis is a global disorder whose breadth of organ involvement can often be underappreciated. Head and neck manifestations include involvement of the skin, salivary glands, sinonasal cavity, and larynx. Of cases of upper airway sarcoidosis, laryngeal sarcoidosis and airway compromise portend a greater risk of fatal outcomes. People representing all racial groups have been diagnosed with sarcoidosis. Although many studies have evaluated incidence and manifestations of sarcoidosis in multiple ethnicities, few studies have explored racial predilection for laryngeal involvement. However, assertions that disease severity and poor outcome may be tied to the African diaspora as well as related socio-economic and cultural realities have been recognized. We present our case series of six African-American patients diagnosed with sarcoidosis and presented with complaints of voice change and increased shortness of breath. Four of them required expeditious, surgical management of the airway. Two had limited supraglottic involvement and have avoided tracheotomy with aggressive and timely pharmacotherapeutic intervention and close clinical surveillance. Early recognition of laryngeal manifestations of sarcoidosis and airway compromise is essential to provide patients with conservative management without the need for aggressive surgical intervention.

Sarcoidosis in Australia

The incidence of sarcoidosis in Australia is unknown. The clinical features, diagnostic strategy and treatment of sarcoidosis in Australia have been poorly documented. We analysed the medical records of 122 patients with sarcoidosis presenting to a respiratory service, between 1995 and 2005, which serves a regional southeastern Australian population of approximately 200,000. The incidence of sarcoidosis from 2000 to 2005 remained static and ranged from 4.4 to 6.3 patients per 100,000 population. The data showed that 55% were women and 28% were current smokers. Systems involved included lung parenchyma (66%), thoracic adenopathy (58%), skin (22%), ocular (18%), joint (11%), gastrointestinal tract (5%), central nervous system (3%) and hypercalcaemia (3%). Fifty-one per cent of patients had an increased serum angiotensin-converting enzyme level. The diagnosis was secured based on histological confirmation in 69%. Forty-three per cent of the patients were treated with oral corticosteroids and 10% with inhaled steroids. Sarcoidosis in Australia is a multi-system disease of unknown aetiology. This is the first reported incidence of sarcoidosis in Australia. The incidence is similar to another US-based epidemiological study of a predominately white population. The development of a larger multicentre database would assist in the identification, clinical description and treatment of sarcoidosis.

World Trade Center “Sarcoid-Like” Granulomatous Pulmonary Disease in New York City Fire Department Rescue Workers

Previous reports suggest that sarcoidosis occurs with abnormally high frequency in firefighters. We sought to determine whether exposure to World Trade Center (WTC) "dust" during the collapse and rescue/recovery effort increased the incidence of sarcoidosis or "sarcoid-like" granulomatous pulmonary disease (SLGPD). During the 5 years after the WTC disaster, enrollees in the Fire Department of New York (FDNY) WTC monitoring and treatment programs who had chest radiograph findings suggestive of sarcoidosis underwent evaluation, including the following: chest CT imaging, pulmonary function, provocative challenge, and biopsy. Annual incidence rates were compared to the 15 years before the WTC disaster. After WTC dust exposure, pathologic evidence consistent with new-onset sarcoidosis was found in 26 patients: all 26 patients had intrathoracic adenopathy, and 6 patients (23%) had extrathoracic disease. Thirteen patients were identified during the first year after WTC dust exposure (incidence rate, 86/100,000), and 13 patients were identified during the next 4 years (average annual incidence rate, 22/100,000; as compared to 15/100,000 during the 15 years before the WTC disaster). Eighteen of 26 patients (69%) had findings consistent with asthma. Eight of 21 patients (38%) agreeing to challenge testing had airway hyperreactivity (AHR), findings not seen in FDNY sarcoidosis patients before the WTC disaster. After the WTC disaster, the incidence of sarcoidosis or SLGPD was increased among FDNY rescue workers. This new information about the early onset of WTC-SLGPD and its association with asthma/AHR has important public health consequences for disease prevention, early detection, and treatment following environmental/occupational exposures.

Sarcoidosis in Singapore: Epidemiology, clinical presentation and ethnic differences

The aim of this study is to better understand the epidemiological and clinical features of patients with sarcoidosis in Singapore and to ascertain if ethnic differences exist. A review of hospital medical records from June 1998 to May 2004 to identify patients with sarcoidosis. There were 59 patients with sarcoidosis identified (19 Chinese, 29 Asian Indian and 11 Malay). The estimated annual incidence of sarcoidosis in Singapore was 0.56 per 100,000. There was a significant difference between the observed and expected disease frequency in Chinese (32.2% vs 78.7%) and Indians (49.2% vs 6.0%, P < 0.005). A bimodal distribution of age at diagnosis was seen with peaks in the 30-39 years and 50-59 years age groups; 38.9% of cases were over the age of 50. Chinese patients were more likely to be asymptomatic (57.9%, P = 0.015) and less likely to have impaired spirometry (P = 0.013). Pulmonary sarcoid presented largely as stage 0 or stage 1 disease (74.4%). Overall mean spirometry was unimpaired and prognosis was good with 79.2% showing no radiological deterioration. There were no significant differences in organ involvement or treatment between ethnic groups. Sarcoidosis in Singapore is rare and the incidence differs between ethnic groups. Chinese appear to have a lower incidence and a less symptomatic presentation; Indians have a higher incidence and poorer clinical course.

Genetics of Sarcoidosis

Sarcoidosis likely results from an interplay of environmental and genetic factors. Despite a recent large multicenter study, A Case-Control Etiologic Study of Sarcoidosis (ACCESS), no single causative environmental agent has been identified. Family clustering and differences in racial incidence of sarcoidosis support an inherited susceptibility to sarcoidosis. Siblings of patients with sarcoidosis have about a fivefold increased risk of developing sarcoidosis. Certain human leukocyte antigen (HLA) alleles have been consistently associated with sarcoidosis susceptibility. Furthermore, HLA-DRB1*0301/DQB1*0201 has been associated with good prognosis in Löfgren's syndrome. Many candidate genes studied based on their potential function in immunopathogenesis have been evaluated in case-control studies, but few have been consistently associated with sarcoidosis across populations. Two genome scans have been reported in sarcoidosis. One in African Americans reporting linkage to chromosome 5 and the other in German families reporting linkage to chromosome 6. Follow-up studies on chromosome 6 identified the BTNL2 gene, a B7 family costimulatory molecule to be associated with sarcoidosis. Advances in genotyping and statistical analysis are helping to elucidate the genetics of sarcoidosis.

Sarkoidoza ślinianki przyusznej

Sarcoidosis is a granulomatous multisystemic disease of unknown origin that can occur in any organ of the body and commonly affects young adults. Pulmonary sarcoidosis is by far the most common manifestation of the disease but occasionally it presents with involvement of the lymph nodes, skin, eyes, liver, spleen, heart, bones, joints, nervous system and the oral organs. The diagnosis of sarcoidosis is complex and includes the presence of noncaseating granuloma in involved tissues, changes in chest x-ray and many other tests. The aim of the study was to present the rare case of parotid sarcoidosis which was the origin of discussion of diagnostics and treatment of sarcoidosis in ENT field. The authors present a review of updated opinions on pathogenesis, diagnostics and treatment of sarcoidosis, completed with own experience concerning a case of extrapulmonary sarcoidosis that manifested with isolated parotid gland enlargement. 1. The incidence of head and neck sarcoidosis is rare, although this etiology should be taken into account in ENT diagnostics. 2. The lesions can occur in many organs, most common manifestation is: lungs, peripherial lymph nodes, skin, eyes, articulations, liver and spleen. 3. Course of sarcoidosis is not characteristic, chest x-ray, biopsy and the analysis of clinical signs are required to settle the diagnosis. 4. In cases of isolated salivary gland sarcoidosis the preoperative diagnosis is difficult, fine-needle biopsy and Doppler ultrasonography can be helpful. 5. The possibility of exacerbations and recidivations makes the long follow-up necessary.

Incidence of sarcoidosis in head and neck cancer

Sarcoidosis and sarcoid-like reactions have been reported to be associated with malignancies, particularly testicular cancer and lymphoma. They occur either synchronously or metachronously, and may also occur after chemotherapy. We know of only two other case reports of granulomatous reactions in head and neck cancer. The present case highlights the association of sarcoidosis with head and neck cancer, and the importance of considering the diagnosis in patients who may have metastatic disease.

Sarcoidosis after treatment with interferon-α: A case series and review of the literature

Recombinant interferon-alpha (rINF-alpha) is an immunomodulator used in the treatment of various conditions, including viral infections and malignancies. The use of rINF-alpha has been associated with the development of sarcoidosis in recent case reports. In this series, we report the incidence of sarcoidosis in recipients of rINF-alpha for hepatitis C viral (HCV) infection at our institution. We also review the 57 additional cases of sarcoidosis associated with rINF-alpha described in the literature, including clinical presentation, radiographic findings, management, and outcomes, and discuss the potential mechanisms by which rINF-alpha may lead to the development of sarcoidosis.

Scar sarcoidosis in childhood: case report and review of the literature

Sarcoidosis is a rare disease among children, manifesting differently in children below and above 4-5 years of age. Although the exact incidence and prevalence of childhood sarcoidosis is not known, the cutaneous involvement is frequent in both children and adults. Infiltration of old cutaneous scars with sarcoid granuloma in the active phase of disease, known as scar sarcoidosis is one of the uncommon cutaneous manifestations of sarcoidosis. We report a case of scar sarcoidosis in an 11-year-old child along with a brief review of the literature.

サルコイド病変が心臓に限局したサルコイドーシスの１例

A 64-year-old woman was transferred to the intensive care unit with dyspnea and palpitation on effort. Chest x-ray film showed cardiomegaly and pulmonary congestion. We carefully examined for sarcoidosis as a differential diagnosis of heart failure. Serum lysozyme was mildly high, but human atrial natriuretic peptide (HANP) and brain natriuretic peptide (BNP) were strikingly high. Angiotensin converting enzyme was within normal limit. Chest roentgenogram did not reveal bilateral hilar lymphadenopathy. Atrioventricular conduction block was not observed on electrocardiogram. Echocardiographic examination showed left ventricular global hypokinesis with septal thinning and enlargement. Mitral valve regurgitation was recognized by Doppler evaluation. Coronary arteriography showed normal coronary arteries. Endomyocardial biopsy revealed noncaseous epithelioid granulomas containing, Langhans type giant cell accompanied by fibrosis and lymphocyte infiltration. From these data cardiac sarcoidosis was diagnosed. Gallium scintigraphy showed diffuse uptake only in the heart. Treatment with oral prednisolone 20 mg/day was started. Her symptoms improved by several weeks after the medical treatment. In addition, both the value of HANP and BNP were markedly decreased and echocardiogram showed improvement of cardiac systolic function. In Japan, there is a higher incidence of cardiac sarcoidosis than in the West. The prognosis of this condition associated with cardiac dysfunction is reported to be very poor. When progressive heart failure in older patients is seen, cardiac sarcoidosis should also be kept in mind. Endomyocardial biopsy play an important role as the only accurate technique for the diagnosis of cardiac sarcoidosis.

Asymptomatic Systemic Sarcoidosis Arising 5 Years After IFN-αTreatment for Chronic Hepatitis C: A New Challenge for Clinicians

Interferon (IFN)-induced sarcoidosis is well documented. Herein, we report the case of a patient with chronic hepatitis C (CHC) who developed IFN-alpha-induced sarcoidosis. The clinical features of this case make it unique among all cases so far described. The patient was, in fact, asymptomatic for sarcoidosis, and the disease, characterized by liver and lung granulomatosis, was discovered by chance during the CHC follow-up. The diagnosis was made 5 years after IFN-alpha discontinuation. A pathogenetic role for IFN-alpha in our patient is supported by a liver biopsy performed before the therapy with IFN-alpha was started, showing no evidence of granulomatous localizations. This case suggests that the incidence of sarcoidosis during IFN-alpha treatment is underestimated. A search for clinical and laboratory findings typical of the disease, as well as a liver biopsy, should always be included in the follow-up of CHC patients undergoing therapy with IFN-alpha.