Prevalence Of Sarcoidosis Research Articles

Endobronchial ultrasound-guided transbronchial needle aspiration for diagnosis of sarcoidosis in clinically unselected study populations.

Literature suggests that ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) has excellent performance characteristics for diagnosis of sarcoidosis. However, many authors challenge the external validity of EBUS-TBNA results, as most studies were performed in referral centres by highly experienced investigators, and included populations with very high sarcoidosis prevalence. We performed a systematic review and meta-analysis to estimate the role of EBUS-TBNA for diagnosis of sarcoidosis in studies enrolling consecutive patients with lymphadenopathy detected at imaging studies, regardless of the suspected underlying clinical aetiology. The Pubmed, Embase, Cinahl, Web of Science and Cochrane Library databases were screened to identify the pertinent literature. Quality of eligible studies was assessed by Quality Assessment, Data Abstraction and Synthesis-2 criteria. Pooled diagnostic yield, sensitivity and specificity were calculated, and a summary receiver operating characteristic curve was constructed. Subgroup analysis was planned to identify possible sources of study heterogeneity. Fourteen studies, collectively involving 2097 patients, fulfilled eligibility criteria. The median prevalence of sarcoidosis was 15%. EBUS-TBNA had a pooled diagnostic yield of 0.79 (standard deviation, 0.24), a pooled sensitivity of 0.84 (95% confidence interval (CI), 0.79-0.88) and a pooled specificity of 1.00 (95% CI, 0.99-1.00). Only subgroup analysis exploring the influence of study design seemed to influence the observed inter-study heterogeneity for sensitivity, retrospective studies showing worst sensitivity than prospective ones. The results of EBUS-TBNA for diagnosis of sarcoidosis in clinically unselected populations are excellent and compare favourably with published results from studies conducted in selected populations. High-quality trials would be needed to evaluate factors possibly explaining the observed heterogeneity in sensitivity.

Metastatic Sarcoidosis of Terminal Ileum and Colon: Very Rare, but Important to Differentiate From Crohn’s Disease

Introduction: Sarcoidosis prevalence is reported to be 1-40 per 100,000 in the United States, but intestinal tract involvement is only 3.4% of that, with small bowel involvement considered to be the rarest. For Crohn’s disease, terminal ileum is the most common site, whereas for sarcoidosis, it is the least likely site in intestinal tract. A 66-year-old white male with history of hypertension, remote pulmonary sarcoidosis for 8 years came with complaint of increasing frequency of bowel movements for the past 2 months. Patient denied any other complaints. He was on amlodipine and tamsulosin. He had a 10-pack-year smoking history. Chest x-ray showed resolution of lymphadenopathy seen 8 years ago. CT scan of the abdomen showed small pulmonary nodules and mild splenomegaly. WBC count was 3900/uL, absolute neutrophil count was 3100/uL, with hemoglobin 13 g/dL, MCV 87.4 fL, calcium 8.7 mg/dL with normal chemistry and liver function tests. Outpatient colonoscopy showed 2 mm yellowish plaque-like lesions in colon and thickened folds in terminal ileum. Biopsy report showed multiple non-caseating granulomas involving the lamina propria and the submucosal tissue in both the colon and terminal ileum. Special stains for fungi and acid fast bacilli were negative. A diagnosis was made of small and large bowel sarcoidosis. Usually, treatment is indicated when organ function is impaired. It is not clear whether to treat asymptomatic gastrointestinal sarcoidosis or not. We decided not to treat the patient and follow up very closely. Sarcoidosis can mimic Crohn’s disease because of its non-caseating granulomatous pattern, but diagnosis is made based upon compatible history, demonstration of granulomas in at least 2 different organs, negative staining and culture for acid fast bacilli and fungi, absence of occupational or domestic exposure to toxins, and lack of drug induced disease. On biopsy, it can be differentiated by presence of Schaumann bodies, prominent intramucosal rather than sparse submucosal granumolas, lack of mucosal architectural distortion, acute inflammation, and lack of fistulas. In a very difficult case, ACE level can be really helpful too.Figure 1: Transverse colon sarcoidosis.

Epidemiology of sarcoidosis in Afro-Caribbean people: a 7-year retrospective study in Guadeloupe.

There are no reliable epidemiological data on sarcoidosis in the French West Indies, although this disease is known to be more frequent and more severe in Black African-Americans and West Indians. This retrospective study aimed to assess the incidence and prevalence of sarcoidosis in Guadeloupe over a 7-year period and to determine its epidemiological, clinical, and evolutionary characteristics. Patients were identified through the computerized databases of the three pathology laboratories and two hospitals on the islands of Guadeloupe. Histologically proven cases of sarcoidosis were selected. All patients were recalled at a single study time-point. A total of 75 patients were identified. These included 44 women and 31 men (sex ratio: 1.4), with a mean±standard deviation (SD) age of 47±14years and Fitzpatrick skin types IV-VI. The average incidence was 2.28 per 100,000 inhabitants per year (95% confidence interval [CI] 1.69-3.02). The prevalence of sarcoidosis in 2009 was 21.09 per 100,000 inhabitants (95% CI 16.00-26.18). Most patients (61/71, 85.9%) exhibited multiple organ involvement; the mean±SD number of organs involved was 2.6±1.1. The initiation of systemic therapy was required in 75.7% of cases. Several lines of treatment were necessary in 41.5% of affected patients. At the study time-point, seven patients were found to have died. Four of these deaths were directly attributable to sarcoidosis (mortality rate: 5.3%). This epidemiological study on sarcoidosis in Guadeloupe reveals a low incidence of the disease and a high degree of severity as evidenced by the average number of affected organs, the high frequency of extrathoracic organ involvement, the frequent use of corticosteroids, and a mortality rate of 5.3%.

Sarcoidosis and pregnancy: obstetrical and neonatal outcomes in a population-based cohort of 7 million births.

Data on sarcoidosis in pregnancy is sparse and limited to a few case reports and series. Our aim is to determine the prevalence of sarcoidosis at delivery, and related maternal and newborn outcomes. Using the United States Healthcare Cost and Utilization Project Nationwide Inpatient Sample (HCUP-NIS) from 2003 to 2010, we conducted a population-based retrospective cohort study to compare women with and without sarcoidosis at delivery. We calculated the prevalence of sarcoidosis in pregnancy and used logistic regression analyses to estimate the associated risks of maternal and neonatal outcomes. There were 678 cases of sarcoidosis in 7,094,400 births over an 8-year period for an overall prevalence of nine and six-tenths cases per 100,000 births. Compared with controls, women with sarcoidosis were older, more likely to be African American and to report being smokers. Women with sarcoidosis were more likely to have preeclampsia odds ratio (OR) 1.62 (95% CI 1.18-2.22), eclampsia OR 5.27 (95% CI 1.69-16.40), deep vein thrombosis OR 4.92 (95% CI 1.58-15.33), pulmonary embolism OR 6.68 (95% CI 3.99-11.21), and premature delivery OR 1.73 (95% CI 1.40-2.15). There was also an increased risk of cesarean deliveries and postpartum hemorrhages. There were no cases of maternal death reported. Sarcoidosis in pregnancy is a rare disease associated with an increased risk of adverse obstetrical outcomes. Women with sarcoidosis can carry out successful pregnancies, however should be made aware of the higher risk of adverse events. Given the higher risk of venous thromboembolic events, consideration should be given to thromboprophylaxis in pregnancy.

Elderly-Onset Sarcoidosis: Prevalence, Clinical Course, and Treatment

Sarcoidosis is a systemic disorder of unknown cause characterized by its pathological hallmark, the non-caseating granulomas, and by variable clinical course. While most of the cases affect people aged between 25 and 40 years, approximately 30 % of cases occur in older patients. Elderly-onset sarcoidosis (EOS) is defined as the onset of sarcoidosis in people over 65 years of age. Specific studies on the incidence and prevalence of sarcoidosis in this subgroup are scarce. Several studies suggest that the clinical features of EOS differ from those of sarcoidosis in younger patients. Compared with younger patients, fatigue, uveitis and specific skin lesions are more common, while erythema nodosum and chest x-ray abnormalities are less frequent. The diagnosis of EOS is challenging and may be delayed for many months because of its insidious onset, low prevalence and similarity to other more common disorders. When there is a granulomatous reaction in the elderly, clinicians should doubt the diagnosis and first think of tuberculosis, neoplasia or rare settings such as granulomatosis with polyangiitis or granulomatous reaction due to interferon and tumour necrosis factor-α (TNFα) blockers. A minor salivary gland biopsy also has a higher accuracy for diagnosis in the elderly. The current management of EOS remains empiric because of the lack of randomized, controlled studies. However, the approach to treatment is similar, regardless of the age of the patient. The treatment may be complicated by co-morbidities and increased risk of toxicities from usual treatments, particularly steroids. This review discusses the epidemiology, clinical course, prognosis and treatment of EOS.

Dual Time Point Positron Emission Tomography/Computed Tomography Scan in Evaluation of Intrathoracic Lesions in an Area Endemic for Histoplasmosis and With High Prevalence of Sarcoidosis

BackgroundWe explored the role of dual time point fluorodeoxyglucose positron emission tomography/computed tomography (DTP PET/CT) scan in the differentiation of benign and malignant lung and mediastinal lesions. MethodsWe studied a sample of 72 consecutive patients who underwent DTP PET/CT scan for intrathoracic lesions. Information on demographics, initial and delayed maximum standardized uptake values (SUVmax) of lesions and final diagnosis were collected. Clinical criteria to diagnose benign lesions were defined as stability or regression of the lesion on follow-up after 2 years of initial detection. Sensitivity, specificity, predictive values and likelihood ratio and retention index were calculated using standard methods. ResultsSixty-three (87%) patients had increased SUVmax in delayed scan (1 hour after initial scan). Among the patients with increased delayed uptake, 51 (80%) had malignant lesion and 12 (20%) had nonmalignant lesions. All 9 patients whose SUVmax decreased on delayed scan had nonmalignant lesions. The increased SUV on delayed scan was 100% sensitive in diagnosis of cancer but was only 42% specific. The positive predictive value was 80%, whereas the negative predictive value was 100%. Likelihood ratio for positive test was 1.75. ConclusionsAll the lesions with decreased SUVmax in delayed PET scan were nonmalignant. This was true for both lung and mediastinal lesions. This could be a very helpful diagnostic finding in areas with high prevalence of benign conditions such as histoplasmosis and sarcoidosis. Multiple invasive diagnostic modalities could be prevented in a significant percentage of patients, with attendant decrease in morbidity and health care costs.

A Case of Peutz-Jeghers Syndrome in Association with Sarcoidosis

Purpose: Case Report: Our case involves a 60 year old European gentleman with a past medical history of steroid-dependent sarcoid, HTN, colon cancer and Peutz-Jeghers syndrome (PJS) who is routinely seen in our outpatient office. A routine colonoscopy for CRC screening at the age of 50 found a colonic malignancy which necessitated a sigmoid colectomy. A recurrence of his disease two years later resulted in a left hemicolectomy. At the age of 56, after a negative EGD and colonoscopy, the patient was sent to our institution for further evaluation of microcytic anemia with guaic positive stools. A capsule endoscopy revealed multiple large polyps extending from the 2nd portion of the duodenum to the distal jejunum. The patient then underwent his first DBE with antegrade approach and removal of 15 polyps. However, not all the polyps could be safely removed. The patient subsequently underwent laparoscopic resection of his small bowel. Though the patient did not have the expected findings of a history of intussusceptions, SBO or abdominal pain, close inspection of his oral cavity revealed melanin deposition on his oral mucosa and a faint mucocutaneous pigmentation. With the aforementioned clinical presentation and pathological findings, the patient was given a diagnosis of PJS. In regards to his sarcoid, the patient was diagnosed at the age of 30 when he presented to his PCP with enlarging plaques. Fifteen years later, he developed SOB and pulmonary sarcoidosis was confirmed. We have continued to be involved in his care for management and surveillance of his PJS and see him twice yearly. Discussion: The prevalence of PJS is estimated to be between 1/60,000-1/300,000. Similarly rare, the overall prevalence of sarcoidosis in Caucasian patients in the United States has been noted to be less than 10/100,000 patients. As such, the possibility of both rare diseases independently occurring in a single patient would be exceedingly unlikely. For this reason, we postulate the presence of a common genetic or biochemical pathway. PJS most commonly manifests as multiple gastrointestinal hamartomatous polyps and hyperpigmented mucosal lesions with a predisposition to developing malignancy. The underlying cause of PJS is a germ line mutation in a gene encoding the serine threonine kinase tumor suppressor STK11/LKB1 on band 19p13.3. Though the genetics of sarcoid continue to be a mystery, an association with the HLA-DR allele on band 6p21.31 has been identified in certain populations. We postulate a possible connection between these two rare genetic entities and note that further genetic and population studies may be warranted.

Reply to: Increased prevalence of Sarcoidosis in Ireland

To the Editor,Historically, published work has defined the prevalence ofSarcoidosis in Ireland as between 10 and 33 cases/100,000[1, 2]. Clinicians in Ireland believe this to be an underes-timation. Consequently, we sought to define the number ofactive cases of Sarcoidosis in a defined geographical area,namely County Offaly, in 2011.The population of this area is 76,806 (Irish Census,2011). Approval was obtained from the Midlands MedicalEthics Committee. Analysis of medical records identified65 cases giving a prevalence of 85 cases/100,000. Thirty-three percent presented with Erythema Nodosum (EN). Ofthe non-EN group, a tissue diagnosis was obtained in 80 %of cases. We have identified a prevalence for Sarcoidosis of85 cases/100,000 in County Offaly, Ireland. While theremay be inter-regional variation within Ireland [2], it sug-gests that up to 5,400 cases of Sarcoidosis potentially existwithin Ireland.References

Unexpectedly high prevalence of sarcoidosis in a representative U.S. Metropolitan population

The prevalence of sarcoidosis in the United States is unknown, with estimates ranging widely from 1 to 40 per 100,000. We sought to determine the prevalence of sarcoidosis in our health system compared to other rare lung diseases and to further establish if the prevalence was changing over time. We interrogated the electronic medical records of all patients treated in our health system from 1995 to 2010 (1.48 million patients) using the common ICD9 codes for sarcoidosis (135), lung cancer (162), and several other lung diseases characterized, like sarcoidosis, as "rare lung diseases". The patient demographic information (race, gender, age) was further analyzed to identify signature data patterns. The prevalence of sarcoidosis in our health system increased steadily from 164/100,000 in 1995 to 330/100,000 in 2010, and this trend could not be ascribed simply to changes in patient demographics or patient referral patterns. We further estimate that the prevalence of sarcoidosis exceeds 48 per 100,000 in Franklin County, Ohio, the demographic profile of which is nearly identical to that of the U.S. Sarcoidosis prevalence increased over time relative to lung cancer, a benchmark disease with stable disease prevalence, and exceeded that of other rare lung diseases. We postulate that the observed 2-fold increase in sarcoidosis disease prevalence in our health system is primarily related to improved detection and diagnostic approaches, and we conclude that the actual prevalence of sarcoidosis in central Ohio greatly exceeds current U.S. estimates.

Smoking Inhibits the Frequency of Bronchovascular Bundle Thickening in Sarcoidosis

Smoking has been associated with decreased incidence and prevalence of sarcoidosis, but few studies have evaluated effects of smoking on clinical parameters of the disease. The objectives were to determine the association of smoking with radiographic patterns and to evaluate the associations of these smoking-related radiographic patterns on airflow obstruction in sarcoidosis. Clinical data and computed tomography (CT) scans of 124 patients with sarcoidosis were reviewed. CT scans were assessed for lymph nodes, nodules, bronchiectasis, bronchovascular bundle thickening, displaced hilum, fibrosis, ground glass, emphysema, pleural changes, and alveolar opacities. CT patterns were compared between patients with and without a history of smoking. The effect of smoking on the associations between radiographic patterns and airflow obstruction was assessed with multivariable analysis. Smokers had less frequency of bronchovascular bundle thickening than nonsmokers (11/38 subjects [29%] vs 50/86 subjects [58%], P = .003) and more emphysema (7/38 subjects [18%] vs 1/86 subjects [1%], P = .001). Patients who had bronchovascular bundle thickening were less likely to have ever smoked (11/61 subjects [18%] vs 27/63 subjects [43%], P = .003) or be current smokers (4/61 subjects [7%] vs 15/63 subjects [24%], P = .008). Age (P = .003) and bronchovascular bundle thickening (P = .02) were independent predictors of airflow obstruction. There were no differences in smoking history between patients with airflow obstruction versus those without (10/37 subjects [27%] vs 28/87 subjects [32%], P = .63). In patients with sarcoidosis, smoking is associated with decreased frequency of bronchovascular bundle thickening, an important clinical manifestation of the lung disease. Further, bronchovascular bundle thickening and age are the only independent predictors of airflow obstruction, and smoking does not confound these associations.

Sarcoidosis in Black Women in the United States: Data From the Black Women's Health Study

Sarcoidosis is a systemic granulomatous disorder of unknown cause that occurs among men and women of all races. In the United States, black women are most frequently and most severely affected. There have been few epidemiologic studies of sarcoidosis focusing on black women.In this article, we present data on incidence, prevalence, and clinical characteristics of sarcoidosis among participants in the Black Women's Health Study, a cohort study of 59,000 black women from across the United States. Data on incident disease and potential risk factors are obtained through biennial questionnaires. Follow-up has been > 80% through six completed cycles.There were 685 prevalent cases of sarcoidosis at baseline in 1995 and 435 incident cases reported during 611,585 person-years of follow-up through 2007, for an average annual incidence rate of 71/100,000 and a current prevalence of 2.0%. The sarcoid diagnosis was confirmed in 96% of self-reported cases for whom medical records or physician checklists were obtained. The most frequently affected site was the lung. Most patients also had extrapulmonary involvement, with the most common sites being lymph nodes, skin, and eyes. Prednisone had the highest prevalence of use, followed by inhaled corticosteroids.This study confirms previous reports of high incidence and prevalence of sarcoidosis among black women, as well as the extent of extrapulmonary disease, frequent need for steroid therapy, and comorbid conditions in this population. The prospective identification of sarcoidosis cases from a defined population will enable a valid assessment of risk factors for incident disease as follow-up continues.

Genome-wide association study of rheumatoid arthritis by a score test based on wavelet transformation

We have conducted a genome-wide association study on the Genetic Analysis Workshop (GAW) 16 rheumatoid arthritis data using a multilocus score test based on wavelet transform proposed recently by the authors. The wavelet-based test automatically adjusts for the amount of noise suppressed from the data. The power of the test is also increased by using the genetic information contained in the spatial ordering of single-nucleotide polymorphisms on a chromosome. After adjusting for the effect of population stratification, the test identified some previously discovered rheumatoid arthritis susceptibility loci (HLA-DRB1 and rs3761847) as well as some loci (rs2076530 and rs3130340) known to have association with sarcoidosis and bone mineral density. It was previously reported that patients with rheumatoid arthritis have elevated prevalence of sarcoidosis and have reduced bone mass. This new test provides a useful tool in genome-wide association studies.

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.

Use of Insurance Claims Data to Determine Prevalence and Confirm a Cluster of Sarcoidosis Cases in Vermont

In 2006, the Vermont Department of Health was asked to respond to a potential cluster of sarcoidosis cases related to a Vermont office building. Sarcoidosis prevalence has not been formally described for the United States. A range of < 1-40/100,000 is commonly reported; however, we have not identified primary sources supporting this conclusion. Because of the wide prevalence range and lack of a local estimate, confirming existence of a cluster was difficult. We ascertained the prevalence of sarcoidosis cases in Vermont by using insurance claims data to determine whether or not a cluster of sarcoidosis cases was related to the office building. We calculated county and state annual prevalence proportions for sarcoidosis for 2004 and 2005 and annual building prevalences for 1992-2006. The pooled sarcoidosis case prevalence for Vermont was 66.1/100,000. The pooled building annual prevalence (1,128/100,000) was statistically different from the county in which the building is located (odds ratio = 15.5, 95% confidence interval 3.0, 50.3). We reported the first statewide sarcoidosis prevalence in the United States. This prevalence exceeded previous limited and unsubstantiated U.S. reports. Even with Vermont's elevated sarcoidosis prevalence, the presence of a cluster in this building was apparent.

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.

AIRFLOW LIMITATION IN BLACK VERSUS WHITE PATIENTS WITH SARCOIDOSIS

PURPOSE: The prevalence of sarcoidosis in African-Americans is 150-200 per 100,000 population, approximately 3-4 times the European-American population. Morbidity and mortality due to sarcoidosis is higher in African-Americans. We hypothesize that African-American patients with sarcoidosis have different degrees of pulmonary function limitation than their European-American counterparts.

Sarcoidosis Masquerading as an Acute Coronary Syndrome

Sarcoidosis is a common multisystem granulomatous disease that affects 20 per 100,000 people in the world. Although primarily a pulmonary disease, sarcoidosis can affect multiple organ systems. Cardiac involvement of sarcoidosis is most commonly manifested as ventricular ectopy and arrhythmias; atrioventricular conduction disturbances, including complete heart block; congestive heart failure; and even may result in sudden death. Although myocardial infarction is most commonly the result of epicardial coronary artery disease, an infiltrative process such as sarcoidosis may simulate an acute coronary syndrome. Given the high prevalence of sarcoidosis, it is important to be aware of the myriad of cardiac manifestations of this infiltrative process.

Severe cholestatic granuloumatous hepatitis in sarcoidosis disease with minimal pulmonary involvement

Extrapulmonary manifestations of sarcoidosis are frequently reported. Liver involvement is usually inapparent, with only mild elevation of liver function tests, whereas progressive cholestasis and/or cirrhosis are rarely reported. Sarcoidosis prevalence differs among racial and ethnic groups, and it is known to affect blacks more acutely and more severely than it does people of other races. We report the case of a black nigerian 35-yr old woman, living in Italy since 1988, admitted because fever, itching and increased cholestatic liver tests (GT 1837 IU, ALP 3854 IU, total bilirubin 3.5 mg/dl, AST 71 IU, ALT 84 IU, Cholesterol 448 mg/dl). Abdominal US revealed gallstones and associated cholecystitis, and hepatosplenomegaly. She underwent laparoscopic cholecystectomy: during the procedure an enlarged lymph node close to the gallbladder was collected and a liver biopsy was performed. Histology showed multiple confluent non caseating granulomas, with epitheliod and multinucleated giant cells. All serological and antibodies tests, and microbiological cultures were negative. Chest X-ray, CT and HRCT were normal, whereas abdominal CT confirmed an important hepatosplenomegaly, with numerous hypodense nodular splenic lesions, and hepatic hilar-node enlargement. No significant pulmonary physiologic abnormalities were detected, nevertheless a broncoscopy was performed, with transbronchial lung biopsy and bronco-alveolar-lavage collections. Lung biopsies confirmed the presence of granulomatous involvement and the BAL showed a typical sarcoidosis pattern. Serum ACE (angiotensin-converting-enzyme) activity was 70 U/L. Corticosteroid therapy was started (prednisolone 50 mg daily, with gradual tapering of the dose), plus azatioprine (150 mg daily) and ursodeoxycholic acid (1350 mg daily). After six month of treatment transaminases, bilirubin and ACE levels were in the normal range, whereas GT and ALP decreased significantly (1249 and 1331, respectively). This single case of sarcoidosis presented to the clinician almost exclusively with liver disease; it confirms that in Africans the disease is characterized by more severe extrapulmonary involvement. The consideration of sarcoidosis in such cases is of utmost importance, since the differential diagnosis of hepatic granulomas includes infectious diseases in which treatment with corticosteroids could be fatal.

Is there a role for microorganisms in the pathogenesis of sarcoidosis?

Sarcoidosis is a granulomatous disease that has the immunopathological features of being antigen-driven. It is a complex disease that appears to arise from the interaction of one or more triggers with an immunologically predisposed host. Previous reports of familial clustering and varying prevalence of sarcoidosis in different populations could reflect differences in ethnic predisposition or differences in local environmental exposures. This review focuses specifically on these areas that have been the subjects of intensive investigation recently. Specific focus is provided on the issue of an infective trigger and highlights popular candidates. It is concluded that microbes are a likely trigger (but not as an infection) in a genetically predisposed individual and that this initial event culminates in the sarcoidosis granulomatous response.

Familial sarcoidosis in a jordanian family

Abstract Sarcoidosis is an idiopathic granulomatous disease involving one or more multiple organ systems, characterised by the histologic finding of noncaseating epitheloid cell granulomas. This is the first report of familial sarcoidosis in Jordan. We report a mother and her son who Presented with bilateral hilar lymphadenopathy and lung infiltrates. Sarcoidosis is a rare disease in Jordan. We reported thirty-three cases of sarcoidosis(1). Although the familial tendency is reported in many studies(2), this was not the case in our study. Though the etiology is unknown; both Prevalence of sarcoidosis in different ethnic groups and familial occurrence of the disease strongly suggest the hypothesis of a possible genetic predisposition(3).