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

doi:10.1378/chest.10-0413

Chest

Volume 139, Issue 1, January 2011, Pages 144-150

https://doi.org/10.1378/chest.10-0413 Get rights and content

Background

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.

Methods

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.

Results

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.

Conclusions

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.

Section snippets

Establishment of the BWHS and Follow-up

The human subjects' protocol for this study was approved by the Boston University Medical Center Institutional Review Board. The BWHS began in 1995 when 59,000 women aged 21 to 69 years enrolled through postal health questionnaires, which were sent to subscribers of Essence magazine, members of selected black women's professional organizations, and friends and relatives of early respondents. Participants indicated their informed consent by completing the questionnaires. At baseline, subjects

Prevalence, Incidence, and Demographics Among BWHS and Sarcoidosis Cases

Among the 59,027 participants in the BWHS, 685 had sarcoidosis at the time they enrolled in the study, for a prevalence of 1,160/100,000 or 1.2%. In addition, 435 incident cases were reported during 611,585 person-years of follow-up from 1995 through 2007, for an average annual incidence rate of 71/100,000. Incidence was highest, 92/100,000, among women in their 40s (Fig 1). The median age at diagnosis was 32 years (range: 16-63 years) for prevalent cases and 44 years (range: 22-77 years) for

Discussion

Our study reports on the largest sample to date of sarcoidosis in black women in the United States. We estimated an annual incidence rate of sarcoidosis of 71/100,000 over a 12-year period based on 435 incident cases, with a peak age-specific rate of 92/100,000 at ages 40 to 49. The current prevalence is 2.0%. Rybicki et al¹¹ observed an average annual incidence rate of 39/100,000 during a 5-year period of observation, and a peak age-specific incidence of 107/100,000 for black women aged 30 to

Acknowledgments

Author contributions: Dr Cozier: contributed to originating the idea for this study, supervising the data collection, designing the physician questionnaire and participant supplemental survey, analyzing and interpreting the data, and writing the article.

Dr Berman: contributed to originating the idea for this study, designing the physician questionnaire and participant supplemental survey, analyzing and interpreting the data, and writing the article.

Dr Palmer: contributed to originating the idea

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This is a retrospective, observational study. The demographic information of patients with suspected cardiac sarcoidosis and cardiac PET/CT imaging within a national registry of sarcoidosis were reviewed (n = 4561). An individual-level, continuous, time-state transition model was used for the evaluation of long-term cost-effectiveness for the combined cardiac PET/MR compared to standard of care cardiac MR followed by cardiac PET/CT.
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Sarcoidosis is a sterile non-necrotizing granulomatous disease without known causes that can involve multiple organs with a predilection for the lung and thoracic lymph nodes. Worldwide it is estimated to affect 2–160/100,000 people and has a mortality rate over 5 years of approximately 7%. For sarcoidosis patients, the cause of death is due to sarcoid in 60% of the cases, of which up to 80% are from advanced cardiopulmonary failure (pulmonary hypertension and respiratory microbial infections) in all races except in Japan were greater than 70% of the sarcoidosis deaths are due to cardiac sarcoidosis.
Scadding stages for pulmonary sarcoidosis associates with clinical outcomes. Stages I and II have radiographic remission in approximately 30%–80% of cases. Stage III only has a 10%–40% chance of resolution, while stage IV has no change of resolution. Up to 40% of pulmonary sarcoidosis patients progress to stage IV disease with lung parenchyma fibroplasia, bronchiectasis with hilar retraction and fibrocystic disease. These patients are at highest risk for the development of precapillary pulmonary hypertension, which may occur in up to 70% of these patients. Sarcoid patients with pre-capillary pulmonary hypertension can respond to targeted pulmonary arterial hypertension medications. Stage IV fibrocytic sarcoidosis with significant pulmonary physiologic impairment, >20% fibrosis on HRCT or pre-capillary pulmonary hypertension have the highest risk of mortality, which can be >40% at 5-years.
First line treatment for patients who are symptomatic (cough and dyspnea) with parenchymal infiltrates and abnormal pulmonary function testing (PFT) is oral glucocorticoids, such as prednisone with a typical starting dose of 20–40 mg daily for 2 weeks to 2 months. Prednisone can be tapered over 6–18 months if symptoms, spirometry, PFTs, and radiographs improve. Prolonged prednisone may be required to stabilize disease. Patients requiring prolonged prednisone ≥10 mg/day or those with adverse effects due to glucocorticoids may be prescribed second and third line treatements. Second and third line treatments include immunosuppressive agents (e.g., methotrexate and azathioprine) and anti-tumor necrosis factor (TNF) medication; respectively. Effective treatments for advanced fibrocystic pulmonary disease are being explored. Despite different treatments, relapse rates range from 13% to 75% depending on the stage of sarcoid, number of organs involved, socioeconomic status, and geography.
The mortality rate for sarcoidosis over a 5 year follow up is approximately 7%. Unfortunately, 10%–40% of patients with sarcoidosis develop progressive pulmonary disease, and >60% of deaths resulting from sarcoidosis are due to advance cardiopulmonary disease. Oral glucocorticoids are the first line treatment, while methotrexate and azathioprine are considered second and anti-TNF agents are third line treatments that are used solely or as glucocorticoid sparing agents for symptomatic extrapulmonary or pulmonary sarcoidosis with infiltrates on chest radiographs and abnormal PFT. Relapse rates have ranged from 13% to 75% depending on the population studied.
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The sample included 277 caucasian patients [mean age, 50±13.6; 69.7% between 31-60 years; 56.3% men]. In total, 65% had stage II sarcoidosis, whereas only 8.3% had stage III/IV disease. Mean pulmonary function (FVC, FEV₁, FEV₁/FVC and DLCO) at diagnosis was 103±21.8, 96±22.2, 76.2±8 and 81.7±21.7, respectively. The percentage of patients with normal FVC and DLCO was 72.2% and 51.8%, respectively. Radiological stage did not change significantly during follow-up (5 years; p=0.080) and only progressed in 13 patients (5.7%). At 3 years, FVC improved, whereas DLCO exacerbated significantly (p<0.001 for the two). Disease progressed in 34.5% of the patients (57/165) whose pulmonary function and radiological stage were available (both baseline and at 3 years). Age was associated with disease progression [OR=1.04 (95%CI=1.01, 1.06)]. Risk increased by 4% for each year older a patient was at diagnosis.
At 3 years, a third of patients experienced sarcoidosis progression. Age was the only factor associated with disease prognosis.
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The prevalence of sarcoidosis-related cardiomyopathy is increasing. Sarcoidosis impacts cardiac function through granulomatous infiltration of the heart, resulting in conduction disease, arrhythmia, and/or heart failure. The diagnosis of cardiac sarcoidosis (CS) can be challenging and requires clinician awareness as well as differentiation from overlapping diagnostic phenotypes, such as other forms of myocarditis and arrhythmogenic cardiomyopathy. Clinical manifestations, extracardiac involvement, histopathology, and advanced cardiac imaging can all lend support to a diagnosis of CS. The mainstay of therapy for CS is immunosuppression; however, no prospective clinical trials exist to guide management. Patients may progress to developing advanced heart failure or ventricular arrhythmia, for which ventricular assist device therapies or heart transplantation may be considered. The existing knowledge gaps in CS call for an interdisciplinary approach to both patient care and future investigation to improve mechanistic understanding and therapeutic strategies.

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Funding/Support: This work was supported by the National Cancer Institute [Grant CA58420]; and National Heart, Lung, and Blood Institute [Grant K01HL088709].

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (http://www.chestpubs.org/site/misc/reprints.xhtml).

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Chest

Original ResearchDiffuse Lung DiseaseSarcoidosis in Black Women in the United States: Data From the Black Women's Health Study

Background

Methods

Results

Conclusions

Section snippets

Establishment of the BWHS and Follow-up

Prevalence, Incidence, and Demographics Among BWHS and Sarcoidosis Cases

Discussion

Acknowledgments

Chest

Am J Med

Ann Epidemiol

Am J Clin Nutr

Fertil Steril

Chest

Epidemiology of sarcoidosis

Epidemiol Rev

Future directions in sarcoidosis research: summary of an NHLBI working group

Am J Respir Crit Care Med

Sarcoidosis

N Engl J Med

Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999

Am J Respir Crit Care Med

Epidemiology

Clinical characteristics of patients in a case control study of sarcoidosis

Am J Respir Crit Care Med

Sarcoidosis 2001

Postgrad Med J

Extrapulmonary sarcoidosis

Semin Respir Infect

Epidemiology, demographics, and genetics of sarcoidosis

Semin Respir Infect

Racial differences in sarcoidosis incidence: a 5-year study in a health maintenance organization

Am J Epidemiol

Familial sarcoidosis: analysis of 91 families

Sarcoidosis

Familial risk ratio of sarcoidosis in African-American sibs and parents

Am J Epidemiol

Familial aggregation of sarcoidosis. A case-control etiologic study of sarcoidosis (ACCESS)

Am J Respir Crit Care Med

The epidemiology of sarcoidosis in Rochester, Minnesota: a population-based study of incidence and survival

Am J Epidemiol

Design of a case control etiologic study of sarcoidosis (ACCESS)

J Clin Epidemiol

Dual effect of parity on breast cancer risk in African-American women

J Natl Cancer Inst

Original Research
Diffuse Lung Disease
Sarcoidosis in Black Women in the United States: Data From the Black Women's Health Study