Outcome of Sarcoidosis
Section snippets
Spontaneous regression
The clinical expression, natural history, and prognosis of sarcoidosis are highly variable [2]. Spontaneous regression occurs in nearly two thirds of patients, but Siltzbach's scheme and the authors' experience suggest that this takes several years in most cases. Clarification of the natural history of sarcoidosis is confounded by the influence of corticosteroid therapy, which usually is offered to patients who have signs and symptoms. In the classic report from Katz [9], 60% to 80% of patients
Persistence and chronicity
In most patients, sarcoidosis evolves into chronic courses. Acute onset with good prognosis can be expected in cases with erythema nodosum (EN), anterior uveitis, and acute arthritis. There has been discussion on how long patients who have sarcoidosis should be followed to identify cases in the chronic stage.
In the authors' study, they selected a special population of patients who were asymptomatic, whose disease had been detected when they were in their 20s, and who had no extrathoracic
Relapse and recurrence
In most the large series published to date, one third to one half of patients who had sarcoidosis were treated with corticosteroids [10]. Patients stabilize or improve with treatment, but 16% to 74% of them relapse when the corticosteroids are tapered or discontinued [7], [10], [12].
In a large study from the United States, 337 patients who had sarcoidosis were divided into three groups: spontaneous remission, corticosteroid-induced remission, and recalcitrant [13]. The rate of relapse was 74%
Deterioration
Deterioration usually means a progression of pulmonary fibrosis, cardiac failure, prominent decrease in visual acuity, and impaired activity of daily living caused by neuromuscular lesions in patients who have sarcoidosis.
One study reported 17 deaths by sarcoidosis among a population of 254 patients followed for 27 years from the initial diagnosis at admission [15]. In this report, respiratory symptoms at presentation were independently related to overall mortality. A similar result has been
Mortality and morbidity
According to death certificate reports of 26,866,600 people who died from 1979 to 1991 in the United States, 5791 deaths were caused by sarcoidosis or a complication of the disease. This mortality rate seems to be low. The reported mortality caused by sarcoidosis varies by region, sex, and race. The age-adjusted mortality rates increased from 1.3 deaths per 1 million population in 1979 to 1.6 deaths per 1 million population in 1991 among men, and from 1.9% to 2.5%, respectively, among women.
Organ involvement of sarcoidosis and outcomes
A large population of sarcoidosis patients (818) was analyzed retrospectively to study the prognoses for individual manifestations of the disease [24]. EN, acute arthritis, and BHL were confirmed to have good prognoses, while cor pulmonale, nephrocalcinosis, lupus pernio, and upper respiratory mucosal involvement had unfavorable clinical courses. Hepatomegaly carried a worse prognosis than splenomegaly or pulmonary involvement without BHL. These results reflected the classical understanding
Clinical phenotypes
The WASOG (World Association of Sarcoidosis and Other Granulomatous Disorders) task group tried to define clinical phenotypes in association with clinical outcomes in sarcoidosis patients diagnosed according to the standardized diagnostic instrument proposed by the ACCESS study [25]. The clinical phenotypes were defined at least 5 years after detection or onset. Five years was identified as the start of a chronic phase, based on rates of remnant shadows on chest radiographs (see Fig. 1). At the
Genotypes, clinical phenotypes, and clinical outcomes
An interplay between genetic and environmental factors has been considered in the pathogenesis of sarcoidosis. No single causative environmental factor has been identified from the ACCESS study. Family clustering and differences in the racial incidence of sarcoidosis support an inherited susceptibility. Although several susceptibility genes have been reported, few of them are associated with clinical outcomes. As part of the ACCESS study, one group tested the hypothesis that sibling pairs who
Pulmonary hypertension as a prognostic factor
Pulmonary hypertension can be associated with sarcoidosis, although this is generally uncommon. Most cases are detected in patients who have advanced pulmonary fibrotic lesions or prominent hilar lymphadenopathy, and very few are found in association with veno-occlusive disease [35], [36], [37], [38]. This clinical manifestation is not constellated adequately in the current clinical phenotypes or in the organ involvement standardized by ACCESS. When pulmonary hypertension is diagnosed in a
Novel drug therapy and the outcome of sarcoidosis
Corticosteroid is a standard therapeutic drug for sarcoidosis patients whose clinical phenotype requires treatment. Some reports demonstrate favorable responses, although there has been debate as to whether corticosteroids should be introduced earlier or later in the clinical course [11], [45], [46], [47]. The wide distribution of the organ involvement, the presence of extrathoracic lesions, and several other prognostic factors suggest that a portion of patients in the subgroup requiring
Summary
Clinical outcomes of sarcoidosis include spontaneous regression, improvement with treatment, persistence of lesions (chronicity), relapse/recurrence, and deterioration. Based on the proposed clinical phenotypes, most patients are in the chronic stage, and more than 40% of patients are under treatment. Spontaneous regression and deterioration have similar frequencies. Several factors can be detected as prognostic factors associated with chronicity or deterioration. No definite relation can be
Acknowledgments
The article was approved by the ethical committee in Central Clinic/Research Center. The authors thank Mr. Simon Johnson for his linguistic review.
References (50)
Sarcoidosis: clinical features and management
Med Clin North Am
(1967)- et al.
Lofgren's syndrome revisited: a study of 186 patients
Am J Med
(1999) - et al.
Pulmonary sarcoidosis
Clin Chest Med
(1997) - et al.
The clinical course and prognosis of patients with severe, moderate, or mild sarcoidosis
J Clin Epidemiol
(1993) - et al.
Outcome in sarcoidosis. The relationship of relapse to corticosteroid therapy
Chest
(1997) - et al.
The Copenhagen National Lung Transplant Group: survival after single-lung, double-lung, and heart–lung transplantation
J Heart Lung Transplant
(2005) - et al.
Predicting mortality in patients with sarcoidosis awaiting lung transplantation
Chest
(2003) - et al.
Sarcoidosis mortality in the United States, 1979–1991; an analysis of multiple-cause mortality data
Am J Med
(1996) - et al.
Comparison of sarcoidosis phenotypes among affected African American siblings
Chest
(2006) - et al.
The gene polymorphism of tumor necrosis factor-beta, but not that of tumor necrosis factor-alpha, is associated with the prognosis of sarcoidosis
Chest
(2001)
Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis
Chest
Distinctive clinical, radiographic, and functional characteristics of patients with sarcoidosis-related pulmonary hypertension
Chest
Sarcoidosis-associated pulmonary hypertension: outcome with long-term epoprostenol treatment
Chest
Vasoresponsiveness of sarcoidosis-associated pulmonary hypertension
Chest
American Thoracic Society (ATS), The European Respiratory Society (ERS), and the World Association of sarcoidosis and other granulomatous disorders (WASOG). Statement of sarcoidosis
Am J Respir Crit Care Med
Clinical courses and prognoses of pulmonary sarcoidosis
Curr Opin Pulm Med
Presenting characteristics as predictors of duration of treatment in sarcoidosis
Q J Med
Treatment of pulmonary sarcoidosis. State of the art
Lung transplantation in sarcoidosis
Semin Respir Crit Care Med
Clinical presentation and natural history of sarcoidosis
Outcome of the treatment for sarcoidosis
Am J Respir Crit Care Med
British Thoracic Society Sarcoidosis Study; effects of long- term corticosteroid treatment
Thorax
Recurrent sarcoidosis: a study of 17 patients with 24 episodes of recurrence
Sarcoidosis Vasc Diffuse Lung Dis
Respiratory symptoms at presentation and long-term vital prognosis in patients with pulmonary sarcoidosis
Sarcoidosis
Predicting respiratory failure in sarcoidosis patients
Sarcoidosis Vasc Diffuse Lung Dis
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This work was supported by a Grant from the Central Clinic/Research Center Foundation.