Poor Outcome and Mortality in Patients with Lower Lung-Dominant Sarcoidosis.

Background: Pulmonary sarcoidosis predominantly affects the upper lung zones but sometimes affects the lower lung zones. We hypothesised that patients with lower lung zone-dominant sarcoidosis had lower baseline forced vital capacity, progressive restrictive lung function decline, and higher long-term mortality. Methods: We retrospectively reviewed clinical data including the pulmonary function tests of 108 consecutive patients with pulmonary sarcoidosis pathologically confirmed by lung and/or mediastinal lymph node biopsy from 2004 to 2014 from our database. Results: Eleven patients (10.2%) with lower lung zone-dominant sarcoidosis were compared with 97 patients with nonlower lung zone-dominant sarcoidosis. The median age of the patients with lower dominance was significantly older (71 vs. 56, p = 0.0005). The patient with lower dominance had a significantly lower baseline percent forced vital capacity (FVC) (96.0% vs. 103%, p = 0.022). The annual change in FVC was -112 mL in those with lower dominance vs. 0 mL in nonlower dominance (p = 0.0033). Fatal acute deterioration was observed in three patients (27%) in the lower dominant group. Overall survival in the lower dominant group was significantly worse. Conclusions: Patients with lower lung zone-dominant sarcoidosis had an older age and lower baseline FVC with disease progression and acute deterioration associated with higher long-term mortality.

Outcomes of SARS-CoV-2 infection in patients with pulmonary sarcoidosis: A multicenter retrospective research network study.

BACKGROUND: Analyses of COVID-19 infection outcomes in patients with preexisting pulmonary sarcoidosis are lacking and are limited to case reports or small case series with the largest study reporting outcomes of 37 patients. RESEARCH QUESTION: Retrospective cohort study to assess clinical outcomes of 945 patients with pulmonary sarcoidosis, presenting with COVID 19, compared to a propensity matched cohort of patients without sarcoidosis. STUDY DESIGN AND METHODS: Analysis of a multi-center research network TriNETX was performed including patients more than 16 years of age diagnosed with COVID-19. Outcomes in COVID-19 positive patients with concurrent pulmonary sarcoidosis were compared with a propensity score matched cohort of patients without pulmonary sarcoidosis. RESULTS: A total of 278,271 patients with COVID-19 on the research network were identified, 954 patients (0.34 %) carried a diagnosis of pulmonary sarcoidosis. Mean age of patients with sarcoidosis was 56.3 ± 13.2 years, with female predominance (n = 619, 64.89 %). 49.69 % of the participants were African American (n = 474). Co-morbidities including hypertension, chronic lower respiratory diseases, diabetes mellitus, ischemic heart disease, nicotine dependence, and chronic kidney disease were more common in patients with pulmonary sarcoidosis when compared to the non-pulmonary sarcoidosis cohort (all p values < 0.01). In unmatched analysis, pulmonary sarcoidosis group had higher mortality, increased risk for hospitalization, intubation and need for renal replacement therapy. After propensity score matching, no difference in any of the outcome measures was observed. INTERPRETATION: Crude COVID-19 mortality and other clinical outcome measures are poor in pulmonary sarcoidosis cohort; however, propensity-matched analyses revealed no difference in outcomes, showing that higher mortality is driven by higher burden of comorbidities.

Prevalence and attributable health burden of chronic respiratory diseases, 1990-2017: a systematic analysis for the Global Burden of Disease Study 2017.

BACKGROUND: Previous attempts to characterise the burden of chronic respiratory diseases have focused only on specific disease conditions, such as chronic obstructive pulmonary disease (COPD) or asthma. In this study, we aimed to characterise the burden of chronic respiratory diseases globally, providing a comprehensive and up-to-date analysis on geographical and time trends from 1990 to 2017. METHODS: Using data from the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2017, we estimated the prevalence, morbidity, and mortality attributable to chronic respiratory diseases through an analysis of deaths, disability-adjusted life-years (DALYs), and years of life lost (YLL) by GBD super-region, from 1990 to 2017, stratified by age and sex. Specific diseases analysed included asthma, COPD, interstitial lung disease and pulmonary sarcoidosis, pneumoconiosis, and other chronic respiratory diseases. We also assessed the contribution of risk factors (smoking, second-hand smoke, ambient particulate matter and ozone pollution, household air pollution from solid fuels, and occupational risks) to chronic respiratory disease-attributable DALYs. FINDINGS: In 2017, 544·9 million people (95% uncertainty interval [UI] 506·9-584·8) worldwide had a chronic respiratory disease, representing an increase of 39·8% compared with 1990. Chronic respiratory disease prevalence showed wide variability across GBD super-regions, with the highest prevalence among both males and females in high-income regions, and the lowest prevalence in sub-Saharan Africa and south Asia. The age-sex-specific prevalence of each chronic respiratory disease in 2017 was also highly variable geographically. Chronic respiratory diseases were the third leading cause of death in 2017 (7·0% [95% UI 6·8-7·2] of all deaths), behind cardiovascular diseases and neoplasms. Deaths due to chronic respiratory diseases numbered 3 914 196 (95% UI 3 790 578-4 044 819) in 2017, an increase of 18·0% since 1990, while total DALYs increased by 13·3%. However, when accounting for ageing and population growth, declines were observed in age-standardised prevalence (14·3% decrease), age-standardised death rates (42·6%), and age-standardised DALY rates (38·2%). In males and females, most chronic respiratory disease-attributable deaths and DALYs were due to COPD. In regional analyses, mortality rates from chronic respiratory diseases were greatest in south Asia and lowest in sub-Saharan Africa, also across both sexes. Notably, although absolute prevalence was lower in south Asia than in most other super-regions, YLLs due to chronic respiratory diseases across the subcontinent were the highest in the world. Death rates due to interstitial lung disease and pulmonary sarcoidosis were greater than those due to pneumoconiosis in all super-regions. Smoking was the leading risk factor for chronic respiratory disease-related disability across all regions for men. Among women, household air pollution from solid fuels was the predominant risk factor for chronic respiratory diseases in south Asia and sub-Saharan Africa, while ambient particulate matter represented the leading risk factor in southeast Asia, east Asia, and Oceania, and in the Middle East and north Africa super-region. INTERPRETATION: Our study shows that chronic respiratory diseases remain a leading cause of death and disability worldwide, with growth in absolute numbers but sharp declines in several age-standardised estimators since 1990. Premature mortality from chronic respiratory diseases seems to be highest in regions with less-resourced health systems on a per-capita basis. FUNDING: Bill & Melinda Gates Foundation.

Predictors of mortality in fibrosing pulmonary sarcoidosis.

INTRODUCTION: Pulmonary fibrosing sarcoidosis is associated with increased mortality. This study was aimed to explore the prognosis value of a panel of parameters for predicting mortality. METHODS: This retrospective study included 216 patients with confirmed stage 4 pulmonary sarcoidosis. Stage 4 diagnosis date served as baseline. The following information was systematically present at baseline: epidemiological characteristics; treatments; pulmonary function; composite physiologic index (CPI); systolic pulmonary artery pressure at echocardiography; pulmonary fibrosis extent, main pulmonary artery/ascending aorta diameters ratio (MPAD/AAD) and MPAD/body surface area (BSA) measured and calculated using computed tomography, Walsh's algorithm based on CPI, lung fibrosis extent and MPAD/AAD ratio, and modified Walsh's algorithm with MPAD/BSA replacing MPAD/AAD allowed to estimate good or bad prognosis profiles. The primary outcome of the study was all cause mortality and lung transplantation. The value of baseline parameters was tested as predictors of mortality using univariate and multivariate analyses. RESULTS: Median follow-up was 105 months. There were 41 deaths and 5 transplantations. At multivariate analysis, survival was independently predicted by several parameters including CPI, lung fibrosis extent, pulmonary hypertension at echography or MPAD/BSA ratio, Walsh's algorithm, and geographic origin. The modified Walsh's algorithm was most highly predictive. CONCLUSION: Survival was best predicted by geographic origin, lung fibrosis extent, PH at echography or MPAD/BSA ratio, as well as by various scores among them the modified Walsh's algorithm had very high predictive value thanks to MPAD/BSA ratio which accurately predicted mortality.

Lung transplantation for pulmonary sarcoidosis.

Although relatively few patients with pulmonary sarcoidosis develop advanced disease that progresses to respiratory insufficiency despite receiving best practice pharmacologic interventions, lung transplantation may be the only therapeutic option for such patients to both prolong survival and provide improved quality of life. Lung transplant can be successfully performed for patients with end-stage pulmonary sarcoidosis, and post-transplant survival is similar to that for other transplant indications such as idiopathic pulmonary fibrosis. However, appropriate timing of referral, comprehensive assessment of potential candidates for lung transplant, placement of patients on the lung transplant waiting list when within the transplant window as appropriate, choosing the best procedure (bilateral versus single lung transplant), and optimal peri-operative and post-transplant management are key to successful lung transplant outcomes for patients with sarcoidosis.

Why do people die from pulmonary sarcoidosis?

PURPOSE OF REVIEW: In sarcoidosis, the design and validation of an appropriate risk stratification strategy is hampered by the considerable variability in initial presentation, disease evolution, and outcome. Although spontaneous resolution of the disease is described in a large proportion of patients, approximately 20-30% would present with chronic or progressive lung disease that has been associated with morbidity and mortality. Higher morbidity and mortality can be related to both the disease severity and extent as well as its treatments. We review the utility of integration of clinical, pathological, and radiological features of pulmonary sarcoidosis to detect pulmonary sarcoidosis patient at risk of developing severe, fibrotic lung disease. RECENT FINDINGS: Recently published studies suggested a mortality rate of 11-14 per 1000 person-years. Demographic characteristics such as age, sex, and race may play a role but conflicting evidence are reported depending on the origin of the population. To date, there are no tools that can reliably predict the exact group of pulmonary sarcoidosis patients to progress to fibrosis. Imaging contributes significantly to the diagnosis and management of patients with sarcoidosis as it can provide useful information regarding the discrimination between reversible and irreversible disease, the extent of the parenchymal damage and the presence of possible complications. Symptoms and lung function tests are the rest of the key determinants and their change over time should be considered. SUMMARY: This review concentrates on the definition of advanced pulmonary sarcoidosis and determinants of mortality in the pulmonary sarcoidosis group of patients.

Predictors of Mortality in Pulmonary Sarcoidosis.

OBJECTIVE: The goal of this study was to assess the prognostic strength of factors in predicting respiratory death in a large cohort of patients with sarcoidosis with at least 8 years' follow-up. METHODS: Data were collected on age, sex, self-declared race, time of death, spirometry findings, diffusing capacity for carbon monoxide, chest radiograph stage, extent of fibrosis on high-resolution CT (HRCT) scanning, and presence of precapillary pulmonary hypertension (PH). The Gender-Age-Physiology (GAP) index and Walsh model criteria for high vs low risk were calculated. RESULTS: A total of 452 patients were identified who had complete pulmonary function testing and chest imaging. The median age at time of entry into the study was 50 years (range, 25-78 years). PH was confirmed by right heart catheterization in 29 (6.4%). Of 452 patients, 42 died during the time of the study, including 38 (8.4%) who died of sarcoidosis-associated respiratory failure and 4 who died of non-sarcoidosis causes. The overall mortality from sarcoidosis was 3.9% and 9.0% at 5 and 10 years, respectively. Alive patients were significantly younger than patients who died from sarcoidosis. Increased mortality was seen for black patients, stage 4 chest radiographs, > 20% fibrosis on HRCT scanning, or PH. The two composite scores (GAP and the Walsh model) were predictive of increased mortality according to univariate analysis. Using the Cox proportional hazards regression model, only age, extent of fibrosis on HRCT scanning, and PH were independent predictors of mortality. CONCLUSIONS: Although most patients with sarcoidosis do well, increased mortality was seen in those patients who were older, had extensive fibrosis on HRCT scanning, or who had PH.

Chronic pulmonary aspergillosis complicating sarcoidosis.

Chronic pulmonary aspergillosis (CPA) complicating sarcoidosis (SA) is associated with high mortality, and there is a lack of clarity regarding the relative contributions of SA or CPA.This was a retrospective single-centre study on CPA-SA.In total, 65 patients (44 men), aged 41.4±13.5 and 48.3±11.9 years at the time of SA and CPA diagnoses, respectively, were included between 1980 and 2015. Of these, 64 had fibrocystic SA, most often advanced, with composite physiological index (CPI) >40 (65% of patients) and pulmonary hypertension (PH) (31%), and 41 patients (63%) were treated for SA (corticosteroids or immunosuppressive drugs). Chronic cavitary pulmonary aspergillosis (CCPA) was the most frequent CPA pattern. Regarding treatment, 55 patients required long-term antifungals, 14 interventional radiology, 11 resection surgery and two transplantation. Nearly half of the patients (27; 41.5%) died (mean age 55.8 years); 73% of the patients achieved 5-year survival and 61% 10-year survival. Death most often resulted from advanced SA and rarely from haemoptysis. CPI, fibrosis extent and PH predicted survival. Comparison with paired healthy controls without CPA did not show any difference in survival, but a higher percentage of patients had high-risk mould exposure.CPA occurs in advanced pulmonary SA. CPA-SA is associated with high mortality due to the underlying advanced SA rather than to the CPA. CPI, fibrosis extent and PH best predict outcome.

Association of HLA antigens with the clinical course of sarcoidosis and familial disease.

Patients with sarcoidosis usually have a benign course and a favourable prognosis. Although spontaneous remission is common, a progressive disease with a severe prognosis occurs in a small but significant number of patients. The aim of this study was to evaluate the potential significance of HLA antigens as a clinical marker on the outcome of sarcoidosis patients. We conducted a retrospective cohort study for HLA class I and II allels in 74 sarcoidosis patients and 72 healthy transplant donors. Bronchoscopy and bronchial biopsies were performed in each patient. Two or more positive bronchial biopsy samples revealing granulomatous inflammation was defined as diffuse while one positive biopsy sample was considered as limited endobronchial disease. Three or more extrapulmonary organ involvement was denoted as severe extrapulmonary disease. The patients were followed-up at least for eight years.  Incidence of progressive disease was significantly high in patients with positive HLA-DRB1*07, DRB1*14 (p<0.05) and DRB1*15 (p <0.001) allels. HLA-DRB1*14 and DRB1*15 were associated with severe extrapulmonary organ involvement (p<0.001). HLA-DRB1 *14 (p<0.05) and DRB1*15 (p<0.001) were significantly more frequent in patients with diffuse endobronchial involvement. Incidence of familial disease was 14.8% with a 6.7% identical HLA typing. Presence of HLA class I and II allels may influence the severity and prognosis of sarcoidosis significantly. Apart from defining genetic susceptibility, HLA class I and class II allels appear to be relevant and crucial markers for the to predict the clinical outcome of sarcoidosis. Distinct heterogenity of sarcoidosis may arise from the particular presence of different allels in invidual patients.

Prognostic significance of surfactant protein A, surfactant protein D, Clara cell protein 16, S100 protein, trefoil factor 3, and prostatic secretory protein 94 in idiopathic pulmonary fibrosis, sarcoidosis, and chronic pulmonary obstructive disease.

BACKGROUND: Identification of serum and bronchoalveolar lavage fluid (BALF) biomarkers may facilitate diagnosis and prognostication in various lung disorders. OBJECTIVE: Serum and BALF levels of surfactant protein A (SP-A), surfactant protein D (SP-D), Clara cell protein 16 (CC16), S100 protein, trefoil factor 3 (TFF3), and prostatic secretory protein 94 (PSP94) were evaluated in 94 consecutive patients (idiopathic pulmonary fibrosis (IPF; n=18), sarcoidosis (n=25), chronic obstructive pulmonary disease (COPD; n=51)), and in 155 healthy controls. METHODS: Biomarkers were measured at diagnosis and compared with disease characteristics. Both uniparametric and multiparametric analyses were used. RESULTS: Seven significant correlations were found: 1) BALF PSP94 level correlated with prognosis of sarcoidosis (P=0.035); 2) BALF SP-D level with pulmonary functions in IPF (P=0.032); 3) BALF SP-D and TFF3 with IPF mortality (P=0.049 and 0.017, respectively); 4) serum TFF3 level with COPD mortality (P=0.006,); 5) serum SP-A with pulmonary functions impairment in IPF (P=0.011); 6) serum SP-D level was associated with HRCT interstitial score in IPF (P=0.0346); and 7) serum SP-A was associated with staging of COPD according to spirometry (P<0.001). Moreover, our analysis showed that some biomarker levels differed significantly among the diseases: 1) BALF SP-D level differed between sarcoidosis and IPF; 2) serum SP-A level differed among IPF, sarcoidosis, COPD and was also different from healthy controls; 3) serum S100A6, S100A11 levels differed among IPF, sarcoidosis, COPD from healthy controls 4) serum SP-D, CC16, TFF-3 levels distinguished IPF patients from healthy controls; and 5) serum CC16, TFF3, PSP94 distinguished COPD patients from healthy controls. Our study shows that some of selected biomarkers should have prognostic value in the analysed lung disorders. On the other hand, these biomarkers do not appear to be unequivocally suitable for differential diagnosis of these disorders.

Causes of death in patients with chronic sarcoidosis.

INTRODUCTION: Sarcoidosis is a multi-system, granulomatous disorder of unknown etiology that is associated with a variable prognosis and sometimes results in death. There are conflicting reports regarding the causes of death in patients with sarcoidosis. METHODS: Forty-four consecutive patients with sarcoidosis who underwent an autopsy (35 patients) or died at Mayo Clinic (Rochester, MN, USA) over a 20-yr period, from January 1, 1994 to December 31, 2013 were analyzed. RESULTS: The median age at death was 63 years (range, 33-94 years) and there were 22 (50%) women. Sarcoidosis had not been clinically diagnosed in 16 (36%) patients before death. Fifteen deaths (34%) were related to sarcoidosis and included seven deaths (16%) from cardiac sarcoidosis and four deaths (9%) from progressive pulmonary sarcoidosis. Other sarcoidosis-related causes of death included advanced hepatic sarcoidosis (5%) and opportunistic infections (5%) related to immunosuppressive therapy for treating sarcoidosis. Among seven patients dying from cardiac sarcoidosis, three had been diagnosed with sarcoidosis during life and cardiac involvement was known in two of them. Six of seven deaths from cardiac sarcoidosis occurred in the autopsied cohort while all four deaths from pulmonary sarcoidosis occurred in those not autopsied. CONCLUSIONS: In the majority of patients dying with sarcoidosis the cause of death is unrelated to sarcoidosis. Cardiac involvement is the most common cause of sarcoidosis-related deaths in patients subjected to postmortem examination and was usually undiagnosed during life. The cause distribution of death in patients with sarcoidosis differed depending on whether autopsy was performed.

Evaluation of Tpeak-Tend interval and Tpeak-end/QT ratio in patients with Sarcoidosis.

UNLABELLED: rdiogram (ECG), can be used as a marker of transmural dispersion of repolarization. Also, Tp-e/QT and Tp-e/QTc ratios are used as an index of arrhythmogenesis. Prolonged Tp-e interval and Tp-e/QT ratio was found associated with sudden cardiac death in different clinical conditions. Novel ventricular repolarization parameters, Tp-e interval, Tp-e/QT and Tp-e/QTc ratios have not been evaluated in patients with sarcoidosis without cardiac symptoms previously.Therefore, we investigated Tp-e interval in patients with pulmonary sarcoidosis. METHODS: Sixty-one consecutive patients diagnosed with biopsy-proven pulmonary sarcoidosis were retrospectively reviewed. The 12-lead ECG examinations were performed at speed of 25 mm/s while the patients were in supine position.  QT dispersion, Tp-e intervals and Tp-e/QT ratio were measured. RESULTS: Comparison of ECG parameters between two groups showed a significantly higher average heart rate (p< 0.05) in the patients with sarcoidosis than controls. QT dispersion was higher in patients group. Tp-e interval was significantly prolonged in the study group compared to the control group (92±21 ms and 85±14 ms, respectively; p< 0.05). Tp-e/QT and Tp-e/QTc ratios were significantly higher in patients with sarcoidosis compared to control subjects (all p values < 0.05). CONCLUSIONS: The novel repolarization parameters Tp-e interval, Tp-e/QT and Tp-e/QTc ratios are increased in patients with sarcoidosis without any cardiac symptoms. These parameters might be used as a marker for predicting the ventricular arrhythmias and sudden cardiac death in patients with sarcoidosis.

Lung transplantation for pulmonary sarcoidosis. Twenty-five years of experience in the USA.

OBJECTIVE: Lung transplantation is the ultimate treatment for end-stage pulmonary sarcoidosis. Post-transplant survival outcomes remain unclear. METHODS: Survival models were used to assess survival and graft outcomes in patients with sarcoid among 20,896 lung transplants performed in the USA. RESULTS: 695 lung recipients were transplanted for pulmonary sarcoidosis. Sarcoid lung recipients had similar median survival rate (69.7 months (IQR 60.2-79.3)) compared with the non-sarcoid lung recipients (63.1 months (IQR 61.4-64.8), p=0.88). In multivariate Cox regression, sarcoidosis was not independently associated with worse mortality (HR 0.96 (95% CI 0.85 to 1.08), p=0.51). Among the sarcoid lung recipients, double lung transplantation (HR 0.76 (0.58 to 0.99), p=0.04) and lung allocation score era (HR 0.74 (0.56 to 0.97), p=0.03) were associated with improved survival. CONCLUSIONS: Recipients of lung transplants for pulmonary sarcoidosis had similar outcomes compared with non-sarcoid lung recipients.

Patient confidence and quality of life in idiopathic pulmonary fibrosis and sarcoidosis.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) and sarcoidosis impact significantly on health-related quality of life (HRQOL). There are few studies on the impact of patient confidence on HRQOL in these conditions. OBJECTIVES: 1. To investigate whether patient confidence is associated with HRQOL, anxiety, depression, dyspnoea or fatigue. 2. To assess if patient confidence is associated with inpatient admissions, access to community healthcare and, for IPF patients, mortality and disease severity. METHODS: Study participants self-completed seven questionnaires: Hospital Anxiety and Depression Scale, EuroQol 5D (EQ5D), King's Brief Interstitial Lung Disease questionnaire, St George's Respiratory Questionnaire, MRC dyspnoea scale, Fatigue Assessment Scale and a non-validated questionnaire assessing patient confidence, symptom duration and access to community healthcare. Lung function and follow-up data were collected from hospital electronic databases. Spearman's rank correlation coefficients were calculated to assess for correlation between patient confidence, questionnaire variables and inpatient admissions. Chi-square tests were performed to assess for association between patient confidence, mortality and disease severity. RESULTS: 75 IPF patients and 69 sarcoidosis patients were recruited to the study. Patient confidence in IPF was significantly negatively correlated with depression and fatigue, and significantly positively correlated with EQ5D scores, but not healthcare outcomes. No associations were found between confidence and any of the variables assessed in sarcoidosis. CONCLUSIONS: Lower levels of confidence in IPF patients are associated with higher levels of depression and fatigue and worse HRQOL. Efforts should be made to improve patient confidence to assess the impact on HRQOL.

Placenta-derived mesenchymal-like cells (PDA-001) as therapy for chronic pulmonary sarcoidosis: a phase 1 study.

BACKGROUND: Placental derived mesenchymal-like cells have been found to have immunosuppressive effects on T cell function. We studied mesenchymal-like cells as immunomodulators in chronic pulmonary sarcoidosis. METHODS: PDA-001 cells were culture-expanded in vitro as a plastic-adherent, undifferentiated cell population that expresses the nominal phenotype CD34-, CD10+, CD105+ and CD200+. Four patients with refractory pulmonary sarcoidosis received two infusions of 150 million PDA-001 cells in 240 ml dextran-40 solution one week apart. During and for two hours after the first infusion, the pulmonary artery pressure was monitored. Prior to first infusion and within 24 hours after the second infusion, bronchoscopy and bronchoalveolar lavage (BAL) were performed. Patients underwent initial and serial pulmonary function testing and were followed for two years. RESULTS: After the first infusion, all patients had a mild, non-clinically significant increase in mean pulmonary artery pressure, but none exhibited right heart failure or volume overload. In the year following treatment, there was no significant change in the FVC, but two patients had improvement in their chest x-ray and had prednisone withdrawn. BAL samples after the second infusion were sufficiently viable to undergo FACS analysis in three cases and in two patients, CD10+CD49c+C105+ cells (indicative of PDA-001 cells) were found. CONCLUSION: The use of placental derived mesenchymal-like cells led to a mild increase in pulmonary artery pressure. In some cases, these cells were found in the BAL 24 hours after the second dose. Two of four patients demonstrated some steroid sparing benefit, including one patient with prolonged remission.

Disease Recurrence and Acute Cellular Rejection Episodes During the First Year After Lung Transplantation Among Patients With Sarcoidosis.

INTRODUCTION: Sarcoidosis is reported to recur after lung transplantation (LT). We sought to determine the frequency of recurrent disease after LT and predictors of recurrence. We also evaluated the incidence and severity of acute cellular rejection (ACR) episodes among these patients. METHODS: The database of LT patients at Cleveland Clinic was interrogated for sarcoidosis patients who underwent LT between May 1993 and 2011. Charts were reviewed for demographics, type of transplant, posttransplant biopsy findings, and outcomes. RESULTS: Data were available for 30 patients (mean age, 50 ± 9.3 years; range, 30-65 years; M-to-F ratio, 17:13; single-to-double-to-heart lung ratio, 5:24:1). Recurrence of sarcoidosis was noted among 7 patients (pathological recurrence in all and radiological findings suggesting recurrence in 1 patient) with no impact on overall outcomes. Presence of granulomas on explanted lungs was the only predictor of recurrence (85.7% vs 30.4%, odds ratio, 13.7; 1.4-136.2; P = 0.02).Overall burden of ACR episodes on all bronchoscopies was significantly lower in patients with disease recurrence (7.6 % vs 21.3% of biopsies, P = 0.038). Among patients with recurrent disease, ACR did not develop once disease recurrence had been seen on transbronchial biopsy. CONCLUSIONS: A significant proportion of sarcoidosis patients have disease recurrence after LT and presence of active granulomas on explant is associated with subsequent recurrence. There may be an association of recurrence with lower frequency of ACR episodes. There does not appear to be any impact of sarcoidosis recurrence on 1-, 3-, or 5-year survivals.