Sarcoidosis Diagnostic Score (SDS) system: Impact of race, sex, organ involvement and duration of symptoms prior to diagnosis.

BACKGROUND: The Sarcoidosis Diagnostic Score (SDS) system has been established for sarcoidosis patients based on the WASOG organ involvement criteria. We evaluated modifications of the SDS system to determine if they improved its the diagnostic accuracy. METHODS: Biopsy-confirmed patients with sarcoidosis seen during a 7-month period at 9 sarcoidosis centers across the world. Patients with non-sarcoidosis seen at the same sites were served as control patients. Comparing the SDS-biopsy and SDS-clinical values of five groups: duration of symptoms prior to evaluation (≤1 years vs.>1 years, ≤2 years vs.>2 years), organ involvement (lung, eye, or cardiac), race, and sex. RESULTS: A total of 990 patients with sarcoidosis and 1011 controls were included in this study. The SDS-clinical was significantly more discriminating for those undergoing assessment with symptoms for more than one year (z-statistic=2.570, p = 0.0102) or two years (z-statistic=2.546, p = 0.0109). However, the addition of two points for both >1 years and >2 years since onset of symptoms did not increase sensitivity and specificity of diagnosis with the SDS system. The SDS-clinical cut-off for patients with ocular or cardiac disease was two points higher than that for lung disease. There was no difference in SDS-clinical or biopsy AUC values based on gender or race. CONCLUSIONS: The longer the duration of symptoms prior to diagnosis, the more likely the diagnosis of sarcoidosis was correct. For patients presenting with ocular or cardiac symptoms, evidence of multi-organ involved can improve the diagnostic accuracy of the SDS-clinical.

Fibrotic Pulmonary Sarcoidosis.

Fibrotic pulmonary sarcoidosis (fPS) affects about 20% of patients. fPS carries a significant morbidity and mortality. However, its prognosis is highly variable, depending mainly on fibrosis extent, functional impairment severity, and the development of pulmonary hypertension. Moreover, fPS outcomes are also influenced by several other complications, including acute exacerbations, and infections. fPS natural history is unknown, in particular regarding the risk of progressive self-sustaining fibrosis. The management of fPS is challenging, including anti-inflammatory treatment if granulomatous activity persists, rehabilitation, and in highly selected patients antifibrotic treatment and lung transplantation.

Infectious Complications of Pulmonary Sarcoidosis.

In this review, the infectious complications observed in sarcoidosis are considered from a practical point of view to help the clinician not to overlook them in a difficult context, as pulmonary sarcoidosis makes the recognition of superinfections more difficult. An increased incidence of community-acquired pneumonia and of opportunistic pneumonia has been reported, especially in immunosuppressed patients. Pulmonary destructive lesions of advanced sarcoidosis increase the incidence of chronic pulmonary aspergillosis and infection by other agents. Screening and treatment of latent tuberculosis infection are crucial to prevent severe tuberculosis. Severity in COVID-19 appears to be increased by comorbidities rather than by sarcoidosis per se. The diagnosis of infectious complications can be challenging and should be considered as a potential differential diagnosis when the exacerbation of sarcoidosis is suspected. These complications not only increase the need for hospitalizations, but also increase the risk of death. This aspect must be carefully considered when assessing the overall health burden associated with sarcoidosis. The impact of immune dysregulation on infectious risk is unclear except in exceptional cases. In the absence of evidence-based studies on immunosuppressants in the specific context of pulmonary sarcoidosis, it is recommended to apply guidelines used in areas outside sarcoidosis. Preventive measures are essential, beginning with an appropriate use of immunosuppressants and the avoidance of unjustified treatments and doses. This approach should take into account the risk of tuberculosis, especially in highly endemic countries. Additionally, parallel emphasis should be placed on vaccinations, especially against COVID-19.

U-net convolutional neural network applied to progressive fibrotic interstitial lung disease: Is progression at CT scan associated with a clinical outcome?

BACKGROUND: Computational advances in artificial intelligence have led to the recent emergence of U-Net convolutional neural networks (CNNs) applied to medical imaging. Our objectives were to assess the progression of fibrotic interstitial lung disease (ILD) using routine CT scans processed by a U-Net CNN developed by our research team, and to identify a progression threshold indicative of poor prognosis. METHODS: CT scans and clinical history of 32 patients with idiopathic fibrotic ILDs were retrospectively reviewed. Successive CT scans were processed by the U-Net CNN and ILD quantification was obtained. Correlation between ILD and FVC changes was assessed. ROC curve was used to define a threshold of ILD progression rate (PR) to predict poor prognostic (mortality or lung transplantation). The PR threshold was used to compare the cohort survival with Kaplan Mayer curves and log-rank test. RESULTS: The follow-up was 3.8 ± 1.5 years encompassing 105 CT scans, with 3.3 ± 1.1 CT scans per patient. A significant correlation between ILD and FVC changes was obtained (p = 0.004, ρ = -0.30 [95% CI: -0.16 to -0.45]). Sixteen patients (50%) experienced unfavorable outcome including 13 deaths and 3 lung transplantations. ROC curve analysis showed an aera under curve of 0.83 (p < 0.001), with an optimal cut-off PR value of 4%/year. Patients exhibiting a PR ≥ 4%/year during the first two years had a poorer prognosis (p = 0.001). CONCLUSIONS: Applying a U-Net CNN to routine CT scan allowed identifying patients with a rapid progression and unfavorable outcome.