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The patient global assessment (PGA) is a reported outcome instrument used to gauge the patient's well-being. We performed a prospective study of patients seen at the University of Cincinnati Sarcoidosis Clinic. Two groups were studied: those at first visit during the time period (initial) and those seen at least one more time by the same physician (follow-up). A total of 1006, including 677 initial, visits occurred during the six-month period. Patients in whom anti-inflammatory treatment was initiated or increased had a significantly lower PGA score (ANOVA p < 0.001, p < 0.05 for increased versus all others). There was no significant difference in initial PGA score based on race, sex, or age. The change in PGA was significantly lower for patients in whom treatment was increased (ANOVA p < 0.001, increased different from all others, p < 0.05). The PGA was significantly lower for patients in whom anti-inflammatory therapy was increased; however, there was overlap between groups., Author(s): Robert P. Baughman [sup.1] [sup.2], Jacob Kotzin [sup.1], Elyse E. Lower [sup.1] Author Affiliations: (1) grid.413561.4, 0000 0000 9881 9161, Department of Medicine, University of Cincinnati Medical Center, , [...]

We present an updated overview of the hematological involvementassociated with sarcoidosis, including a management approach forcytopenias and revisiting the association with hematologicalmalignancies. Theetiology of cytopenias in sarcoidosis can be attributed to two majoretiopathogenic mechanisms: infiltration of hematopoietic organs suchas the spleen and bone marrow, and autoimmune-mediated cytopenias.With respect to the association with hematological malignancies, itrequires careful evaluation of patients from a chronologicalperspective. Patients must be classified into one of three pathogenicscenarios, including preexisting hematological malignancies,synchronous development of malignancy and sarcoidosis due to commonpredisposing factors, or sarcoidosis as a predisposing factor formalignancies. The association between sarcoidosis and hematologic involvement isbest understood as a pathogenic continuum, with cytopenias andhematologic neoplasms intertwined due to various etiopathogenicmechanisms. These mechanisms include sarcoid infiltration ofhematopoietic organs, common predisposing immunogenetics for thedevelopment of autoimmune cytopenias and malignancies, and anincreased risk of neoplasm development in patients with autoimmunecytopenias. Collaboration among the main specialties involved in theclinical management of these patients is crucial for an earlymonitoring and management. [ABSTRACT FROM AUTHOR]

Purpose The dose of repository corticotropin (RCI) and need for a loading dose in sarcoidosis patients receiving chronic corticosteroids are unclear. We performed a single-blind prospective study, comparing two doses of RCI in sarcoidosis. Methods Chronic pulmonary sarcoidosis patients receiving prednisone therapy with deterioration by 5% in FVC in the previous year were studied. RCI was administered subcutaneously at a loading dose of 80 units RCI for 10 days. Patients were randomized at day 14 to receive either 40- or 80-unit RCI twice a week. The dose of prednisone was modified by the clinician who was blinded to the patient's dosage of RCI. Results Sixteen patients completed the full 24 weeks of the study. At week 24, there was a decrease in the dose of prednisone, and improvements in DLCO, King's Sarcoidosis Questionnaire health status and fatigue score. There was no significant change in FVC % predicted. For the PET scan, there was a significant fall in the standard uptake value (SUV) of the lung lesions. Only 3/8 patients remained on 80 units RCI for full 24 weeks. There was no significant difference in the response to therapy for those treated with 40- versus 80-unit RCI. Conclusions Repository corticotropin treatment was prednisone-sparing and associated with significant improvement in DLCO, PET scan, and patient-reported outcome measures. A dose of 40-unit RCI twice a week was as effective as 80-unit RCI and was better tolerated., Author(s): Robert P. Baughman [sup.1] , Nadera Sweiss [sup.2] , Ruth Keijsers [sup.3] , Surinder S. Birring [sup.4] , Ralph Shipley [sup.5] , Lesley Ann Saketkoo [sup.6] , Elyse E. [...]

Rationale: The Sarcoidosis Diagnostic Score (SDS) has been established to quantitate the clinical features consistent with sarcoidosis in a monocentric study.Objectives: We aimed to confirm the diagnostic value of SDS in a large, multicontinental study and to assess the utility of SDS in differentiating sarcoidosis from alternative diagnosis including infectious and non-infectious granulomatous diseases.Methods: We included patients with biopsy-confirmed sarcoidosis at nine centers across the world. Patients without sarcoidosis seen at the same sites served as control patients. Using a modified World Association of Sarcoidosis and Other Granulomatous Disorders organ assessment instrument, we scored all patients for presence of granuloma on biopsy, highly probable symptoms, and least probable symptoms for each area. Two sarcoidosis scores were generated: SDS Biopsy (with biopsy) and SDS Clinical (without biopsy). SDS Clinical and Biopsy were calculated for all patients. We calculated and compared the Area Under Curve (AUC) for SDS Clinical and Biopsy according to different diagnosis scenarios.Results: A total of 1041 patients with sarcoidosis and 1035 without sarcoidosis were included. SDS Clinical (AUC=0.888, 95%CI: 0.874-0.902) and SDS Biopsy (AUC=0.979, 95%CI: 0.973-0.985) according to AUC were good to excellent for differentiating sarcoidosis from alternative diagnosis. SDS Clinical was less discriminatory in male (p=0.01) and in "high tuberculosis prevalence" centers (p<0.001). However, SDS Clinical (AUC=0.684, 95%CI: 0.602-0.766) and SDS Biopsy (AUC=0.754, 95%CI: 0.673-0.835) were not sufficiently discriminative for non-infectious granulomatous diseases, but both SDS could differentiate sarcoidosis from infectious granulomatous diseases. Algorithms were proposed for the SDS Clinical and SDS Biopsy to assist the clinician in the diagnostic process, and proposed cut-off values for the SDS Clinical and SDS Biopsy allowing the diagnosis of sarcoidosis to be safely confirmed or rejected in most cases except non-infectious granulomatous disease.Conclusions: This multicontinental study confirms that both SDS Clinical and SDS Biopsy have good to excellent performance to discriminate sarcoidosis from alternative diagnoses. Differences in the AUC were seen for "high tuberculosis prevalence" versus "low tuberculosis prevalence" centers and males versus females. Both SDSs had good discriminatory function for infectious granulomatous disease but failed in case of non-infectious granulomatous disease such as berylliosis. [ABSTRACT FROM AUTHOR]