WASOG statement on the diagnosis and management of sarcoidosis-associated pulmonary hypertension

Sarcoidosis-associated pulmonary hypertension (SAPH) is an important complication of advanced sarcoidosis. Over the past few years, there have been several studies dealing with screening, diagnosis and treatment of SAPH. This includes the results of two large SAPH-specific registries. A task force was established by the World Association of Sarcoidosis and Other Granulomatous disease (WASOG) to summarise the current level of knowledge in the area and provide guidance for the management of patients. A group of sarcoidosis and pulmonary hypertension experts participated in this task force. The committee developed a consensus regarding initial screening including who should undergo more specific testing with echocardiogram. Based on the results, the committee agreed upon who should undergo right-heart catheterisation and how to interpret the results. The committee felt there was no specific phenotype of a SAPH patient in whom pulmonary hypertension-specific therapy could be definitively recommended. They recommended that treatment decisions be made jointly with a sarcoidosis and pulmonary hypertension expert. The committee recognised that there were significant defects in the current knowledge regarding SAPH, but felt the statement would be useful in directing future studies

Sarcoidosis patient with lupus pernio and infliximab-induced myositis: Response to Acthar gel

Infliximab is an effective treatment for sarcoidosis patients with persistent disease despite glucocorticoids and immunosuppressive therapy. Patients receiving infliximab can experience side effects, inducing an autoimmune reaction. Treatment is unclear for sarcoidosis patients who develop autoimmune reactions to infliximab. We report a case of a patient with advanced sarcoidosis who developed a myositis type reaction to infliximab characterized by diffuse muscle achiness and weakness and marked elevations in serum creatinine phosphokinase (CPK) and aldolase. Manifestations of sarcoidosis and myositis improved after Acthar treatment. This is the first report of successful treatment with Acthar in a patient with advanced sarcoidosis with an autoimmune reaction to infliximab

SACE and IL-2R as serum biomarkers for evaluation of multi-organ involvement and prognosis of sarcoidosis

Abstract Background Serum biomarkers in the evaluation of organ involvement and prognostic monitoring of sarcoidosis have not been determined. The purpose of this study was to identify common biomarkers that could be used to assess organ involvement and monitor outcomes in sarcoidosis patients. Methods From Mar 2013 to Sep 2021, patients with newly diagnosed pulmonary sarcoidosis were enrolled in this study in Shanghai Pulmonary Hospital. The information from medical records was retrospectively collected including diagnosis, organ involvement, laboratory tests and follow up data. Differences of continuous variables between groups were analyzed by unpaired Student’s t-test. Multivariate logistic regression model was performed to identify potential independent factors associated with multiple organ involvement. Results A total of 832 patients were included in the study. There were 339 (40.7%) patients with single organ pulmonary involvement, while 493 (59.3%) patients had two to seven organs involved. Among the routine serum tests, only the serum angiotensin converting enzyme (SACE) level was an independent factor of multiple organ involvement. Compared to those patients without involvement, SACE levels were higher in patients with extra-thoracic lymph node, skin, or spleen involvement as well as abnormal calcium metabolism. Interleukin-2 receptor (IL-2R) levels were higher in patients with extra-thoracic lymph node, spleen involvement and abnormal calcium metabolism than in those without it. The mean levels of SACE and IL-2R showed upward trends paralleling the increase on number of organs involved. In follow up, SACE and IL-2R levels were both decreased in an improved patient group, while there was no obvious difference was noticed before and after treatment in patients with persistent disease. Conclusion SACE and IL-2R were useful as serum biomarkers in the initial evaluation of organ involvement as well as monitoring prognosis in sarcoidosis

METHOTREXATE IN SARCOIDOSIS:HEMATOLOGIC AND HEPATIC TOXICITY ENCOUNTERED IN A LARGE COHORT OVER A SIX YEAR PERIOD

Background: Methotrexate (MTX) is a second line agent for treatment of sarcoidosis. Its long term safety and efficacy in sarcoidosis remains unclear. Methods: This was a retrospective review of patients seen at the University of Cincinnati Sarcoidosis Clinic over a six year period. For each visit, complete blood count, liver function testing, and dosing and outcome of MTX was noted. For efficacy, we compared the outcome of therapy of a matching subgroup of patients treated with either MTX or infliximab for one year and results scored as improved, stable, or worse based on response of the target organ. Results: Over six years, 1606 sarcoidosis patients were seen with a total of 13,576 clinical visits. During the study period, 607 patients (38% of total) were receiving MTX and had available blood work. Moderate elevation of alanine aminotransferase (ALT) (>3 times upper limit normal) was seen in nine (1.6%) patients. White blood count o