Anti-Inflammatory Therapy in Advanced Sarcoidosis: Results from The Registry for Advanced Sarcoidosis (REAS)

Baughman, Robert P.; Vucinic, Violeta; Ndili, Ogugua; Judson, Marc A.; Maier, Lisa A.; Porquera, Eva M. Carmona; Grutters, Jan C.; Wells, Athol U.; Randolph, Adam; Lower, Elyse E.

doi:10.1164/ajrccm-conference.2020.201.1_meetingabstracts.a1553

Cited by 7 publications

(8 citation statements)

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“…It has been reported that 5%-13% of patients present with neurological lesions [13][14][15], and of these, hypothalamic and pituitary lesions have been reported in ~3% [16]. Sarcoidosis often resolves spontaneously [17], but steroid treatment is often required to treat the neuropathies, including lesions of the hypothalamus and pituitary. However, such treatment is often unsuccessful: Anthony et al studied 46 patients with neuropathy and poor thalamic/pituitary function [18], of which 43 required treatment with steroids, but only five patients improved with treatment.…”

Section: Discussionmentioning

confidence: 99%

Lymphocytic panhypophysitis and anti-rabphilin-3A antibody with pulmonary sarcoidosis

et al. 2022

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Purpose: To explore the clinical significance of anti-rabphillin-3A antibody for the differential diagnosis of lymphocytic panhypophysitis. Methods and Results: A 58-year-old Japanese man developed uveitis of unknown cause in 2017. In 2019, he became aware of polyuria. In August 2020, he noticed transient diplopia and was diagnosed with right abducens nerve palsy. At the same time, he complained of fatigue and loss of appetite. Head magnetic resonance imaging demonstrated enlargement of the pituitary stalk and pituitary gland, corresponding to hypophysitis. Hormone stimulation tests showed blunted responses with respect to all anterior pituitary hormones. Central diabetes insipidus was diagnosed on the basis of a hypertonic saline loading test. Taking these findings together, a diagnosis of panhypopituitarism was made. Computed tomography showed enlargement of hilar lymph nodes. Biopsies of the hilar lymph nodes revealed non-caseating epithelioid cell granulomas that were consistent with sarcoidosis. Biopsy of the anterior pituitary revealed mild lymphocyte infiltration in the absence of IgG4-positive cells, non-caseating granulomas, or neoplasia. Western blotting revealed the presence of anti-rabphilin-3A antibody, supporting a diagnosis of lymphocytic panhypophysitis. Because the patient had no visual impairment or severe uveitis, we continued physiological hormone replacement therapy and topical steroid therapy for the uveitis. Conclusion: To the best of our knowledge, this is the first case of anti-rabphilin 3A antibody positive lymphocytic panhypophysitis comorbid with sarcoidosis, diagnosed by both pituitary and hilar lymph node biopsy. The utility of anti-rabphilin-3A antibody for the differential diagnosis of hypophysitis like this case should be clarified with further 4 case studies.

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Section: Discussionmentioning

confidence: 99%

Lymphocytic panhypophysitis and anti-rabphilin-3A antibody with pulmonary sarcoidosis

et al. 2022

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“…To determine possible related characteristics to active signaling pathways, basic characteristics (age at diagnosis, gender, comorbidities as reported in the medical records), Scadding stage at diagnosis and follow-up, presence of Löfgren syndrome, and therapy, as well as organ manifestation, were collected from medical records of patients. The disease status of patients was retrospectively examined and classified according to the Clinical Outcome Status (COS) [ 27 ] 2 and 5 years after diagnosis. COS categorizes patients into 9 scores: (1) resolved disease, never treated, (2) resolved disease, no therapy > one year, (3) minimal disease, never treated, (4) minimal disease, no therapy > one year, (5) persistent disease, never treated, (6) persistent disease, no therapy > one year, (7) persistent disease, current therapy but no worsening in prior year and asymptomatic, (8) persistent disease, current therapy but no worsening in prior year and symptomatic, and (9) persistent disease, current therapy which worsened in the prior year.…”

Section: Methodsmentioning

confidence: 99%

Simultaneous Assessment of mTORC1, JAK/STAT, and NLRP3 Inflammasome Activation Pathways in Patients with Sarcoidosis

Kraaijvanger

Ambarus

Damen

et al. 2023

IJMS

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The unknown etiology of sarcoidosis, along with the variability in organ involvement and disease course, complicates the effective treatment of this disease. Based on recent studies, the cellular inflammatory pathways involved in granuloma formation are of interest regarding possible new treatment options, such as the mechanistic (formerly mammalian) target of rapamycin complex 1 (mTORC1) pathway, the Janus kinase/signal transducers and activators of transcription (JAK/STAT) pathway, and the nucleotide-binding domain, leucine-rich-containing family, pyrin domain-containing-3 (NLRP3) inflammasome pathway. The aim of this study was to explore the potential coexpression of these three inflammatory pathways in patients with sarcoidosis and see whether possible differences were related to disease outcome. The tissue of 60 patients with sarcoidosis was used to determine the activity of these three signaling pathways using immunohistochemistry. The activation of NLRP3 was present in 85% of all patients, and the activation of mTORC1 and JAK/STAT was present in 49% and 50% of patients, respectively. Furthermore, the presence of NLRP3 activation at diagnosis was associated with a chronic disease course of sarcoidosis. Our finding of different new conceptual inflammatory tissue phenotypes in sarcoidosis could possibly guide future treatment studies using the available inhibitors of either NLRP3, JAK-STAT, and mTORC1 inhibitors in a more personalized medicine approach.

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“…Phenotypic classifications aim to predict individual patient outcomes, guide specific therapeutic interventions and assist clinicians in the management of patients, or refer them to experienced centers [13][14][15]. In 1960, Karl Wurm first proposed a phenotypical staging of sarcoidosis [16], and it was modified by Guy Scadding in 1961 [17].…”

Section: Phenotyping the Diseasementioning

confidence: 99%

Phenotypes and Serum Biomarkers in Sarcoidosis

Della Zoppa,

Bertuccio,

Campo

et al. 2024

Diagnostics

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Sarcoidosis is a multisystem disease, which is diagnosed on a compatible clinical presentation, non-necrotizing granulomatous inflammation in one or more tissue samples, and exclusion of alternative causes of granulomatous disease. Considering its heterogeneity, numerous aspects of the disease remain to be elucidated. In this context, the identification and integration of biomarkers may hold significance in clinical practice, aiding in appropriate selection of patients for targeted clinical trials. This work aims to discuss and analyze how validated biomarkers are currently integrated in disease category definitions. Future studies are mandatory to unravel the diverse contributions of genetics, socioeconomic status, environmental exposures, and other sociodemographic variables to disease severity and phenotypic presentation. Furthermore, the implementation of transcriptomics, multidisciplinary approaches, and consideration of patients’ perspectives, reporting innovative insights, could be pivotal for a better understanding of disease pathogenesis and the optimization of clinical assistance.

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Anti-Inflammatory Therapy in Advanced Sarcoidosis: Results from The Registry for Advanced Sarcoidosis (REAS)

Cited by 7 publications

References 0 publications

Lymphocytic panhypophysitis and anti-rabphilin-3A antibody with pulmonary sarcoidosis

Lymphocytic panhypophysitis and anti-rabphilin-3A antibody with pulmonary sarcoidosis

Simultaneous Assessment of mTORC1, JAK/STAT, and NLRP3 Inflammasome Activation Pathways in Patients with Sarcoidosis

Phenotypes and Serum Biomarkers in Sarcoidosis

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