Definition and Consensus Diagnostic Criteria for Neurosarcoidosis: From the Neurosarcoidosis Consortium Consensus Group

Barney J. Stern, Walter Royal, Jeffrey M. Gelfand, David B. Clifford, Jinny Tavee, Siddharama Pawate, Joseph R. Berger, Allen Jr. Aksamit, Allan Krumholz, Carlos A. Pardo, David R. Moller, Marc A. Judson, Marjolein Drent, Robert P. Baughman

Research output: Contribution to journalArticle

17 Citations (Scopus)

Abstract

Importance: The Neurosarcoidosis Consortium Consensus Group, an expert panel of physicians experienced in the management of patients with sarcoidosis and neurosarcoidosis, engaged in an iterative process to define neurosarcoidosis and develop a practical diagnostic approach to patients with suspected neurosarcoidosis. This panel aimed to develop a consensus clinical definition of neurosarcoidosis to enhance the clinical care of patients with suspected neurosarcoidosis and to encourage standardization of research initiatives that address this disease. Observations: The work of this collaboration included a review of the manifestations of neurosarcoidosis and the establishment of an approach to the diagnosis of this disorder. The proposed consensus diagnostic criteria, which reflect current knowledge, provide definitions for possible, probable, and definite central and peripheral nervous system sarcoidosis. The definitions emphasize the need to evaluate patients with findings suggestive of neurosarcoidosis for alternate causal factors, including infection and malignant neoplasm. Also emphasized is the need for biopsy, whenever feasible and advisable according to clinical context and affected anatomy, of nonneural tissue to document the presence of systemic sarcoidosis and support a diagnosis of probable neurosarcoidosis or of neural tissue to support a diagnosis of definite neurosarcoidosis. Conclusions and Relevance: Diverse disease presentations and lack of specificity of relevant diagnostic tests contribute to diagnostic uncertainty. This uncertainty is compounded by the absence of a pathognomonic histologic tissue examination. The diagnostic criteria we propose are designed to focus investigations on NS as accurately as possible, recognizing that multiple pathophysiologic pathways may lead to the clinical manifestations we currently term NS. Research recognizing the clinical heterogeneity of this diagnosis may open the door to identifying meaningful biologic factors that may ultimately contribute to better treatments.

Original languageEnglish (US)
JournalJAMA Neurology
DOIs
StateAccepted/In press - Jan 1 2018

Fingerprint

Sarcoidosis
Uncertainty
Neurosarcoidosis
Peripheral Nervous System
Biological Factors
Research
Routine Diagnostic Tests
Anatomy
Patient Care
Central Nervous System
Physicians
Biopsy
Infection
Neoplasms
Therapeutics

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Stern, B. J., Royal, W., Gelfand, J. M., Clifford, D. B., Tavee, J., Pawate, S., ... Baughman, R. P. (Accepted/In press). Definition and Consensus Diagnostic Criteria for Neurosarcoidosis: From the Neurosarcoidosis Consortium Consensus Group. JAMA Neurology. https://doi.org/10.1001/jamaneurol.2018.2295

Definition and Consensus Diagnostic Criteria for Neurosarcoidosis : From the Neurosarcoidosis Consortium Consensus Group. / Stern, Barney J.; Royal, Walter; Gelfand, Jeffrey M.; Clifford, David B.; Tavee, Jinny; Pawate, Siddharama; Berger, Joseph R.; Aksamit, Allen Jr.; Krumholz, Allan; Pardo, Carlos A.; Moller, David R.; Judson, Marc A.; Drent, Marjolein; Baughman, Robert P.

In: JAMA Neurology, 01.01.2018.

Research output: Contribution to journalArticle

Stern, BJ, Royal, W, Gelfand, JM, Clifford, DB, Tavee, J, Pawate, S, Berger, JR, Aksamit, AJ, Krumholz, A, Pardo, CA, Moller, DR, Judson, MA, Drent, M & Baughman, RP 2018, 'Definition and Consensus Diagnostic Criteria for Neurosarcoidosis: From the Neurosarcoidosis Consortium Consensus Group', JAMA Neurology. https://doi.org/10.1001/jamaneurol.2018.2295
Stern, Barney J. ; Royal, Walter ; Gelfand, Jeffrey M. ; Clifford, David B. ; Tavee, Jinny ; Pawate, Siddharama ; Berger, Joseph R. ; Aksamit, Allen Jr. ; Krumholz, Allan ; Pardo, Carlos A. ; Moller, David R. ; Judson, Marc A. ; Drent, Marjolein ; Baughman, Robert P. / Definition and Consensus Diagnostic Criteria for Neurosarcoidosis : From the Neurosarcoidosis Consortium Consensus Group. In: JAMA Neurology. 2018.
@article{cdf90e8b571d4c6da8c86d79851c5bc7,
title = "Definition and Consensus Diagnostic Criteria for Neurosarcoidosis: From the Neurosarcoidosis Consortium Consensus Group",
abstract = "Importance: The Neurosarcoidosis Consortium Consensus Group, an expert panel of physicians experienced in the management of patients with sarcoidosis and neurosarcoidosis, engaged in an iterative process to define neurosarcoidosis and develop a practical diagnostic approach to patients with suspected neurosarcoidosis. This panel aimed to develop a consensus clinical definition of neurosarcoidosis to enhance the clinical care of patients with suspected neurosarcoidosis and to encourage standardization of research initiatives that address this disease. Observations: The work of this collaboration included a review of the manifestations of neurosarcoidosis and the establishment of an approach to the diagnosis of this disorder. The proposed consensus diagnostic criteria, which reflect current knowledge, provide definitions for possible, probable, and definite central and peripheral nervous system sarcoidosis. The definitions emphasize the need to evaluate patients with findings suggestive of neurosarcoidosis for alternate causal factors, including infection and malignant neoplasm. Also emphasized is the need for biopsy, whenever feasible and advisable according to clinical context and affected anatomy, of nonneural tissue to document the presence of systemic sarcoidosis and support a diagnosis of probable neurosarcoidosis or of neural tissue to support a diagnosis of definite neurosarcoidosis. Conclusions and Relevance: Diverse disease presentations and lack of specificity of relevant diagnostic tests contribute to diagnostic uncertainty. This uncertainty is compounded by the absence of a pathognomonic histologic tissue examination. The diagnostic criteria we propose are designed to focus investigations on NS as accurately as possible, recognizing that multiple pathophysiologic pathways may lead to the clinical manifestations we currently term NS. Research recognizing the clinical heterogeneity of this diagnosis may open the door to identifying meaningful biologic factors that may ultimately contribute to better treatments.",
author = "Stern, {Barney J.} and Walter Royal and Gelfand, {Jeffrey M.} and Clifford, {David B.} and Jinny Tavee and Siddharama Pawate and Berger, {Joseph R.} and Aksamit, {Allen Jr.} and Allan Krumholz and Pardo, {Carlos A.} and Moller, {David R.} and Judson, {Marc A.} and Marjolein Drent and Baughman, {Robert P.}",
year = "2018",
month = "1",
day = "1",
doi = "10.1001/jamaneurol.2018.2295",
language = "English (US)",
journal = "JAMA Neurology",
issn = "2168-6149",
publisher = "American Medical Association",

}

TY - JOUR

T1 - Definition and Consensus Diagnostic Criteria for Neurosarcoidosis

T2 - From the Neurosarcoidosis Consortium Consensus Group

AU - Stern, Barney J.

AU - Royal, Walter

AU - Gelfand, Jeffrey M.

AU - Clifford, David B.

AU - Tavee, Jinny

AU - Pawate, Siddharama

AU - Berger, Joseph R.

AU - Aksamit, Allen Jr.

AU - Krumholz, Allan

AU - Pardo, Carlos A.

AU - Moller, David R.

AU - Judson, Marc A.

AU - Drent, Marjolein

AU - Baughman, Robert P.

PY - 2018/1/1

Y1 - 2018/1/1

N2 - Importance: The Neurosarcoidosis Consortium Consensus Group, an expert panel of physicians experienced in the management of patients with sarcoidosis and neurosarcoidosis, engaged in an iterative process to define neurosarcoidosis and develop a practical diagnostic approach to patients with suspected neurosarcoidosis. This panel aimed to develop a consensus clinical definition of neurosarcoidosis to enhance the clinical care of patients with suspected neurosarcoidosis and to encourage standardization of research initiatives that address this disease. Observations: The work of this collaboration included a review of the manifestations of neurosarcoidosis and the establishment of an approach to the diagnosis of this disorder. The proposed consensus diagnostic criteria, which reflect current knowledge, provide definitions for possible, probable, and definite central and peripheral nervous system sarcoidosis. The definitions emphasize the need to evaluate patients with findings suggestive of neurosarcoidosis for alternate causal factors, including infection and malignant neoplasm. Also emphasized is the need for biopsy, whenever feasible and advisable according to clinical context and affected anatomy, of nonneural tissue to document the presence of systemic sarcoidosis and support a diagnosis of probable neurosarcoidosis or of neural tissue to support a diagnosis of definite neurosarcoidosis. Conclusions and Relevance: Diverse disease presentations and lack of specificity of relevant diagnostic tests contribute to diagnostic uncertainty. This uncertainty is compounded by the absence of a pathognomonic histologic tissue examination. The diagnostic criteria we propose are designed to focus investigations on NS as accurately as possible, recognizing that multiple pathophysiologic pathways may lead to the clinical manifestations we currently term NS. Research recognizing the clinical heterogeneity of this diagnosis may open the door to identifying meaningful biologic factors that may ultimately contribute to better treatments.

AB - Importance: The Neurosarcoidosis Consortium Consensus Group, an expert panel of physicians experienced in the management of patients with sarcoidosis and neurosarcoidosis, engaged in an iterative process to define neurosarcoidosis and develop a practical diagnostic approach to patients with suspected neurosarcoidosis. This panel aimed to develop a consensus clinical definition of neurosarcoidosis to enhance the clinical care of patients with suspected neurosarcoidosis and to encourage standardization of research initiatives that address this disease. Observations: The work of this collaboration included a review of the manifestations of neurosarcoidosis and the establishment of an approach to the diagnosis of this disorder. The proposed consensus diagnostic criteria, which reflect current knowledge, provide definitions for possible, probable, and definite central and peripheral nervous system sarcoidosis. The definitions emphasize the need to evaluate patients with findings suggestive of neurosarcoidosis for alternate causal factors, including infection and malignant neoplasm. Also emphasized is the need for biopsy, whenever feasible and advisable according to clinical context and affected anatomy, of nonneural tissue to document the presence of systemic sarcoidosis and support a diagnosis of probable neurosarcoidosis or of neural tissue to support a diagnosis of definite neurosarcoidosis. Conclusions and Relevance: Diverse disease presentations and lack of specificity of relevant diagnostic tests contribute to diagnostic uncertainty. This uncertainty is compounded by the absence of a pathognomonic histologic tissue examination. The diagnostic criteria we propose are designed to focus investigations on NS as accurately as possible, recognizing that multiple pathophysiologic pathways may lead to the clinical manifestations we currently term NS. Research recognizing the clinical heterogeneity of this diagnosis may open the door to identifying meaningful biologic factors that may ultimately contribute to better treatments.

UR - http://www.scopus.com/inward/record.url?scp=85052945216&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85052945216&partnerID=8YFLogxK

U2 - 10.1001/jamaneurol.2018.2295

DO - 10.1001/jamaneurol.2018.2295

M3 - Article

C2 - 30167654

AN - SCOPUS:85052945216

JO - JAMA Neurology

JF - JAMA Neurology

SN - 2168-6149

ER -