Sarcoid neuropathy

Jinny O Tavee MD (Dr. Tavee of the Cleveland Clinic Foundation has no relevant financial relationships to disclose.)
Louis H Weimer MD, editor. (Dr. Weimer of Columbia University has received consulting fees from Roche.)
Originally released April 23, 1996; last updated July 24, 2016; expires July 24, 2019

This article includes discussion of sarcoid neuropathy and sarcoidosis small fiber neuropathy. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

The author reviews current concepts of sarcoidosis-associated neuropathies. Clinical features, diagnostic considerations, and management strategies are discussed. In particular, the recent literature on sarcoidosis-associated small fiber neuropathy and possible new target-directed treatment options are highlighted. Also, concepts regarding the use of immunomodulatory agents are highlighted.

Key points

 

• Sarcoidosis can involve the peripheral nervous system in a variety of ways.

 

• Patients can have a motor, sensory, sensorimotor, and/or autonomic neuropathy; the neuropathy can be primarily axonal or demyelinating.

 

• Patients can have involvement of isolated peripheral nerves, such as a carpal tunnel syndrome, or generalized involvement.

 

• Sarcoidosis is associated with virtually all peripheral neuropathy subtypes, eg, large fiber sensorimotor, pure motor, etc.

 

• Small fiber sensory neuropathy is the most common peripheral nerve manifestation and is seen in up to a third of patients with systemic disease.

 

• Small fiber sensory neuropathy can be associated with autonomic dysfunction.

 

• Epidermal nerve fiber density analysis and autonomic testing provide objective confirmation for the diagnosis of small fiber neuropathy.

 

• Sarcoidosis-associated neuropathies may respond to immunosuppressive medications.

Historical note and terminology

Sarcoidosis was first described by Sir Jonathan Hutchinson more than a century ago (Hutchinson 1898; Burns 2003). The term "sarcoidosis" is derived from "sarkoid," used by Caesar Boeck at the turn of the twentieth century (Boeck 1899). Neurologic manifestations of sarcoidosis were reviewed as early as 1935 by Levin (Levin 1935). Peripheral nerve involvement with sarcoidosis was first recorded by Winkler and Mazza (Winkler 1905; Mazza 1908).

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