eMedicine Specialties > Neurology > Neuromuscular Diseases
Sarcoidosis and Neuropathy: Treatment & Medication
Updated: Mar 8, 2007
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
Several treatment regimens have been proposed. However, no definitive treatment exists.
- Corticosteroids remain the standard treatment.
- Immunosuppressants such as cyclosporine, methotrexate, and cyclophosphamide have been used with varying results. Almost all of the studies completed to date have involved treatment of CNS sarcoidosis as opposed to peripheral neuropathy. A case report of a biopsy-proven axonal sensorimotor polyneuropathy responding to intravenous immunoglobulin while unresponsive to steroid therapy is described.
Medication
Medications used in peripheral neuropathy in sarcoidosis are the same as those used for systemic sarcoidosis and neurosarcoidosis. Immunosuppressants are used to dampen or alter the inflammatory activity. Corticosteroids are preferred. Nonresponders may be tried on cyclosporine, azathioprine, and/or methotrexate.
Immunosuppressants
Corticosteroids alter the immune response and may lead to resolution of the granulomas in sarcoidosis.
Prednisone (Orasone, Sterapred, Deltasone)
Most commonly used oral corticosteroid, works by altering immune system and decreasing inflammatory reaction that is responsible for granuloma formation. Tuberculin skin test required prior to commencing high daily dose of steroids. Improvement has been reported in patients with sarcoid polyneuropathy who received methylprednisolone (1 g/wk for 8 wk) when oral prednisone failed. Disagreement exists about optimal treatment dose, but doses listed here are typical.
Often, high dose required for period of 2-4 wk before tapering; taper may need to be continued for several months before discontinuing treatment altogether.
Occasionally, patients respond to methylprednisolone pulses when high-dose oral prednisone fails.
Adult
Starting dose: 1-1.5 mg/kg/d PO; maintain this dose until clinical response seen; taper dose steadily over several months, titrating according to clinical response
Pediatric
1-1.5 mg/kg/d PO; if possible, taper dose more quickly in children because of adverse metabolic and growth retardation effects of steroids
Estrogens may decrease clearance; may increase digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism (consider increasing maintenance dose); patients taking diuretics should be monitored for hypokalemia
Active systemic infection; relative contraindications include congestive cardiac failure, poorly controlled hypertension, poorly controlled diabetes mellitus
Pregnancy
B - Usually safe but benefits must outweigh the risks.
Precautions
Perform monthly random blood glucose and blood pressure checks to identify adverse effects early; patients at risk for multiple complications, including severe infections; abrupt discontinuation of glucocorticoids may cause adrenal crisis; other adverse effects include hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, and growth suppression
Cytotoxic drug/immunosuppressants
These agents suppress the autoimmune response, which is responsible for granuloma formation.
Cyclosporine (Sandimmune, Neoral)
Used extensively in patients who have undergone transplant. Beneficial effects in neurosarcoidosis have been reported, although most clinical scenarios have been central and not peripheral nervous system sarcoidosis. Has been found to have benefit when used as adjunct to steroids in 6 patients with CNS involvement of neurosarcoidosis.
Adult
3-6 mg/kg/d PO
Pediatric
Administer as in adults
Use cautiously with nephrotoxic medications; several medications may increase or decrease levels and availability
Documented hypersensitivity; relative contraindications include poorly controlled hypertension, rapidly rising blood pressure while on drug, rheumatoid arthritis, poor renal function, malignancies, concurrent PUVA or UVB therapy
Pregnancy
B - Usually safe but benefits must outweigh the risks.
Precautions
Closely monitor blood pressure and perform weekly renal function tests (BUN, creatinine) during first month of therapy, then monthly tests after first month
Azathioprine (Imuran)
Cytostatic drug that has been used in numerous immune-mediated diseases. Active component, 6-mercaptopurine, thought to have immune-suppressing properties.
Adult
50 mg/d PO initial dose; increase by 50 mg/d weekly to desired dose
3-5 mg/kg PO usual dose for all age groups
Pediatric
Administer as in adults
Allopurinol increases toxicity; ACE inhibitors may induce severe leukopenia; methotrexate may increase plasma levels of methotrexate metabolite; may decrease effects of anticoagulants; may decrease plasma levels of cyclosporine; may decrease or reverse pharmacologic actions of neuromuscular blockers
Documented hypersensitivity; relative contraindications include known liver disease, bone marrow suppression with cytopenias
Pregnancy
B - Usually safe but benefits must outweigh the risks.
Precautions
Use in pregnancy must be decided after recognizing teratogenic potential of azathioprine; GI or systemic reaction may develop within 2 wk of initiating azathioprine, necessitating discontinuation of medication
Methotrexate (Folex, Rheumatrex)
Antimetabolite used as immunosuppressant, often in rheumatoid arthritis, severe psoriasis, and certain neoplastic diseases. Its use for neurosarcoidosis has not been tested sufficiently.
Adult
Dosage varies depending on indication; one patient with CNS neurosarcoidosis has been described as benefiting from 25 mg/wk PO
Starting dose varies from 25-30 mg/d PO; maintenance dose usually 7.5 mg/d
Pediatric
Not established except for cancer therapy
NSAIDs administered concurrently with methotrexate can cause fatal interaction
Oral aminoglycosides may decrease absorption and blood levels; charcoal lowers plasma levels of both oral and IV methotrexate, particularly with high-dose therapies; etretinate may increase hepatotoxicity; folic acid or its derivatives contained in some vitamins may decrease response; indomethacin and phenylbutazone can increase plasma levels, possibly by inhibiting renal prostaglandin synthesis or through competitive renal secretion; may decrease phenytoin serum concentrations; probenecid, salicylates, and sulfonamides, including TMP-SMZ, may increase therapeutic and toxic effects; procarbazine may increase nephrotoxicity; may increase plasma levels of thiopurines
Documented hypersensitivity; liver disease; blood dyscrasias
Pregnancy
X - Contraindicated in pregnancy
Precautions
May have toxic effects on hematologic, renal, gastrointestinal, pulmonary, or neurologic systems; monitor CBC counts monthly, and liver and renal function every 1 to 3 months, during therapy—monitor frequently during initial dosing or when changing doses, also when risk of elevated levels, such as dehydration; stop drug immediately if significant drop in blood counts
Aspirin, NSAIDs, or low-dose steroids may be administered concomitantly with this medication, but possibility of increased toxicity with concomitant NSAIDs, including salicylates, has not been tested
More on Sarcoidosis and Neuropathy |
| Overview: Sarcoidosis and Neuropathy |
| Differential Diagnoses & Workup: Sarcoidosis and Neuropathy |
 Treatment & Medication: Sarcoidosis and Neuropathy |
| Follow-up: Sarcoidosis and Neuropathy |
| References |
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References
Abrey LE, Rosenblum MK, DeAngelis LM. Sarcoidosis of the cauda equina mimicking leptomeningeal malignancy. J Neurooncol. Sep 1998;39(3):261-5. [Medline].
Agbogu BN, Stern BJ, Sewell C, Yang G. Therapeutic considerations in patients with refractory neurosarcoidosis. Arch Neurol. Sep 1995;52(9):875-9. [Medline].
Allen RK, Merory J. Intravenous pulse methyl prednisolone in the successful treatment of severe sarcoid polyneuropathy with pulmonary involvement. Aust N Z J Med. Feb 1985;15(1):45-6. [Medline].
Baughman RP, Lower EE. Newer therapies for cutaneous sarcoidosis: the role of thalidomide and other agents. Am J Clin Dermatol. 2004;5(6):385-94. [Medline].
Brochet B, Louiset P, Lagueny A, et al. [Peripheral neuropathies disclosing sarcoidosis]. Rev Neurol (Paris). 1988;144(10):590-5. [Medline].
Challenor YB, Felton CP, Brust JC. Peripheral nerve involvement in sarcoidosis: an electrodiagnostic study. J Neurol Neurosurg Psychiatry. Nov 1984;47(11):1219-22. [Medline].
Chapelon C, Ziza JM, Piette JC, et al. Neurosarcoidosis: signs, course and treatment in 35 confirmed cases. Medicine (Baltimore). Sep 1990;69(5):261-76. [Medline].
Delaney P. Neurologic manifestations in sarcoidosis: review of the literature, with a report of 23 cases. Ann Intern Med. Sep 1977;87(3):336-45. [Medline].
Dewberry RG, Schneider BF, Cale WF, Phillips LH 2nd. Sarcoid myopathy presenting with diaphragm weakness. Muscle Nerve. Aug 1993;16(8):832-5. [Medline].
Galassi G, Gibertoni M, Mancini A, et al. Sarcoidosis of the peripheral nerve: clinical, electrophysiological and histological study of two cases. Eur Neurol. 1984;23(6):459-65. [Medline].
Heaney D, Geddes JF, Nagendren K, Swash M. Sarcoid polyneuropathy responsive to intravenous immunoglobulin. Muscle Nerve. Mar 2004;29(3):447-50. [Medline].
Koffman B, Junck L, Elias SB, et al. Polyradiculopathy in sarcoidosis. Muscle Nerve. May 1999;22(5):608-13. [Medline].
Mayock RL, Bertrand P, Morrison CE, Scott JH. Manifestations of Sarcoidosis. Analysis of 145 Patients, wirh A Review of Nine Series Selected from the Literature. Am J Med. Jul 1963;35:67-89. [Medline].
Nemni R, Galassi G, Cohen M, et al. Symmetric sarcoid polyneuropathy: analysis of a sural nerve biopsy. Neurology. Oct 1981;31(10):1217-23. [Medline].
Oh SJ. Sarcoid polyneuropathy: a histologically proved case. Ann Neurol. Feb 1980;7(2):178-81. [Medline].
Payne CR, Tait D, Batten JC. Sarcoidosis and chronic sensory neuropathy. Postgrad Med J. Nov 1980;56(661):781-2. [Medline].
Scott TS, Brillman J, Gross JA. Sarcoidosis of the peripheral nervous system. Neurol Res. Dec 1993;15(6):389-90. [Medline].
Soriano FG, Caramelli P, Nitrini R, Rocha AS. Neurosarcoidosis: therapeutic success with methotrexate. Postgrad Med J. Feb 1990;66(772):142-3. [Medline].
Stern B. Neurological complication of sarcoidosis. Neurology and Neurosurgery update series. 1985;37:2-7.
Stern BJ, Krumholz A, Johns C, et al. Sarcoidosis and its neurological manifestations. Arch Neurol. Sep 1985;42(9):909-17. [Medline].
Stern BJ, Schonfeld SA, Sewell C, et al. The treatment of neurosarcoidosis with cyclosporine. Arch Neurol. Oct 1992;49(10):1065-72. [Medline].
Tajima Y, Shinpo K, Itoh Y, et al. Infiltrating cell profiles of sarcoid lesions in the muscles and peripheral nerves: an immunohistological study. Intern Med. Dec 1997;36(12):876-81. [Medline].
Zajicek J. Sarcoidosis of the cauda equina: a report of three cases. J Neurol. Nov 1990;237(7):424-6. [Medline].
Zuniga G, Ropper AH, Frank J. Sarcoid peripheral neuropathy. Neurology. Oct 1991;41(10):1558-61. [Medline].
Further Reading
Keywords
sarcoidosis, neuropathy, granulomas, neurosarcoidosis, sarcoid granulomas, peripheral neuropathy
