Association of small fiber neuropathy with cardiac sympathetic dysfunction in sarcoidosis.
OBJECTIVE
Recently we found that small fiber neuropathy (SFN) occurs frequently in sarcoidosis. Autonomic dysfunction may be a feature of SFN. Since cardiac autonomic dysfunction has been identified as a strong predictor of morbidity and mortality, recognition of cardiac autonomic involvement is of clinical relevance. It was hypothesised that SFN might be related to cardiac sympathetic denervation in sarcoidosis.


METHODS
In the present study 45 consecutive sarcoidosis patients (13 without SFN assessed by thermal threshold testing (TTT), 32 with SFN (abnormal TTT) were enrolled. To rule out bias due to myocardial ischemia, cases with abnormal Thallium (201Tl) perfusion scintigraphy were excluded (n = 2). Cardiovascular autonomic function testing (Ewing tests) and 123I-MIBG (metaiodobenzylguanidine) scintigraphy were used to assess cardiac autonomic function. Further cardiac diagnostic work-up included ECG, Holter recording and echo Doppler cardiography.


RESULTS
Mild to moderate heterogeneity of 123I-MIBG uptake regional in the myocardium was demonstrated in a substantial number of the studied sarcoidosis population, especially in those with SFN (abnormal TTT). Mean inferior-anterior ratios were 0.85+/-0.17 (SFN) and 1.0+/-0.17 (no SFN; p = 0.003), respectively. Four out of the 14 cases with abnormal MIBG scintigraphy and SFN showed an abnormal Ewing test.


CONCLUSIONS
Cardiac sympathetic dysfunction assessed by use of 123I-MIBG myocardial scanning appeared to be heterogeneous in sarcoidosis patients and dependent on the presence or absence of SFN. MIBG scintigraphy may be of additional value in the management and follow-up of sarcoidosis patients. Future study is warranted to explore possible prognostic and therapeutic implications of these findings in sarcoidosis.
Hoitsma
Elske
E
Dept of Neurology, Management Center, University Hospital Maastricht, the Netherlands.
Faber
Carin G
CG
van Kroonenburgh
Marinus J P G
MJ
Gorgels
Anton P M
AP
Halders
Serve G E A
SG
Heidendal
Guido A K
GA
Kessels
Alfons G H
AG
Reulen
Jos P H
JP
Drent
Marjolein
M
D019797:3-Iodobenzylguanidine; D000328:Adult; D001342:Autonomic Nervous System Diseases / Q000000981:diagnostic imaging* / Q000209:etiology*; D016022:Case-Control Studies; D015150:Echocardiography, Doppler; D004562:Electrocardiography; D005260:Female; D006321:Heart / Q000294:innervation; D006331:Heart Diseases / Q000000981:diagnostic imaging* / Q000209:etiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D019275:Radiopharmaceuticals; D012507:Sarcoidosis / Q000150:complications* / Q000000981:diagnostic imaging*; D013794:Thallium Radioisotopes; D015899:Tomography, Emission-Computed, Single-Photon
D019275:Radiopharmaceuticals; D013794:Thallium Radioisotopes; D019797:3-Iodobenzylguanidine