Macrophage clustering as a diagnostic marker in sural nerve biopsies of patients with CIDP

Neurology. 2005 Dec 27;65(12):1924-9. doi: 10.1212/01.wnl.0000188879.19900.b7.


Background: In adult patients with a slowly progressive demyelinating neuropathy, it may be difficult to distinguish between a hereditary neuropathy and chronic inflammatory demyelinating polyneuropathy (CIDP). The authors previously observed clustering of macrophages around endoneurial blood vessels in sural nerve biopsies from patients with CIDP.

Objectives: To quantitate macrophage clustering around endoneurial blood vessels in CIDP vs hereditary neuropathies.

Methods: The authors studied 21 patients with CIDP, 18 patients with hereditary neuropathies, and 5 normal sural nerves. Numbers of macrophages, T-cells, and blood vessels were counted after immunohistochemical staining. The presence of three or more macrophages around one blood vessel was defined as a cluster. In a subsequent validation analysis, 65 stored biopsy specimens obtained from patients with a chronic neuropathy were re-evaluated for perivascular macrophage clustering according to criteria derived from the quantitative analysis of the first 221 biopsies in a blinded fashion.

Results: The percentage of endoneurial vessels with macrophage clusters was higher in CIDP than in hereditary neuropathies (CIDP median = 9.4, range 0 to 48; hereditary NP median = 0, range 0 to 7.7; p < 0.001). The evaluation of the 65 further biopsies showed that the presence of one perivascular macrophage cluster per fascicle proved to be a valid criterion to differentiate between inflammatory and other forms of neuropathy (chi2 test p = 0.0000025, sensitivity 75%, specificity 72%).

Conclusion: The presence of clusters of macrophages around endoneurial vessels in sural nerve biopsies may serve as a useful additional marker for establishing the pathologic diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP).

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Biomarkers
  • Biopsy
  • Blood Vessels / immunology
  • Blood Vessels / pathology
  • Blood Vessels / physiopathology
  • Diagnosis, Differential
  • Female
  • Hereditary Sensory and Autonomic Neuropathies / diagnosis
  • Humans
  • Macrophages / immunology
  • Macrophages / pathology*
  • Male
  • Microcirculation / immunology
  • Microcirculation / pathology
  • Microcirculation / physiopathology
  • Middle Aged
  • Observer Variation
  • Peripheral Nerves / blood supply
  • Peripheral Nerves / pathology
  • Peripheral Nerves / physiopathology
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / diagnosis*
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / immunology
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / physiopathology
  • Predictive Value of Tests
  • Reproducibility of Results
  • Sural Nerve / blood supply
  • Sural Nerve / pathology*
  • Sural Nerve / physiopathology*


  • Biomarkers