Table 2.

ECD organ involvement in NIH patients and in the literature

Organ system and clinical findingsNIH patients,* n (%)Veyssier-Belot et al (%)Haroche et al (%)
Bone57 (95)NR96
Kidney39 (65)2768
Periaortic encasement37 (62)NR66
Hypogonadism36 (60)NRNR
Lung31 (52)1443
Bone pain28 (47)4740
Maxilla and mandible§24 (47)NRNR
Diabetes insipidus28 (47)2925
CNS disease infiltration23 (38)1751
Retro-orbital area ± exophthalmos15 (27)2925
Heart (pseudotumor in RA)22 (37)NR19
Xanthelasma20 (33)1928
Skin15 (25)10NR
Pericardial disease5 (8)742
  • CNS, central nervous system; NR, not reported; RA, right atrioventricular groove.

  • * Number of patients out of the 60 patients prospectively investigated.

  • Data included 59 cases (1996).3

  • Data included 122 historical cases (2014).4

  • § Panoramic dental imaging was performed in 51 cases.