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Chronic multifocal osteomyelitis(CMO; CRMO3)

MedGen UID:
140822
Concept ID:
C0410422
Disease or Syndrome
Synonym: Chronic recurrent multifocal osteomyelitis
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Related genes: LPIN2, IL1RN, IL1R1
 
Monarch Initiative: MONDO:0009813
OMIM®: 147810; 259680
OMIM® Phenotypic series: PS609628
Orphanet: ORPHA324964

Definition

Chronic recurrent multifocal osteomyelitis-3 (CRMO3) is an autosomal dominant autoinflammatory bone disease characterized by early childhood onset of bone pain and arthritis caused by sterile osteomyelitis. The disorder results from constitutive activation of the IL1-mediated inflammatory pathway due to loss of IL1 receptor sensitivity to its antagonist IL1RN (147679). et al. (2023) suggested the term 'Loss of IL1R1 Sensitivity to IL1RA (IL1RN)' or 'LIRSA' as a designation for this disorder. For a discussion of genetic heterogeneity of CRMO, see 609628. [from OMIM]

Clinical features

From HPO
Bone pain
MedGen UID:
57489
Concept ID:
C0151825
Sign or Symptom
An unpleasant sensation characterized by physical discomfort (such as pricking, throbbing, or aching) localized to bone.
Osteomyelitis
MedGen UID:
10497
Concept ID:
C0029443
Disease or Syndrome
Osteomyelitis is an inflammatory process accompanied by bone destruction and caused by an infecting microorganism.
Palmoplantar pustulosis
MedGen UID:
45290
Concept ID:
C0030246
Disease or Syndrome
A chronic, relapsing, pustular eruption that is localized to the palms and soles.
Fever
MedGen UID:
5169
Concept ID:
C0015967
Sign or Symptom
Body temperature elevated above the normal range.
Elevated erythrocyte sedimentation rate
MedGen UID:
57727
Concept ID:
C0151632
Finding
An increased erythrocyte sedimentation rate (ESR). The ESR is a test that measures the distance that erythrocytes have fallen after one hour in a vertical column of anticoagulated blood under the influence of gravity. The ESR is a nonspecific finding. An elevation may indicate inflammation or may be caused by any condition that elevates fibrinogen.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
Follow this link to review classifications for Chronic multifocal osteomyelitis in Orphanet.

Professional guidelines

PubMed

Hofmann SR, Schnabel A, Rösen-Wolff A, Morbach H, Girschick HJ, Hedrich CM
J Rheumatol 2016 Nov;43(11):1956-1964. Epub 2016 Sep 1 doi: 10.3899/jrheum.160256. PMID: 27585682
Cole WG
Clin Orthop Relat Res 1991 Mar;(264):84-9. PMID: 1997255

Recent clinical studies

Etiology

Rausch P, Hartmann M, Baines JF, von Bismarck P
Arthritis Res Ther 2022 Feb 22;24(1):54. doi: 10.1186/s13075-021-02711-8. PMID: 35193655Free PMC Article
Ferguson PJ, Laxer RM
Semin Immunopathol 2015 Jul;37(4):407-12. Epub 2015 Apr 17 doi: 10.1007/s00281-015-0488-2. PMID: 25894861
Golla A, Jansson A, Ramser J, Hellebrand H, Zahn R, Meitinger T, Belohradsky BH, Meindl A
Eur J Hum Genet 2002 Mar;10(3):217-21. doi: 10.1038/sj.ejhg.5200789. PMID: 11973628
Ehrenborg C, Wesslén L, Jakobson A, Friman G, Holmberg M
J Clin Microbiol 2000 Feb;38(2):682-7. doi: 10.1128/JCM.38.2.682-687.2000. PMID: 10655367Free PMC Article
Cole WG
Clin Orthop Relat Res 1991 Mar;(264):84-9. PMID: 1997255

Diagnosis

Rausch P, Hartmann M, Baines JF, von Bismarck P
Arthritis Res Ther 2022 Feb 22;24(1):54. doi: 10.1186/s13075-021-02711-8. PMID: 35193655Free PMC Article
Alamoudi A, Selvam NP, Kashtwari D, Ruprecht A, Hansen M
J Clin Pediatr Dent 2021 Oct 1;45(4):273-277. doi: 10.17796/1053-4625-45.4.10. PMID: 34534306
Cimolai N
J Infect Public Health 2011 Sep;4(4):157-68. doi: 10.1016/j.jiph.2011.05.001. PMID: 22000842
Carr AJ, Cole WG, Roberton DM, Chow CW
J Bone Joint Surg Br 1993 Jul;75(4):582-91. doi: 10.1302/0301-620X.75B4.8331113. PMID: 8331113
Cole WG
Clin Orthop Relat Res 1991 Mar;(264):84-9. PMID: 1997255

Therapy

Alamoudi A, Selvam NP, Kashtwari D, Ruprecht A, Hansen M
J Clin Pediatr Dent 2021 Oct 1;45(4):273-277. doi: 10.17796/1053-4625-45.4.10. PMID: 34534306
Shamriz O, Engelhard D, Rajs AP, Kaidar-Shwartz H, Casanova JL, Averbuch D
Pediatr Infect Dis J 2013 Dec;32(12):1345-7. doi: 10.1097/01.inf.0000437067.43859.4c. PMID: 24569307

Prognosis

Alamoudi A, Selvam NP, Kashtwari D, Ruprecht A, Hansen M
J Clin Pediatr Dent 2021 Oct 1;45(4):273-277. doi: 10.17796/1053-4625-45.4.10. PMID: 34534306
Cimolai N
J Infect Public Health 2011 Sep;4(4):157-68. doi: 10.1016/j.jiph.2011.05.001. PMID: 22000842
Stewart A, Carneiro R, Pollock L, Shaw D
Skeletal Radiol 1994 Apr;23(3):225-7. doi: 10.1007/BF00197468. PMID: 8016677
Carr AJ, Cole WG, Roberton DM, Chow CW
J Bone Joint Surg Br 1993 Jul;75(4):582-91. doi: 10.1302/0301-620X.75B4.8331113. PMID: 8331113

Clinical prediction guides

Rausch P, Hartmann M, Baines JF, von Bismarck P
Arthritis Res Ther 2022 Feb 22;24(1):54. doi: 10.1186/s13075-021-02711-8. PMID: 35193655Free PMC Article

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