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Chondrocalcinosis 2(CCAL2; CPPDD; CPPDD2)

MedGen UID:
163633
Concept ID:
C0856830
Disease or Syndrome
Synonyms: Calcium gout; Calcium gout, familial; Calcium pyrophosphate arthropathy; Calcium pyrophosphate arthropathy, familial; Calcium pyrophosphate dihydrate deposition disease; Chondrocalcinosis familial articular; Familial calcium pyrophosphate deposition; Pseudogout, familial
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Gene (location): ANKH (5p15.2)
 
Monarch Initiative: MONDO:0007319
OMIM®: 118600
Orphanet: ORPHA1416

Definition

Chondrocalcinosis, or cartilage calcification, is a common condition that usually results from deposition of crystals of calcium pyrophosphate dihydrate (CPPD) in articular hyaline and fibro-cartilage. CPPD crystal deposition may be asymptomatic or associated with characteristic acute attacks ('pseudogout') or chronic arthritis. It can be detected radiographically. Chondrocalcinosis occurs in 3 forms: a primary hereditary form (e.g., CCAL2); a form associated with metabolic disorders (e.g., hyperparathyroidism, hemochromatosis, and hypomagnesemia); and a sporadic form, which may in some cases represent the hereditary form (summary by Hughes et al., 1995 and Richette et al., 2009). Genetic Heterogeneity of Chondrocalcinosis Another form of chondrocalcinosis (CCAL1; 600668) has been mapped to chromosome 8q. [from OMIM]

Clinical features

From HPO
Arthropathy
MedGen UID:
7190
Concept ID:
C0022408
Disease or Syndrome
Any disorder of the joints.
Osteoarthritis
MedGen UID:
45244
Concept ID:
C0029408
Disease or Syndrome
Osteoarthritis (OA) is a degenerative disease of the joints characterized by degradation of the hyaline articular cartilage and remodeling of the subchondral bone with sclerosis (Meulenbelt et al., 2006). Clinical problems include pain and joint stiffness often leading to significant disability and joint replacement. Osteoarthritis exhibits a clear predilection for specific joints; it appears most commonly in the hip and knee joints and lumbar and cervical spine, as well as in the distal interphalangeal and the first carpometacarpal (base of thumb) and proximal interphalangeal joints of the hand; however, patients with osteoarthritis may have 1, a few, or all of these sites affected (Stefansson et al., 2003). According to a conservative estimate, greater than 70% of the population of the United States at age 65 years is affected by the disease, reflecting its age dependence. Genetic Heterogeneity of Susceptibility to Osteoarthritis Susceptibility to osteoarthritis has been associated with variation in other genes: OS2 (140600) with variation in the MATN3 gene (602109) on chromosome 2p24; OS3 (607850) with variation in the ASPN gene (608135) on chromosome 9q22; and OS5 (612400) with variation in the GDF5 gene (601146) on chromosome 20q11. Other susceptibility loci for osteoarthritis have been mapped to chromosomes 2q33 (OS4; 610839) and 3p24 (OS6; 612401).
Polyarticular chondrocalcinosis
MedGen UID:
870801
Concept ID:
C4025258
Disease or Syndrome

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChondrocalcinosis 2

Professional guidelines

PubMed

Rosenthal AK, Ryan LM
Rheum Dis Clin North Am 2014 May;40(2):343-56. Epub 2014 Feb 19 doi: 10.1016/j.rdc.2014.01.010. PMID: 24703351Free PMC Article

Recent clinical studies

Etiology

Nassir M, Arad U, Lee SY, Journo S, Mirza S, Renn C, Zimmermann H, Pelletier J, Sévigny J, Müller CE, Fischer B
Purinergic Signal 2019 Jun;15(2):247-263. Epub 2019 Apr 25 doi: 10.1007/s11302-019-09649-2. PMID: 31025169Free PMC Article
Vele P, Simon SP, Damian L, Felea I, Muntean L, Filipescu I, Rednic S
Med Ultrason 2018 May 2;20(2):159-163. doi: 10.11152/mu-1193. PMID: 29730681
Di Matteo A, Filippucci E, Salaffi F, Carotti M, Carboni D, Di Donato E, Grassi W
Clin Exp Rheumatol 2017 Jul-Aug;35(4):647-652. Epub 2017 Mar 23 PMID: 28339356
Carpenter L, Juliano N, Herb R
J Am Osteopath Assoc 2017 Mar 1;117(3):199. doi: 10.7556/jaoa.2017.036. PMID: 28241333
Löffler C, Sattler H, Peters L, Löffler U, Uppenkamp M, Bergner R
J Rheumatol 2015 Mar;42(3):513-20. Epub 2014 Nov 15 doi: 10.3899/jrheum.140634. PMID: 25399385

Diagnosis

Tamborrini G, Hügle T, Ricci V, Filippou G
Reumatismo 2023 Dec 19;75(4) doi: 10.4081/reumatismo.2023.1583. PMID: 38115778
Omoumi P, Zufferey P, Malghem J, So A
Rheum Dis Clin North Am 2016 Nov;42(4):621-644. Epub 2016 Sep 9 doi: 10.1016/j.rdc.2016.07.005. PMID: 27742018
Miksanek J, Rosenthal AK
Curr Rheumatol Rep 2015 Mar;17(3):20. doi: 10.1007/s11926-015-0496-1. PMID: 25761927Free PMC Article
McQueen FM, Doyle A, Dalbeth N
Rheum Dis Clin North Am 2014 May;40(2):231-49. Epub 2014 Feb 20 doi: 10.1016/j.rdc.2014.01.004. PMID: 24703345
Filippucci E, Di Geso L, Girolimetti R, Grassi W
Clin Exp Rheumatol 2014 Jan-Feb;32(1 Suppl 80):S42-7. Epub 2014 Feb 17 PMID: 24528621

Therapy

Carpenter L, Juliano N, Herb R
J Am Osteopath Assoc 2017 Mar 1;117(3):199. doi: 10.7556/jaoa.2017.036. PMID: 28241333
Rosenthal AK, Ryan LM
Rheum Dis Clin North Am 2014 May;40(2):343-56. Epub 2014 Feb 19 doi: 10.1016/j.rdc.2014.01.010. PMID: 24703351Free PMC Article
Ottaviani S, Brunier L, Sibilia J, Maurier F, Ardizzone M, Wendling D, Gill G, Palazzo E, Meyer O, Dieudé P
Joint Bone Spine 2013 Mar;80(2):178-82. Epub 2012 Sep 28 doi: 10.1016/j.jbspin.2012.07.018. PMID: 23022422
Beggs AE, Reeves DJ, Noel NS
Am J Health Syst Pharm 2012 Dec 15;69(24):2147-8. doi: 10.2146/ajhp120330. PMID: 23230037
Bindi P, Lavaud S, Bernieh B, Toupance O, Chanard J
Nephrol Dial Transplant 1990;5(3):199-203. doi: 10.1093/ndt/5.3.199. PMID: 2113647

Prognosis

Carpenter L, Juliano N, Herb R
J Am Osteopath Assoc 2017 Mar 1;117(3):199. doi: 10.7556/jaoa.2017.036. PMID: 28241333
Löffler C, Sattler H, Peters L, Löffler U, Uppenkamp M, Bergner R
J Rheumatol 2015 Mar;42(3):513-20. Epub 2014 Nov 15 doi: 10.3899/jrheum.140634. PMID: 25399385
McQueen FM, Doyle A, Dalbeth N
Rheum Dis Clin North Am 2014 May;40(2):231-49. Epub 2014 Feb 20 doi: 10.1016/j.rdc.2014.01.004. PMID: 24703345
Filippucci E, Di Geso L, Girolimetti R, Grassi W
Clin Exp Rheumatol 2014 Jan-Feb;32(1 Suppl 80):S42-7. Epub 2014 Feb 17 PMID: 24528621
Bindi P, Lavaud S, Bernieh B, Toupance O, Chanard J
Nephrol Dial Transplant 1990;5(3):199-203. doi: 10.1093/ndt/5.3.199. PMID: 2113647

Clinical prediction guides

Di Matteo A, Filippucci E, Salaffi F, Carotti M, Carboni D, Di Donato E, Grassi W
Clin Exp Rheumatol 2017 Jul-Aug;35(4):647-652. Epub 2017 Mar 23 PMID: 28339356
Löffler C, Sattler H, Peters L, Löffler U, Uppenkamp M, Bergner R
J Rheumatol 2015 Mar;42(3):513-20. Epub 2014 Nov 15 doi: 10.3899/jrheum.140634. PMID: 25399385
McQueen FM, Doyle A, Dalbeth N
Rheum Dis Clin North Am 2014 May;40(2):231-49. Epub 2014 Feb 20 doi: 10.1016/j.rdc.2014.01.004. PMID: 24703345
Filippucci E, Di Geso L, Girolimetti R, Grassi W
Clin Exp Rheumatol 2014 Jan-Feb;32(1 Suppl 80):S42-7. Epub 2014 Feb 17 PMID: 24528621
Filippucci E, Delle Sedie A, Riente L, Di Geso L, Carli L, Ceccarelli F, Sakellariou G, Iagnocco A, Grassi W
Clin Exp Rheumatol 2013 Sep-Oct;31(5):659-64. Epub 2013 Sep 18 PMID: 24050142

Recent systematic reviews

Filippou G, Adinolfi A, Iagnocco A, Filippucci E, Cimmino MA, Bertoldi I, Di Sabatino V, Picerno V, Delle Sedie A, Sconfienza LM, Frediani B, Scirè CA
Osteoarthritis Cartilage 2016 Jun;24(6):973-81. Epub 2016 Jan 27 doi: 10.1016/j.joca.2016.01.136. PMID: 26826301

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