Jump to content

Calcium pyrophosphate dihydrate crystal deposition disease

From Wikipedia, the free encyclopedia

This is an old revision of this page, as edited by Achromatic (talk | contribs) at 07:10, 31 December 2009 (Tagged). The present address (URL) is a permanent link to this revision, which may differ significantly from the current revision.

Calcium pyrophosphate dihydrate crystal deposition disease
Calcium pyrophosphate

Calcium pyrophosphate dihydrate disease (CPPD) is a rheumatologic disorder with varied clinical manifestations due to precipitation of calcium pyrophosphate dihydrate crystals in the connective tissues. It is more commonly known by alternative names that specify certain clinical or radiographic findings, although neither is synonymous with CPPD. Pseudogout refers to the clinically evident acute synovitis with red, tender, and swollen joints that may resemble gouty arthritis (a similar condition with joint deposition of monosodium urate crystals). Chondrocalcinosis[1][2], on the other hand, refers to the radiographic evidence of calcification in hyaline and/or fibrocartilage. Pyrophosphate arthropathy is a term that may refer to either of the above.[3] Statistically, the knee joint is the most commonly affected.[1]

Allgemein

CPPD crystal deposition disease is a polyarticular arthritis (i.e. it leads to an inflammation of several joints in the body), although it can initially present as monoarticular (i.e. confined to just one joint).[4] CPPD crystals tend to form within articular tissues.[4] Although, in theory, any joint may be affected, the knees, wrists, and hips are the statistically the most commonly attributed areas.[2] In rare cases, pseudogout may affect the spinal canal and cause myelopathy.[5] The exact pathology of CPPD is unknown, although increased adenosine triphosphate (the molecule used as energy currency in all animals) breakdown, which results in increased pyrophosphate levels in joints, is thought to be a method of crystal development.[2] There is some recent evidence suggesting that the gene ANKH is involved in crystal-related inflammatory reactions and inorganic phosphate transport.[2] Excessive calcium (due to hypomagnesemia) has a potential relationship with chondrocalcinosis, and magnesium supplementation may reduce or alleviate symptoms.[6]

Another locus has been described at 8q.[7]

Signs and symptoms

Patients usually present with inflammation of one or more joints often resulting in pain in the affected joint(s).[2] Hyperparathyroidism, hemochromatosis, hypophosphatemia and renal osteodystrophy are often also associated with chondrocalcinosis.[2] Other risk factors for chondrocalcinosis are Wilson's disease and osteoarthritis. In some cases, traumatic arthritis has resulted in chondrocalcinosis.[2][4] In general, the white blood cell count is raised[2]. Rarely, patients may also present with signs of carpal tunnel syndrome.[2]

Diagnosis

Radiography has a large role to play in the diagnosis of chondrocalcinosis with radiographs, CT scans, MRIs, ultrasound and nuclear medicine all having a part.[1] CT scans and MRIs show calcific masses (usually within the ligamentum flavum or joint capsule) however radiography is more successful.[1] As with most conditions, chondrocalcinosis can present with similarity to other diseases such as ankylosing spondylitis or gout.[1][2] Arthrocentesis, or removing synovial fluid from the affected joint, is performed to test the synovial fluid for the calcium pyrophosphate crystals that are present in CPDD

Treatment

Treatment for asymptomatic chondrocalcinosis is not advised to prevent end-organ damage.[2] For acute pseudogout, intra-articular corticosteroid injection, systemic corticosteroids, non-steroidal anti-inflammatory drugs (NSAIDs), or occasionally, high-dose colchicine.[2] NSAIDs are generally administered in low doses to help prevent chondrocalcinosis, however if an acute attack is already occurring, higher doses are administered.[2] Research into surgical removal of calcifications is underway, however this still remains an experimental procedure.[2]

Epidemiology

All cultural races are affected by CPPD, and in the United States around 50% of the population over 85 years of age are affected.[2] Morbidity is the primary result of CPPD, although mortality is never experienced from the disease itself.[2] Females are at a slightly larger risk than men, with an estimated ratio of occurrence of 1.4:1 respectively.[2]

References

  1. ^ a b c d e Rothschild, Bruce M. "Calcium Pyrophosphate Deposition Disease (radiology)". Retrieved 2008-02-18.
  2. ^ a b c d e f g h i j k l m n o p q Rothschild, Bruce M. "Calcium Pyrophosphate Deposition Disease (rheumatology)". Retrieved 2008-02-18.
  3. ^ Longmore, Murray (2007). Oxford Handbook of Clinicial Medicine. Oxford. p. 841. ISBN 0-19-856837-1. {{cite book}}: Unknown parameter |coauthors= ignored (|author= suggested) (help)
  4. ^ a b c Wright GD, Doherty M (1997). "Calcium pyrophosphate crystal deposition is not always 'wear and tear' or aging". Ann. Rheum. Dis. 56 (10): 586–8. doi:10.1136/ard.56.10.586. PMID 9389218.
  5. ^ Walid M S, Yelverton JC, Ajjan M, Grigorian AA. (2008). "Pseudogout of the thoracic spine mimicking a tumor". Russian Neurosurgery Online. 1 (20).{{cite journal}}: CS1 maint: multiple names: authors list (link)
  6. ^ de Filippi JP, Diderich PP, Wouters JM. (1992). "Hypomagnesemia and chondrocalcinosis". Ned Tijdschr Geneeskd. 1 (20).{{cite journal}}: CS1 maint: multiple names: authors list (link)
  7. ^ Baldwin CT, Farrer LA, Adair R, Dharmavaram R, Jimenez S, Anderson L (1995). "Linkage of early-onset osteoarthritis and chondrocalcinosis to human chromosome 8q". Am. J. Hum. Genet. 56 (3): 692–7. PMC 1801178. PMID 7887424. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
Retrieved from "https://en.wikipedia.org/w/index.php?title=Calcium_pyrophosphate_dihydrate_crystal_deposition_disease&oldid=335067656"