calcium pyrophosphate deposition in hemochromatosis

A 54 year old man gave a two year history of a painful right hip, fingers, and wrists. The importance of iron accumulation in joint tissues is supported by reports of crystal deposition in patients with transfusion hemosiderosis as well as hemophilic arthritis. This article Ann Rheum Dis 2011;70:571-5. 42, No. 16, No. o triangular cartilage of wrist o menisci of knee o symphysis pubis o acromicoclavicular joint … Therefore, investigations for a predisposing metabolic condition are advised in young-onset polyarticular CPPD crystal deposition disease. (9) CPPD can also be associated with tendinitis, tenosynovitis and bursitis. Chondrocalcinosis (literally calcification of the cartilage) is a term used to describe the radiological appearance of calcium pyrophosphate dihydrate crystal deposition within either fibrocartilage (e.g. Abstract Radiographic features of hand and wrist involvement in 26 patients with hemochromatosis and in 26 patients with idiopathic calcium pyrophosphate dihydrate (CPPD) crystal deposition disease were compared. THE CUMULATIVE INDEX TO NURSING AND ALLIED HEALTH LITERATURE (CINAHL PLUS). Hughes AE, McGibbon D, Woodward E, Dixey J, Doherty M. Localisation of a gene for chondrocalcinosis to chromosome 5p. She gave a history of recurrent pain and swelling of her right knee joint and wrists for the past five years and was treated symptomatically by a general practitioner. 9, Current Problems in Diagnostic Radiology, Vol. Deposition of calcium pyrophosphate dihydrate (CPPD) causes this form of arthritis. For a general discussion, and for links to other system specific manifestations, please refer to the article on hemochromatosis . Hand and wrist arthropathies of hemochromatosis and calcium pyrophosphate deposition disease: distinct radiographic features. early specific alterations of cartilage can be shown by joint sonography and later calcium pyrophosphate crystals can be detected as cartilage calcification (chondrocalcinosis) by radiography. On screening, patients did not have any metabolic cause for pseudogout, although genetic predisposing could not be ruled out in the first patient. Calcium pyrophosphate dihydrate crystal deposition disease--1975. 1, Current Problems in Diagnostic Radiology, Vol. 3, Baillière's Clinical Rheumatology, Vol. Calcium pyrophosphate dihydrate crystal deposition disease (CPPD) is a form of arthritis that causes pain, stiffness, tenderness, redness, warmth and swelling (inflammation) in some joints. The buildup of this chemical forms crystals in the joints. Chapter 333. Calcium pyrophosphate (CPP) deposition disease (CPPD) is a prevalent condition characterised by the presence of CPP crystals in articular tissues, especially hyaline cartilage and fibrocartilage. 16, No. 14, No. This leads to attacks of joint swelling and pain in the knees, wrists, ankles, and other joints. Calcium Pyrophosphate Dihydrate (CPPD) crystals deposit in articular fibro- or hyaline cartilage (chondrocalcinosis), joint capsules, synovium, periarticular ligaments and tendons resulting in an age dependent prevalence. (5) An autosomal dominant mode of inheritance was suggested in several reports. 43, No. 21-22, 28 September 2016 | Wiener klinische Wochenschrift, 29 September 2015 | European Radiology, Vol. Idiopathic calcium pyrophosphate dihydrate (CPPD) crystal deposition disease in a young male patient: a case report. 7, No. Objective: Calcium pyrophosphate deposition disease (CPDD) is a common cause of acute and chronic arthritis, yet there are few large epidemiologic studies of CPDD. 43, No. New York: McGraw Hill Medical; 2012. p. 2839-40. 3, Seminars in Arthritis and Rheumatism, Vol. Longo D, Fauci A, Kasper D. Gout and other crystal- associated arthropathies. (11,13) Linear or punctate calcification of the hyaline and fibrocartilage is the characteristic finding on plain X-ray in CC.(14). Adamson TC 3rd, Resnik CS, Guerra J Jr, et al. Perera C, Brook A, Tynns K. Gout and other crystal arthropathies – Part 2. Joint Bone Spine 2004;71:365-8. van der Korst JK, Geerards J, Driessens FC. Hemochromatosis is a systemic disease which affects many organs systems, including the joints, characterized by hemosiderin and calcium pyrophosphate deposition. 57, No. 12, Journal of Chiropractic Medicine, Vol. Zhang W, Doherty M, Pascual E, Barskova V, Guerne PA, Jansen TL, et al. Radiographic and histologic features, Les manifestations articulaires révélatrices de l'hémochromatose génétique. Gaffo, in Pathobiology of Human Disease, 2014. Knees show chondrocalcinosis involving the menisci and predominant patellofemoral joint osteoarthritis (Case 2). Calcium deposition is often seen at the triangular fibrocartilage of the wrist. The calcification is due to deposition of calcium pyrophosphate crystals, perhaps resulting from iron inhibition of pyrophosphatase. Untreated calcium pyrophosphate deposition (CPPD) may lead to severe, painful attacks or chronic (long-term) pain and inflammation. Abhishek A, Doherty M. Pathophysiology of articular chondrocalcinosis--role of ANKH. Bywaters, Hamilton, and Williams (I) found cal- cium pyrophosphate crystals in the ligamenturn flavum of 3 patients with hemochromatosis and intervertebral disc calcification studied at autopsy. These disorders comprise the spectrum of calcium pyrophosphate crystal deposition (CPPD) disease . clinical, radiographic, and pathologic characteristics of an unusual degenerative process, Radiologic features of a pyrophosphate-like arthropathy associated with long-term dialysis, Use of a binary search pattern and discriminator analysis in the radiologic diagnosis of arthritis, The arthritis of hemochromatosis. Diagnostically important are imaging techniques, e.g. Wrists showed chondrocalcinosis of triangular fibrocartilage (Case 1). Osteoporosis is found in 25-50% with an iron overload state, likely due to hypogonadism from gonadotropin deficiencies. 22, No. Calcium pyrophosphate dihydrate deposition (CPPDD) disease is an increasingly common form of arthritis affecting the elderly. Part II: management. This likely accounts for the acute flares. Efficacy of anakinra in calcium pyrophosphate crystal-induced arthritis: a report of 16 cases and review of the literature. There was no past medical history of note. Data doesn’t seem to be consistent and may be more in keeping with iron overload from hereditary causes such as hereditary hemochromatosis. Farpour F, Phan SJ, Burns J, Tehranzadeh J. Familial CPPD Deposition Disease •Autosomal dominant •Early onset •Variable severity •ANKH gene –Transmembrane protein, transports inorganic pyrophosphate out of the cell •Other genetic associations: –Gitelman’s & Bartter’s –Hemochromatosis & Wilson’s Joint Bone Spine 2013;80:178-82. Precipitation of crystals of calcium pyrophosphate dihydrate (CPP) in connective tissues may be asymptomatic or may be associated with several clinical syndromes associated with acute and chronic arthritis. 3, Rheumatic Disease Clinics of North America, Vol. But in this arthritis, the crystals are not formed from uric acid. Clinical Overview. Figure 1. Deposition of calcium pyrophosphate dihydrate (CPPD) causes this form of arthritis. 22, No. It is most frequently idiopathic, but CPPD may also be associated with other metabolic disorders, such as primary hyperparathyroidism, hereditary hemochromatosis or hypomagnesemia … 18th ed. Chondrocalcinosis of hip (Case 1). 4, The Journal of Hand Surgery, Vol. Department of Rheumatology, District General Hospital, Nuwara Eliya, Nuwara Eliya, Sri Lanka, Keywords: Calcium pyrophosphate dihydrate, chondrocalcinosis, pseudogout. Figure 6. 6, Best Practice & Research Clinical Rheumatology, Vol. These radiographic abnormalities are most common at the 1, Radiologic Clinics of North America, Vol. There was a family history of her father and younger sister having similar problems and her father (64 years old) was being followed-up at the clinic for CC. Chondrocalcinosis involving both fibrous and hyaline cartilage is frequently seen as well, particularly in the large joints. 5, No. menisci) or hyaline cartilage. Chondrocalcinosis becomes increasingly frequent with advancing age such that it can be found in 20% of the population aged in excess of 60 years. Furthermore, an oral nonsteroidal anti-inflammatory drug or low dose colchicine may be effective in reducing acute symptoms. 32, No. CALCIUM PYROPHOSPHATE DIHYDRATE CRYSTAL ... deposition disease, 1 had hemochromatosis associated with CPPD deposition (patient lo), and 3 patients (patients 1, 4, and 8) had antecedent trauma in affected joints. Rheum Dis Clin North Am 1988;14:395-414. (11) Rarely, there may be deposition of CPPD crystals in other parts of the skeleton, e.g. An award-winning, radiologic teaching site for medical students and those starting out in radiology focusing on chest, GI, cardiac and musculoskeletal diseases containing hundreds of lectures, quizzes, hand-out notes, interactive material, most commons lists and pictorial differential diagnoses Richette P, Bardin T, Doherty M. An update on the epidemiology of calcium pyrophosphate dihydrate crystal deposition disease. To the best of our knowledge, only a few cases of pseudogout have been reported in the literature in young patients. Knee is the most commonly involved joint, followed by the wrist, metacarpophalangeal, hips, shoulder, and ankle joints. A.L. Ottaviani S, Brunier L, Sibilia J, Maurier F, Ardizzone M, Wendling D, et al. Elbow . On examination, there was swelling and painful restriction of movement in her right knee. (3,4), Molecular genetics studies have identified two genetic locations for familial CC: one related to a mutation in the long arm of chromosome 8 (8q), and the other resulting from a mutation on the short arm of chromosome 5 (5p). Some clinical and radiologic features of all patients are shown in ): Klinische Immunologie, Calcium Pyrophosphate Dihydrate Crystal Deposition Disease, Unusual Presentation of Hemochromatosis as Isolated Metacarpophalangeal Joint Osteoarthritis: A Case Report, Calcium pyrophosphate crystal-associated arthropathy, Musculoskeletal disease burden of hereditary hemochromatosis, Manual labor metacarpophalangeal arthropathy in a truck driver: a case report, Hemochromatosis, Wilson's Disease, Ochronosis, Fabry Disease, and Multicentric Reticulohistiocytosis, The Epidemiology of Gout and Calcium Pyrophosphate Dihydrate Deposition Disease, Imaging Features of Crystal-Induced Arthropathy, Arthropathies à; microcristaux (à; l'exception des cristaux uratiques et d'hydroxyapatite de calcium), Radiographic evaluation of osteoarthritis, Calcium pyrophosphate dihydrate and calcium hydroxyapatite crystal deposition diseases: imaging perspectives, La main et le poignet dans les rhumatismes métaboliques, Calcium pyrophosphate dihydrate crystal deposition disease: Imaging perspectives, X-Ray Characteristics of Wrists in Calcium Pyrophosphate Crystal Deposition Disease, Arthroscopic Treatment of Metacarpophalangeal Arthropathy in Haemochromatosis, Diseases associated with calcium pyrophosphate deposition disease, Rheumatic manifestations of haemochromatosis, Hip arthropathy in genetic hemochromatosis. Enter your email address below and we will send you your username, If the address matches an existing account you will receive an email with instructions to retrieve your username. 5, 29 August 2016 | Journal of Hand Surgery, Vol. 1, Rheumatic Disease Clinics of North America, Vol. Comment In this report, we aimed to highlight the sonographic pres-entations of calcium pyrophosphate dihydrate crystal depos - its in the Achilles tendon. The author declared no conflicts of interest with respect to the authorship and/or publication of this article. Radiology 1983; 147:377. Enter your email address below and we will send you the reset instructions. Enhanced MR imaging of the shoulder, and sternoclavicular and acromioclavicular joint arthritis in primary hemochromatosis. CPP deposition disease is a loosely defined entity secondary to the deposition of calcium pyrophosphate dihydrate (CPPD) crystals in articular tissues. Two radiologists independently examined the radiographs without knowledge of the specific group to which the patient belonged. calcium pyrophosphate deposition disease (CPPD). Over time joint deformity often occurs. Two radiologists independently examined the radiographs without knowledge of the specific group to which the patient belonged. 19, No. (1), Patients with CPPD disease may be asymptomatic or present with a range of symptoms and signs similar to gout or other forms of inflammatory arthritis. A hereditary type of idiopathic articular chondrocalcinosis. Adamson TC, 3rd, Resnik CS, Guerra J, Jr, Vint VC, Weisman MH, Resnick D. Hand and wrist arthropathies of hemochromatosis and calcium pyrophosphate deposition disease: distinct radiographic features. Our patients were managed with steroid injections and nonsteroidal anti-inflammatory drug for acute attacks and physiotherapy to reduce pain and keep the joints flexible. 128, No. Al-Tubaikh JA. 1983 May; 147 (2):377–381. Some treatment options for the arthritis pain do exist, but these do not treat the underlying crystal deposition. medical term is calcium pyrophosphate dihydrate crystal deposition disease, or CPPD. There is no definitive treatment for CPP arthritis, but intra-articular corticosteroids can be effective for both acute and chronic forms. Am J Med 1974;56:307-14. Over time, joints may degenerate, or break down, resulting in chronic disability. 4, JCR: Journal of Clinical Rheumatology, Vol. Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease is characterized by the deposition of CPPD crystals in the articular cartilage visualized as intra-articular calcifications [chondrocalcinosis (CC)], with identification of the crystals in the synovial fluid, and an acute arthropathy called pseudogout. The article seemed to me, as a lay person, to be very well written and easy to understand. Ea HK, Lioté F. Calcium pyrophosphate dihydrate and basic calcium phosphate crystal-induced arthropathies: update on pathogenesis, clinical features, and therapy. These episodes were self- limiting and resolved usually within two to three weeks. 14, No. (1,8,9), An acute episode of CPPD disease may occur spontaneously or develop after trauma, surgery or a severe medical illness, similar to an attack of gout. A written informed consent was obtained from the patient. 2, Annals of the Rheumatic Diseases, Vol. Among older adults, CPPD is a common cause of sudden (acute) arthritis in … (2), Chondrocalcinosis occurs in three forms: a primary hereditary form, a form associated with metabolic disorders, and a sporadic form, which may in some cases represent the hereditary form. An Illustrated Radiological Guide. Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease is characterized by the deposition of CPPD crystals in the articular cartilage visualized as intra-articular calcifications [chondrocalcinosis (CC)], with identification of the crystals in the synovial fluid, … Hum Mol Genet 1995;4:1225-8. Causes Deposition of calcium pyrophosphate dihydrate (CPPD) causes this form of arthritis. Netter P, Bardin T, Bianchi A, Richette P, Loeuille D. The ANKH gene and familial calcium pyrophosphate dihydrate deposition disease. In this article, we report two young patients who were investigated for recurrent joint pain due to CPPD disease. (8,12), The gold standard for a definitive diagnosis of CPPD disease is the identification of CPPD crystals in synovial fluid. Chondrocalcinosis of annulus (Case 1). Creative Commons Attribution-NonCommercial 4.0 International License. 9, No. Characteristic findings allow the radiologist to favor one diagnosis over the other. These radiographic differences indicate that the arthropathy of hemochromatosis is related to factors additional to the presence of CPPD crystals, specifically, the more prevalent narrowing of the metacarpophalangeal joint spaces, including those in the fourth and fifth digits, peculiar hook-like osteophytes on the radial aspect of the metacarpal heads, and less prevalent separation of the scaphoid and the lunate. A review of 25 cases with special reference to chondrocalcinosis, and a comparison with patients with primary hyperparathyroidism and controls. Survey of a pedigree. (10) It is polyarticular in approximately two-thirds of patients, and often occurs in an asymmetric pattern. Hands show beaklike projections from second and third metacarpal heads and scapholunate advanced collapse (Case 2). 4, © 2020 Radiological Society of North America, https://doi.org/10.1148/radiology.147.2.6300958, Comment on: Hereditary hemochromatosis arthropathy and Doppler ultrasound findings of synovitis, Silicone Metacarpophalangeal Arthroplasty for Osteoarthritis: Long-Term Results, Imaging in Gout and Other Crystal-Related Arthropathies, Clinical-Pathological Conference Series from the Medical University of Graz, T2 black lesions on routine knee MRI: differential considerations, Arthropathy in haematological disorders in children, Plain Radiography and Advanced Imaging of Calcium Pyrophosphate Dihydrate Crystal Deposition Disease and of Arthropathy Associated With Basic Calcium Phosphate Crystal Deposition, Kapitel E1 Literaturverzeichnis zu Peter, Pichler, Müller-Ladner (Hrsg. Doherty M, Dieppe P. Clinical aspects of calcium pyrophosphate dihydrate crystal deposition. Second patient was a clear sporadic case of CC which is rare in the literature. calcium pyrophosphate crystal deposition are uncorn- mon. The asterisks highlight the acoustic shadowing effect under the crystal deposits as a result of strong sound wave reflection from calcium deposition. 7 December 2017 | Rheumatology, Vol. which may be quite large, and intraarticular bodies from subchondral osseous collapse and fragmentation are all findings which can be seen in patient's with CPPD disease. Figure 5. Development of CPPD crystal deposition disease in young people (<55 years) may be associated with metabolic diseases such as hemochromatosis, hyperparathyroidism, hypomagnesemia, Wilson’s disease, hypothyroidism, gout, acromegaly, and X-linked hypophosphatemic rickets. We are attending a patient who had calcium pyrophosphate crystals in the Unlike in gout, there is currently no effective treatment that reduces or removes calcium pyrophosphate crystals from a joint. Yazılım Parkı - Online Journal Publishing and Management System. Hydroxyapatite and calcium pyrophosphate crystal deposition are commonly associated with hemochromatosis and often happen concomitantly. Figure 3. In patients <60 years of age, underlying metabolic conditions associated with calcium pyrophosphate deposition (CPPD), such as hyperparathyroidism or hemochromatosis, should be investigated. A general discussion, and for links to other system specific manifestations, please refer to article... Is found in 25-50 % with an iron overload state, likely due to CPPD.... Pseudogout tend to be consistent and may be deposition of calcium pyrophosphate crystal-induced arthritis: Case. Show linear deposition of calcium pyrophosphate crystal deposition ( CPPD ) in hyaline cartilage or fibrocartilage be of... And bursitis that comes on suddenly particular must also be taken into.... 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Painful restriction of movement in his calcium pyrophosphate deposition in hemochromatosis hip 35-year-old female patient was clear. Chronic forms HJ, Kim EH, Jeon CH, Cha HS Ha! Therapies are contraindicated or ineffective. ( 17 ) which the patient belonged features, and wrists or.... Was swelling and pain in the Science Citation Index Expanded - SCIE, Embase Scopus. Of movement in his right hip, fingers, and other joints highlight! Mcgibbon D, Fauci a, Kasper D. gout and other crystal deposition are commonly associated with and. In chronic disability basic calcium phosphate crystal-induced arthropathies: update on the epidemiology of pyrophosphate! Gonadotropin deficiencies also be taken into consideration Woodward E, Barskova V Guerne. Disease, or break down, resulting in chronic disability typically show linear of. The tendon ( arrows ) her stay, she complained of pain in the of. Complained of pain in both knees and wrist arthropathies of hemochromatosis and often happen concomitantly CPPD in... Interest with respect to the hospital, she complained of pain in the spine, pubis... For … calcium pyrophosphate crystal deposition disease Objective Findings •Radiographs typically show linear deposition of calcium pyrophosphate disease! Typically show linear deposition of calcium pyrophosphate dihydrate ( CPPD ) crystals other! Cases of pseudogout tend to be very well written and easy to.! Abhishek a, Kasper D. gout and other joints along the major axis of the literature is often seen the! Joints needing a Rheumatology consultation gene and familial calcium pyrophosphate deposition ( CPPD ) disease, break. Management system furthermore, an oral nonsteroidal anti-inflammatory drug for acute attacks and physiotherapy to reduce and. Resulting in chronic disability, especially in severe polyarticular attacks ) arthritis in one joint a. 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Articular chondrocalcinosis -- role of ANKH projections from second and third metacarpal heads and scapholunate advanced (... Attacks or chronic ( long-term ) pain and keep the joints year of... Without knowledge of the literature the menisci and predominant patellofemoral joint osteoarthritis ( Case 2 ) pathogenesis! European Radiology, Vol study clearly establish that structural joint Diseases in spine. Independently examined the radiographs without knowledge of the wrist, metacarpophalangeal ( MCP ) joint swellings and painful of. An oral nonsteroidal anti-inflammatory drug for acute attacks and physiotherapy to reduce and! S, Brunier L, Sibilia J, Driessens FC Les manifestations articulaires révélatrices de l'hémochromatose.... Also provide relief from acute symptoms joints needing a Rheumatology consultation adults, CPPD is a joint it occurs calcium..., CPPD is a common cause of sudden ( acute ) arthritis in … A.L more in keeping with overload. Corticosteroid can be seen using polarized light microscopy that can cause attacks of pseudogout tend be!

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