Calcium Pyrophosphate Dihydrate Deposition Research Articles

AB0842 A Simple Method for the Microscopic Identification of Calcium Pyrophosphate Dihydrate and Hydroxyapatite Deposits in Metabolic and Crystal Induced Diseases

BackgroundArthropathy induced by calcium pyrophosphate dihydrate [Ca2P2O7.2H2O] (CPPD) crystals (chondrocalcinosis, pseudogout, pyrophosphate arthropathy) and arthropathy induced by hydroxyapatite [Ca5(PO4)3(OH)] (HA) crystals (apatite rheumatism) are separate entities.CPPD and HA crystals may...

AB0843 A Microscopic Method for Identification of Calcium Pyrophosphate Dihydrate Deposits in Tissues

BackgroundArthropathy induced by calcium pyrophosphate dihydrate [Ca2P2O7.2H2O] () crystals (chondrocalcinosis, pseudogout, pyrophosphate arthropathy) is characterized by intraarticular or periarticular deposition of . Crystals accumulate in the synovial membranes with or...

SAT0506 Interest of Musculoskeletal Ultrasound in the Diagnosis of Calcium Pyrophosphate Dihydrate Deposition Disease

ObjectivesTo evaluate the sensitivity, specificity and predictive values of musculoskeletal ultrasound (US) in the diagnosis of calcium pyrophosphate dihydrate deposition disease (CPPD).MethodsDesign: prospective monocentric study. Patients: consecutive hospitalized or outpatients...

SAT0510 Ultrasound versus Synovial Fluid Analysis for the Diagnosis of Calcium Pyrophosphate Dihydrate Deposition Disease: is IT Cppd? Ask Us!

BackgroundThe diagnosis of calcium pyrophosphate crystal (CPP) deposition disease (CPPD) is mainly based on the synovial fluid analysis and Xrays. US has demonstrated high sensitivity and specificity values for diagnosing...

The prevalence of and factors related to calcium pyrophosphate dihydrate crystal deposition in the knee joint

The purpose of this study was to reveal the accurate prevalence and related factors to the presence of calcium pyrophosphate dihydrate (CPPD) crystal deposition in cadaveric knee joints. Controlled laboratory study. Six hundred and eight knees from 304 cadavers (332 male knees and 276 female knees, formalin fixed, Japanese anatomical specimens) were included in this study. The average age of the cadavers was 78.3 ± 10.7 years. Knees were macroscopically evaluated for the existence of CPPD, and the depth of cartilage degeneration of the femoro-tibial joint following the Outerbridge's classification. CPPD crystal was confirmed under Fourier transform infrared spectroscopy (FTIR) analysis using light microscopy. Statistical analysis was performed to reveal the correlation between the occurrence of CPPD deposition in the knee joint and gender, age, and the depth of cartilage degeneration of the femoro-tibial joint. The prevalence of grossly visible CPPD crystal was 13% (79 knees). In all of these knees, CPPD crystal was confirmed under FTIR analysis. Statistical analysis showed significant correlation between the occurrence of CPPD deposition and gender (P < 0.001), and depth of cartilage degeneration in the femoro-tibial joint (P < 0.001). In the cartilage degeneration positive knees (Over grade 3 in Outerbridge's classification), average age of CPPD deposition knee was significantly higher than CPPD negative knees. In this study, the prevalence of CPPD deposition disease was evaluated in a relatively large sample size of cadaveric knees. The prevalence of CPPD deposition disease was 13%, and was significantly correlated with the subject's age, gender, and severity of cartilage degeneration in the femoro-tibial joint.

Retro-Odontoid Calcium Pyrophosphate Dehydrate Deposition: Surgical Management and Review of the Literature

Study Design Case report and review of the literature. Objective A retro-odontoid mass is a rare cause of cervical compression and myelopathy. The differential diagnosis includes the following: metastatic disease, primary tumor, collagen disorder, or inflammatory disease. Calcium pyrophosphate dihydrate (CPPD) deposition has been referred to as “crowned dens syndrome” when there are periodontoideal calcifications. There are only a few reported cases where CPPD presents as a cystic retro-odontoid mass in the atlanto-dens interval. In previous descriptions of surgical intervention, transoral resection of the mass is associated with significant morbidity and usually requires stabilization. The objective of this article is to report a case of an unusual presentation of CPPD disease of C1/C2, where we used a novel, minimally invasive surgical technique for decompression without fusion. Patients and Methods An 83-year-old female patient presented with progressive cervical myelopathy over a 3-month period. Computed tomography and magnetic resonance imaging demonstrated a cystic odontoid mass with a separate retro-odontoid compressive mass. A novel, minimally invasive transoral aspiration was performed. Histologic confirmation of CPPD was obtained. Results Postop imaging showed satisfactory decompression, which was maintained at the 6-month follow-up. This correlated with clinical improvement postop and 6-month follow-up. Conclusion CPPD in the atlanto-dens interval may present as a cystic retro-odontoideal mass and should be included in the differential. We used a transoral minimally invasive approach to aspirate the cyst. This novel technique avoided the need for a stabilization procedure or morbid transoral resection and provided excellent results immediately and at 6 months.

Tophaceous pseudogout in a patient undergoing peritoneal dialysis.

Calcium pyrophosphate dihydrate deposition disease (pseudogout) is a crystal arthritis characterized by pyrophosphate dihydrate crystal deposition in the articular cartilage or synovium. In chronic kidney disease patients, the major causes of crystal arthritis are calcium oxalate crystals and basic calcium phosphate crystals. However, pseudogout is apparently infrequent among such patients. This report describes a rare case of tophaceous pseudogout (tumoral form of pseudogout) involving the hip joint in a peritoneal dialysis patient, as revealed by CT scan and synovial fluid aspiration. Fluid aspiration and oral NSAIDs improved the clinical symptoms. This report is the first to describe tophaceous pseudogout in a peritoneal dialysis patient, which suggests that pseudogout should be suspected in dialysis patients with unexplained arthritis.

Large‐volume sample stacking with polarity switching for monitoring of nucleotide pyrophosphatase/phosphodiesterase 1 (NPP1) reactions by capillary electrophoresis

Nucleotide pyrophosphatase/phosphodiesterase 1 (NPP1) is a membrane glycoprotein involved in the hydrolysis of extracellular nucleotides. Its main substrate is ATP yielding AMP and pyrophosphate. NPP1 has been proposed as a novel drug target, for diabetes type 2 and the treatment of calcium pyrophosphate dihydrate deposition disease leading to inflammatory arthritis. The monitoring of NPP1 reactions is difficult because its velocity is very slow requiring highly sensitive analytical procedures. In this study, a method of large-volume sample stacking with polarity switching was developed, and separations were optimized. Large sample volumes were loaded by hydrodynamic injection (5 psi, 13 s) followed by removal of a large plug of sample matrix from the capillary using polarity switching (-10 kV). The stacked analytes were subsequently separated in phosphate buffer (100mM, pH 9.2) at 20 kV. The validated method was found to be linear (R(2) = 0.9927) in the concentration range of 0.05-50μM of AMP, with high accuracy and precision. The determined LOD and LOQ of AMP were 18 nM and 60 nM, respectively. Compared to a previously reported CE procedure using sweeping technique, a fivefold improvement of sensitivity was achieved. Moreover, the new technique was faster, and reproducibility of migration times was improved (RSD value = 1.2%). Importantly, adenine nucleotide analogs and derivatives tested as NPP1 inhibitors could be completely separated from the substrate ATP and the enzymatic product AMP. The method was applied to NPP1 inhibition assays investigating nucleotide-derived inhibitors in the presence of ATP.

Does Chondrocalcinosis Affect Knee Society Scores and Range of Motion After TKA?

Chondrocalcinosis is manifested by crystalline deposits of calcium commonly found during primary TKA for osteoarthritis. Its frequency among patients undergoing TKA is poorly defined, as is its influence on pain or function after TKA. The purposes of this study are to (1) determine the prevalence of chondrocalcinosis in patients undergoing TKA for osteoarthritis; (2) evaluate the effect of chondrocalcinosis on ROM and The Knee Society scores; (3) determine if patients with chondrocalcinosis and severe synovitis who underwent synovectomy are at risk for lower postoperative Knee Society scores and less ROM; and (4) assess if chondrocalcinosis is associated with increased rates of revision surgery. We retrospectively reviewed the medical records of 1500 primary TKAs performed by one surgeon. The minimum followup for patients was 24months (average, 57months; range, 24-120months). There were 511 men and 934 women with an average age of 70years. Fifty-five patients underwent bilateral knee replacements. Crystal deposition was graded prospectively during surgery using a subjective visual scale. A thorough synovectomy was performed on patients with severe synovitis and apparent crystalline deposition suggestive of calcium pyrophosphate dihydrate (CPPD) deposition (n=50). The Knee Society scores, ROM, and revision rates were compared between patients with visible chondrocalcinosis with those without and between patients with mild chondrocalcinosis with those with severe chondrocalcinosis. Chondrocalcinosis was found in 173 male patients (34%) undergoing TKAs during this period compared with 224 female patients (24%) (p<0.001). The Knee Society scores for knee rating and function were similar in patients with or without chondrocalcinosis undergoing TKA. However, patients with visible CPPD deposition who underwent synovectomy for proliferative synovitis had diminished final ROM and Knee Society knee rating scores (107(o) versus 115(o) knee flexion, p<0.001 and 87 versus 94 points, p=0.001). We cannot determine whether this result is because of the synovectomy or severity of the disease, and therefore we cannot recommend a synovectomy at this time. Revision rates were no different among patients with chondrocalcinosis compared with those without it (3.6% versus 2.2%, p=0.2). Chondrocalcinosis is common among patients undergoing TKA for osteoarthritis. The presence of CPPD deposition does not appear to affect the ROM and Knee Society scores of patients with CPPD but without severe synovitis. However, patients with severe synovitis and visible CPPD who underwent thorough synovectomy may be at risk for having decreased postoperative ROM and pain develop. Level III, therapeutic study. See the Instructions for Authors for a complete description of levels of evidence.

A Case of Calcium Pyrophosphate Dihydrate Deposition Disease Associated with Primary Hyperparathyroidism

Division of Rheumatology, Department of Internal Medicine, Keimyung University Donsan Medical Center, Daegu, Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases, Department of Radiology, Hanyang University Hospital for Rheumatic Diseases, Department of Pathology, Hanyang University Hospital, Division of Endoclinology, Department of Internal Medicine, Hanyang University Hospital, Department of Otolaryngology-Head and Neck Surgery, Hanyang University Hospital, YTS Rheumatology Clinic, Seoul, Korea

Tophaceous pseudogout of the temporomandibular joint: a series of 3 cases

We report a series of 3 cases of tophaceous pseudogout of the temporomandibular joint (TMJ). Three patients, two men and one woman, ranging in age between 60 and 75 years, presented with unilateral painful swelling of the TMJ area associated with limitation of mouth opening. Radiographic and computed tomographic images showed opaque masses in the supracondylar region of the TMJ. The preoperative clinical impression was a "neoplastic lesion" in the 3 cases. Microscopic examination revealed numerous deeply basophilic masses of calcified deposits, exhibiting birefringence under polarized light and morphologically consistent with calcium pyrophosphate dihydrate deposition, referred to in these cases as "tophaceous pseudogout." Tophaceous pseudogout is a rare benign arthropathy that presents with clinical and radiographic features mimicking neoplastic conditions of the TMJ. Therefore, it is recommended that tophaceous pseudogout be considered in the differential diagnosis when a TMJ is involved with "neoplasm-like" lesions clinically and radiographically.

Upregulation of ANK Protein Expression in Joint Tissue in Calcium Pyrophosphate Dihydrate Crystal Deposition Disease

Accumulation of excess extracellular inorganic pyrophosphate leads to calcium pyrophosphate dihydrate (CPPD) crystal formation in articular cartilage. CPPD crystal formation occurs near morphologically abnormal chondrocytes resembling hypertrophic chondrocytes. The ANK protein was recently implicated as an important factor in the transport of intracellular inorganic pyrophosphate across the cell membrane. We characterized ANK in joint tissues from patients with and without CPPD deposition and correlated the presence of ANK with markers of chondrocyte hypertrophy. Articular tissues were obtained from 24 patients with CPPD crystal deposition disease, 11 patients with osteoarthritis (OA) without crystals, and 6 controls. We determined the number of ANK-positive cells in joint tissues using immunohistochemistry and in situ hybridization, and correlated ANK positivity with markers of chondrocyte hypertrophy including Runx2, type X collagen, osteopontin (OPN), and osteocalcin (OCN). ANK was detected in synoviocytes, chondrocytes, osteoblasts, and osteocytes. ANK was seen extracellularly only in the matrix of cartilage and meniscus. The number of ANK-positive cells was significantly higher in CPPD than in OA or normal joint tissues. The amount and intensity of ANK immunoreactivity reached maximum levels in the large chondrocytes around crystal deposits. ANK was similarly distributed to and significantly correlated with Runx2, type X collagen, OPN, and OCN. ANK levels were higher in articular tissues from patients with CPPD deposition. ANK was concentrated around crystal deposits and correlated with markers of chondrocyte hypertrophy. These findings support a role for ANK in CPPD crystal formation in cartilage.

Frequency of Atlantoaxial Calcium Pyrophosphate Dihydrate Deposition at CT

Purpose To determine (a) the prevalence of atlantoaxial calcium pyrophosphate dihydrate (CPPD) crystal deposition in a population of patients undergoing computed tomography (CT) for acute trauma and (b) the association between atlantoaxial CPPD crystal deposition and retro-odontoid soft-tissue thickness. Materials and Methods This HIPAA-compliant study was approved by the institutional review board, and the requirement to obtain informed consent was waived. In 513 consecutive patients, CT scans of the cervical spine obtained for acute trauma were retrospectively reviewed for the presence of atlantoaxial CPPD crystal deposition, and the maximal thickness of the retro-odontoid soft tissues was measured. The relationships among imaging findings, age, and sex were assessed with the t test, the χ2 test, Spearman correlation, and logistic and linear regression models as appropriate. Results The overall prevalence of atlantoaxial CPPD crystal deposition was 12.5% (64 of 513 patients), and prevalence increased with age (P < .0001, logistic regression coefficient). In patients aged 60 years and older, the prevalence of CPPD crystal deposition was 34% (58 of 170 patients). In patients aged 80 years and older, the prevalence of CPPD crystal deposition was 49% (37 of 75 patients). There was a positive correlation between age and retro-odontoid soft-tissue thickness (Spearman ρ = 0.48, P < .0001). The mean retro-odontoid soft-tissue thickness in patients with CPPD crystal deposition was greater than that in patients without CPPD crystal deposition (3.4 mm vs 2.2 mm, respectively; P < .0001, t test). Conclusion CPPD crystal deposition in the cervical spine is seen with a higher prevalence than previously reported. CPPD crystal deposition shows a positive correlation with age and retro-odontoid soft-tissue thickening. © RSNA, 2013

Calcific Periarthritis as the Only Clinical Manifestation of Hypophosphatasia in Middle-Aged Sisters

Hypophosphatasia (HPP) is the inborn error of metabolism that features low serum alkaline phosphatase (ALP) activity caused by loss-of-function mutation(s) within the gene for the tissue nonspecific isoenzyme of ALP (TNSALP). In HPP, extracellular accumulation of inorganic pyrophosphate (PPi), a TNSALP substrate and inhibitor of mineralization, leads frequently to premature tooth loss and often to rickets or osteomalacia. In affected adults, the excess PPi sometimes also causes calcium pyrophosphate dihydrate (CPPD) deposition, PPi arthropathy, or pseudogout, or seemingly paradoxical deposition of hydroxyapatite crystals in ligaments or around joints when the condition is called calcific periarthritis (CP). We report three middle-aged sisters with CP as the only clinical manifestation of HPP. Each presented during early adult life with recurrent episodes of pain principally around the shoulders, elbows, wrists, hips, or Achilles tendon. Otherwise, they were in good health, including no history of unusual dental disease, fractures, or pseudofractures. Calcific deposits were identified in symptomatic areas principally by ultrasonographic assessment but also confirmed radiographically. All three sisters had low serum levels of total and bone-specific ALP, hyperphosphatemia, and increased serum concentrations of the TNSALP substrate pyridoxal 5'-phosphate together characteristic of HPP. Mutation analysis revealed that each carried a single unique 18-bp duplication within TNSALP (c.188_205dup18, p.Gly63_Thr68dup) as did two of their healthy sons and their mother, who was without signs of CPPD deposition or CP but had knee osteoarthritis. We find that CP can be the only complication of HPP in adults. Thus, multiple juxta-articular deposits of hydroxyapatite causing CP may be a useful sign of HPP, especially when the CP is familial.

A case of pseudogout of the temporomandibular joint with giant cell reparative granuloma of the temporal bone

Pseudogout, also known as calcium pyrophosphate dihydrate deposition disease (CPPD) with giant cell reparative granuloma (GCRG) of the temporal bone is a rare disease, which is very easy to misdiagnose. When two diseases occur simultaneously, the pathological tissue of diseases is closely associated, which complicates clinical representation and causes enormous difficulty in diagnosis and treatment. We report a case of CPPD of the temporomandibular joint accompanied by surrounding GCRG of temporal bone in a 62-year-old male.

Axial calcium pyrophosphate dihydrate deposition disease revealed by recurrent sterile spondylodiscitis and epidural abscess

Spondylodiscitis are frequent and clinical challenge for practionners. Axial calcium pyrophosphate dihydrate deposition disease (CPDD) is well known for cervical spine involvement with the crowned dens syndrome but other localisations are probably underdiagnosed in sterile spondylodiscitis. We report a case of recurrent sterile spondylodiscitis with epidural abscess related to CPDD proved by vertebral percutaneous needle biopsy with rapid favourable course under colchicine therapy. Axial CPDD could mimic septic spondylodiscitis with epidural abscess on MRI. Sterile spondylodiscitis are probably underdiagnosed forms of microcrystalline disease. Investigations of the presence of microcrystals should be systematically undertaken with tamponed formalin fixed biopsies. If axial CPDD is suspected, colchicine therapy could be a good therapeutic test and would avoid unnecessary antibiotic treatment.

Frequency of Atlantoaxial Calcium Pyrophosphate Dihydrate Deposition at CT

To determine (a) the prevalence of atlantoaxial calcium pyrophosphate dihydrate (CPPD) crystal deposition in a population of patients undergoing computed tomography (CT) for acute trauma and (b) the association between atlantoaxial CPPD crystal deposition and retro-odontoid soft-tissue thickness. This HIPAA-compliant study was approved by the institutional review board, and the requirement to obtain informed consent was waived. In 513 consecutive patients, CT scans of the cervical spine obtained for acute trauma were retrospectively reviewed for the presence of atlantoaxial CPPD crystal deposition, and the maximal thickness of the retro-odontoid soft tissues was measured. The relationships among imaging findings, age, and sex were assessed with the t test, the χ(2) test, Spearman correlation, and logistic and linear regression models as appropriate. The overall prevalence of atlantoaxial CPPD crystal deposition was 12.5% (64 of 513 patients), and prevalence increased with age (P < .0001, logistic regression coefficient). In patients aged 60 years and older, the prevalence of CPPD crystal deposition was 34% (58 of 170 patients). In patients aged 80 years and older, the prevalence of CPPD crystal deposition was 49% (37 of 75 patients). There was a positive correlation between age and retro-odontoid soft-tissue thickness (Spearman ρ = 0.48, P < .0001). The mean retro-odontoid soft-tissue thickness in patients with CPPD crystal deposition was greater than that in patients without CPPD crystal deposition (3.4 mm vs 2.2 mm, respectively; P < .0001, t test). CPPD crystal deposition in the cervical spine is seen with a higher prevalence than previously reported. CPPD crystal deposition shows a positive correlation with age and retro-odontoid soft-tissue thickening.

FRI0501 Ultrasound versus synovial fluid analysis for the diagnosis of calcium pyrophosphate dihydrate deposition disease: preliminary results.

BackgroundThe diagnosis of calcium pyrophosphate crystal (CPP) deposition disease (CPPD) is mainly based on the synovial fluid analysis and Xrays. US has demonstrated high sensitivity and specificity values for diagnosing...

Extent and distribution of CPP deposits in patients affected by calcium pyrophosphate dihydrate deposition disease: an ultrasonographic study

ObjectiveTo assess the extent of calcium pyrophosphate dihydrate (CPP) crystal deposition and the distribution of affected sites, using ultrasonography (US), in patients affected by CPP deposition disease (CPPD).Patients and methods42...

Calcium pyrophosphate dihydrate deposition disease of the filum terminale

Calcium pyrophosphate dihydrate crystal deposition disease (CPPDD) is a rare benign inflammatory joint disorder characterized by the presence of calcium pyrophosphate dihydrate crystal in the interarticular and periarticular tissue. It has been rarely described with spinal localization. A 50-year-old woman, affected by CPPDD, presented a progressive weakness of both lower limbs associated with neurogenic claudication. Neuroradiological examinations revealed the presence of two intradural calcified lesions at level L3-L4, with no post-contrast enhancement. Surgery was performed and the histopathological exams documented the presence of rod-shaped crystals embedded in a fibrocartilaginous stroma. The postoperative course was uneventful and the patient experienced complete symptoms relief with a 5-year follow-up. Intradural CPPD localization at the filum terminale is an extremely rare occurrence. Total removal should be preferably attempted with a long-term focal control of the disease as we observed in our case.