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Calcium pyrophosphate deposition disease of the temporomandibular joint.

ABSTRACT: Calcium pyrophosphate dihydrate deposition disease (CPDD, tophaceous pseudogout) is a rare crystal arthropathy characterized by calcium pyrophosphate crystal deposition in joint spaces, episodes of synovitis, and radiological features of chondrocalcinosis. We present a case of 61-year-old woman who presented with left temporomandibular joint (TMJ) pain, difficulty chewing, left facial numbness, left-sided hearing loss, and left TMJ swelling. Imaging of the temporal fossa revealed a large mass emanating from the temporal bone at the TMJ, extending into the greater wing of the sphenoid and involving the mastoid bone and air cells posteriorly. Fine needle aspiration demonstrated polarizable crystals with giant cells. Intraoperatively, the TMJ was completely eroded by the mass. Final pathology was consistent with tophaceous pseudogout. CPDD has rarely been reported involving the skull base. None of the cases originally described by McCarty had TMJ pseudogout. Symptoms are generally pain, swelling, and hearing loss. Management is nearly always surgical with many patients achieving symptomatic relief with resection. CPDD is associated with many medical problems (including renal failure, gout, and hyperparathyroidism), but our patient had none of these risk factors. This case demonstrates that CPDD can involve the skull base and is best treated with skull base surgical techniques.

SUBMITTER: Srinivasan V

PROVIDER: S-EPMC3658656 | biostudies-other | 2012 Oct

REPOSITORIES: biostudies-other

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Calcium Pyrophosphate Dihydrate Crystal Deposition Disease of the Sternoclavicular Joint.

Project description:Deposition of calcium pyrophosphate dihydrate (CPPD) crystals in the articular structures affects predominantly temporomandibular, knee, hip, spine, and wrist joints, and is a rare condition, often mimicking malignancy. Sternoclavicular joint is extremely rarely involved. We present a patient with swelling of the right upper extremity, in whom on computed tomography a mass posterior to the sternoclavicular joint causing compression of the brachiocephalic vein was detected. A modified resection arthroplasty was performed, and the histopathological findings revealed massive deposits of CPPD in the articular cartilage. To our knowledge, there is only one similar case published in the literature.

| S-EPMC4670306 | biostudies-other

Review: Unmet Needs and the Path Forward in Joint Disease Associated With Calcium Pyrophosphate Crystal Deposition.

Project description:Calcium pyrophosphate (CPP) crystal deposition (CPPD) is prevalent and can be associated with synovitis and joint damage. The population of elderly persons predominantly affected by CPPD is growing rapidly. Since shortfalls exist in many aspects of CPPD, we conducted an anonymous survey of CPPD unmet needs, prioritized by experts from the Gout, Hyperuricemia and Crystal-Associated Disease Network. We provide our perspectives on the survey results, and we propose several CPPD basic and clinical translational research pathways. Chondrocyte and cartilage culture systems for generating CPP crystals in vitro and transgenic small animal CPPD models are needed to better define CPPD mechanism paradigms and help guide new therapies. CPPD recognition, clinical research, and care would be improved by international consensus on CPPD nomenclature and disease phenotype classification, better exploitation of advanced imaging, and pragmatic new point-of-care crystal analytic approaches for detecting CPP crystals. Clinical impacts of CPP crystals in osteoarthritis and in asymptomatic joints in elderly persons remain major unanswered questions that are rendered more difficult by current inability to therapeutically limit or dissolve the crystal deposits and assess the consequent clinical outcome. Going forward, CPPD clinical research studies should define clinical settings in which articular CPPD does substantial harm and should include analyses of diverse clinical phenotypes and populations. Clinical trials should identify the best therapeutic targets to limit CPP crystal deposition and associated inflammation and should include assessment of intraarticular agents. Our perspective is that such advances in basic and clinical science in CPPD are now within reach and can lead to better treatments for this disorder.

| S-EPMC6105380 | biostudies-literature

Trauma Induced Calcium Pyrophosphate Deposition Disease of the Lumbar Spine.

Project description:Inflammatory arthritis, such as pseudogout or otherwise referred to as calcium pyrophosphate (CPP) crystal arthritis or calcium pyrophosphate deposition (CPPD) disease, is characterized by the deposition of crystal formation and deposition in large joints. CPPD is known to affect the elderly population and commonly manifests as inflammation of knees, hips, and shoulders. CPPD disease involving the spine has been infrequently encountered in practice and rarely described in the literature. Here, we describe a case of an 80-year-old female with no known history of inflammatory arthritis who presented with left lower extremity weakness and fall, initially thought to have discitis, later confirming CPPD of the spine through biopsy and ultimately resolution of symptoms with anti-inflammatory agents. Although consisting of different clinical presentations, two other case reports have described CPPD of the spine with similar radiographic findings, to this author's knowledge. With the radiologic similarities, this unique case serves to raise awareness in the medical community and possibly place pseudogout of the spine on the differential list when such cases are encountered. As a result, patients can be initiated on benign anti-inflammatory agents, avoiding invasive testing and unnecessary antibiotic exposure.

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The 2023 ACR/EULAR classification criteria for calcium pyrophosphate deposition disease.

Project description:ObjectiveCalcium pyrophosphate deposition (CPPD) disease is prevalent and has diverse presentations, but there are no validated classification criteria for this symptomatic arthritis. The American College of Rheumatology (ACR) and EULAR have developed the first-ever validated classification criteria for symptomatic CPPD disease.MethodsSupported by the ACR and EULAR, a multinational group of investigators followed established methodology to develop these disease classification criteria. The group generated lists of candidate items and refined their definitions, collected de-identified patient profiles, evaluated strengths of associations between candidate items and CPPD disease, developed a classification criteria framework, and used multi-criterion decision analysis to define criteria weights and a classification threshold score. The criteria were validated in an independent cohort.ResultsAmong patients with joint pain, swelling, or tenderness (entry criterion) whose symptoms are not fully explained by an alternative disease (exclusion criterion), the presence of crowned dens syndrome or calcium pyrophosphate crystals in synovial fluid are sufficient to classify a patient as having CPPD disease. In the absence of these findings, a score>56 points using weighted criteria, comprising clinical features, associated metabolic disorders, and results of laboratory and imaging investigations, can be used to classify as CPPD disease. These criteria had a sensitivity of 92.2% and specificity of 87.9% in the derivation cohort (190 CPPD cases, 148 mimickers), whereas sensitivity was 99.2% and specificity was 92.5% in the validation cohort (251 CPPD cases, 162 mimickers).ConclusionThe 2023 ACR/EULAR CPPD disease classification criteria have excellent performance characteristics and will facilitate research in this field.

| S-EPMC10529191 | biostudies-literature

The 2023 ACR/EULAR Classification Criteria for Calcium Pyrophosphate Deposition Disease.

Project description:ObjectiveCalcium pyrophosphate deposition (CPPD) disease is prevalent and has diverse presentations, but there are no validated classification criteria for this symptomatic arthritis. The American College of Rheumatology (ACR) and EULAR have developed the first-ever validated classification criteria for symptomatic CPPD disease.MethodsSupported by the ACR and EULAR, a multinational group of investigators followed established methodology to develop these disease classification criteria. The group generated lists of candidate items and refined their definitions, collected de-identified patient profiles, evaluated strengths of associations between candidate items and CPPD disease, developed a classification criteria framework, and used multi-criterion decision analysis to define criteria weights and a classification threshold score. The criteria were validated in an independent cohort.ResultsAmong patients with joint pain, swelling, or tenderness (entry criterion) whose symptoms are not fully explained by an alternative disease (exclusion criterion), the presence of crowned dens syndrome or calcium pyrophosphate crystals in synovial fluid are sufficient to classify a patient as having CPPD disease. In the absence of these findings, a score >56 points using weighted criteria, comprising clinical features, associated metabolic disorders, and results of laboratory and imaging investigations, can be used to classify as CPPD disease. These criteria had a sensitivity of 92.2% and specificity of 87.9% in the derivation cohort (190 CPPD cases, 148 mimickers), whereas sensitivity was 99.2% and specificity was 92.5% in the validation cohort (251 CPPD cases, 162 mimickers).ConclusionThe 2023 ACR/EULAR CPPD disease classification criteria have excellent performance characteristics and will facilitate research in this field.

| S-EPMC10543651 | biostudies-literature

Cardiovascular Disease Risk in Calcium Pyrophosphate Deposition Disease: A Nationwide Study of Veterans.

Project description:ObjectiveCalcium pyrophosphate deposition (CPPD) disease represents a common crystalline arthritis with a range of manifestations. Our goal was to investigate risks for cardiovascular events in patients with CPPD.MethodsWe performed a retrospective matched cohort analysis in the Veterans Health Administration Corporate Data Warehouse, 2010-2014. CPPD was defined by ≥1 International Classification of Diseases, Ninth Revision codes for chondrocalcinosis or calcium metabolism disorder. CPPD patients were age- and sex-matched to approximately 4 patients without codes for CPPD; we excluded patients with a cardiovascular event during the 365 days prior to the index date. Demographic information, traditional cardiovascular risk factors, medications, and health care utilization were assessed at baseline. The primary outcome was a major adverse cardiovascular event (MACE: myocardial infarction, acute coronary syndrome, coronary revascularization, stroke, or death). Secondary outcomes included individual components of MACE. Cox proportional hazards models estimated fully adjusted hazard ratios (HRs) and 95% confidence intervals (95% CIs).ResultsWe identified 23,124 CPPD patients matched to 86,629 non-CPPD patients with >250,000 person-years of follow-up. The study population was 96% male, mean age was 78 years, and 75% were White. The frequency of traditional cardiovascular risk factors was similar between the 2 cohorts. CPPD was not significantly associated with risk for MACE (HR 0.98 [95% CI 0.94-1.02]) in fully adjusted models, though risks of myocardial infarction, acute coronary syndrome, and stroke were significantly higher in the CPPD cohort compared to the non-CPPD cohort.ConclusionCPPD did not confer an increased risk for MACE, a composite end point including all-cause mortality. Our results propose CPPD as a novel risk factor for MACE components, including myocardial infarction, acute coronary syndrome, and stroke.

| S-EPMC8918431 | biostudies-literature

Mutations in osteoprotegerin account for the CCAL1 locus in calcium pyrophosphate deposition disease.

Project description:ObjectiveMutations on chromosomes 5p (CCAL2) and 8q (CCAL1) have been linked to familial forms of calcium pyrophosphate deposition disease (CPDD). Mutations in the ANKH gene account for CCAL2, but the identity of CCAL1 has been elusive. Recently, a single Dutch kindred with a mutation in the Tumor Necrosis Factor Receptor Super Family member 11B (TNFRSF11B) gene coding for osteoprotegerin (OPG) was described as a gain-of-function mutation. Affected family members had premature generalized osteoarthritis (PGOA) and CPDD. As the TNFRSF11B gene is on 8q, we sought additional evidence that TNFRSF11B was CCAL1, and investigated potential disease mechanisms.DesignDNA from two novel PGOA/CPDD families was screened for sequence variants in the TNFRSF11B gene. Mutations were verified by genotype analysis of affected and unaffected family members. We also investigated effects of normal and mutant OPG on regulators of CPP crystal formation in porcine cartilage.ResultsThe identical TNFRSF11B mutation described in the Dutch family was present in two novel PGOA/CPDD families. ANKH was normal in affected patient fibroblasts. Exogenous OPG did not alter ANKH mRNA or protein levels, affect translocation of ANKH to the membrane, nor increase [pyrophosphate (PPi)] or other key regulators of CPDD.ConclusionWe have firmly established the identity of CCAL1 as TNFRSF11B (OPG). Our findings suggest that this mutation produces disease in an ANKH-independent manner via novel mechanisms not primarily targeting cartilage. This work rationalizes further investigation of OPG pathway components as potential druggable targets for CPDD.

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Calcium Pyrophosphate Dihydrate Deposition Disease in Young Patients: Two Case Reports.

Project description:Calcium pyrophosphate deposition disease (CPDD) is a type of arthritis caused by the deposition of calcium pyrophosphate crystals, and may present as either acute or chronic arthritis. Development of CPPD crystal deposition disease in young people may be associated with metabolic diseases such as hemochromatosis, hyperparathyroidism, hypomagnesemia, Wilson's disease, hypothyroidism, gout, acromegaly, and X-linked hypophosphatemic rickets. Therefore, investigations for a predisposing metabolic condition are advised in young-onset polyarticular CPPD crystal deposition disease. In this article, we report two young patients who were investigated for recurrent joint pain due to CPPD disease.

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A rare case of atypical tumoral presentation of calcium pyrophosphate dihydrate crystal deposition disease.

Project description: Not available

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Identifying Potential Classification Criteria for Calcium Pyrophosphate Deposition Disease: Item Generation and Item Reduction.

Project description:ObjectiveClassification criteria for calcium pyrophosphate deposition (CPPD) disease will facilitate clinical research on this common crystalline arthritis. Our objective was to report on the first 2 phases of a 4-phase process for developing CPPD classification criteria.MethodsCPPD classification criteria development is overseen by a 12-member steering committee. Item generation (phase I) included a scoping literature review of 5 literature databases and contributions from a 35-member combined expert committee and 2 patient research partners. Item reduction and refinement (phase II) involved a combined expert committee meeting, discussions among clinical, imaging, and laboratory advisory groups, and an item-rating exercise to assess the influence of individual items toward classification. The steering committee reviewed the modal rating score for each item (range -3 [strongly pushes away from CPPD] to +3 [strongly pushes toward CPPD]) to determine items to retain for future phases of criteria development.ResultsItem generation yielded 420 items (312 from the literature, 108 from experts/patients). The advisory groups eliminated items that they agreed were unlikely to distinguish between CPPD and other forms of arthritis, yielding 127 items for the item-rating exercise. Fifty-six items, most of which had a modal rating of +/- 2 or 3, were retained for future phases. As numerous imaging items were rated +3, the steering committee recommended focusing on imaging of the knee and wrist and 1 additional affected joint for calcification suggestive of CPP crystal deposition.ConclusionA data- and expert-driven process is underway to develop CPPD classification criteria. Candidate items comprise clinical, imaging, and laboratory features.

| S-EPMC8578594 | biostudies-literature

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