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Purpose of the Review: This review provides an overview of medical conditions and risk factors associated with CPPD. Recent Findings: Recent studies have indicated that CPPD patients may have a higher risk for systemic conditions such as cardiovascular diseases. Summary: Calcium pyrophosphate deposition disease (CPPD) is a common crystal arthropathy that primarily affects older adults, and, in most cases, the aetiology is idiopathic. Age is the most remarkable risk factor and due to the aging population, the prevalence of this condition is expected to increase. Strong evidence supports an association between CPPD and several metabolic and endocrine conditions, including hemochromatosis, hyperparathyroidism, hypomagnesemia, and hypophosphatasia. Additionally, there is growing evidence of an increased risk for cardiovascular diseases among CPPD patients, alongside potential links to rheumatic disorders, gender, medications, and joint trauma. Further research is needed to explore the underlying mechanisms linking CPPD to associated conditions and to develop targeted therapies with the aim of improving patient outcomes. [ABSTRACT FROM AUTHOR]

Calcium pyrophosphate dihydrate deposition disease (CPDD or pseudogout) is a degenerative joint disease. It is defined by the presence of calcium pyrophosphate dihydrate crystals. It usually manifests in the knee and wrist. Manifestation in the temporomandibular joint (TMJ) is only reported in case reports. We present a patient with CPDD mimicking a malignant tumor of the TMJ. A 53-year-old woman presented with progressive pain and a slow-growing swelling of the left TMJ. Imaging showed an extensive mass in the infratemporal fossa extending into the middle cranial fossa and compressing the temporal lobe. Assuming a potential malignancy, we excised the growth, which extended into the dura. We covered the resulting tissue defect within the primary surgery using a microsurgically anastomosed scapular flap and performed further reconstructive surgeries. Calcium pyrophosphate dihydrate crystals were found in the histopathologic examination of the excised tissue, resulting in the diagnosis of CPDD. That is a benign diagnosis, but we treated it like a malignancy. This leads us to the question, was there overtreatment? Tumoral CPDD in the TMJ can be a difficult diagnosis to obtain. The treatment remains controversial, but complete excision of the mass was performed in most reported cases. [ABSTRACT FROM AUTHOR]

Purpose of Review: This article aims to review the challenges to diagnosis and management of calcium crystal deposition diseases and evaluate the literature published over the past 3 years. Recent Findings: The awaited development of classification criteria is an essential step in the progression of calcium crystal deposition disease clinical research. There have been recent improvements in the accuracy of imaging for the diagnosis of crystal deposition diseases with published definitions of characteristic features. Factors associated with acute flares of disease have been identified and an association with increased cardiovascular risk has been demonstrated. Targeted treatment options for calcium crystal diseases remain elusive. However, there have been advances in understanding the molecular mechanisms of disease revealing potential targets for future drug development. Summary: Calcium-crystal deposition diseases are increasing in incidence and prevalence as populations age and continue to associate with a high burden of disability. Despite this, calcium crystal deposition disease remains under-studied with a paucity of evidence-based treatment guidelines. [ABSTRACT FROM AUTHOR]

In this 2-year retrospective case series, we characterise the calcium pyrophosphate deposition disease (CPPD) inpatient cohort at a single centre and assess the efficacy and safety of anakinra in its treatment. Adult inpatients with CPPD between 1st September 2020 and 30th September 2022 were identified by ICD-10 codes and confirmed based on clinical diagnosis and either CPP crystals on aspirate or chondrocalcinosis on imaging. Charts were reviewed for demographic, clinical, biochemical data, treatment choice, and response. Response to treatment was determined from chart documentation and calculated from time of first CPPD treatment. Daily responses to anakinra were recorded if anakinra was used. Seventy patients accounting for 79 cases of CPPD were identified. Twelve cases received anakinra, whilst 67 cases received conventional therapy only. Patient receiving anakinra were predominantly male, had multiple comorbidities, and had higher CRPs and serum creatinine when compared to the non-anakinra group. Anakinra was rapidly effective with the mean time to substantial and complete response being 1.7 and 3.6 days respectively. Anakinra was well tolerated. This study adds to the small amount of retrospective data present about the use of anakinra in CPPD. We observed a rapid response to anakinra in our cohort with minimal adverse drug reactions. Key Points • Treatment of CPPD with anakinra appears to be rapidly efficacious without safety concerns. [ABSTRACT FROM AUTHOR]

Tophaceous pseudogout is a rare variant of the calcium pyrophosphate dihydrate (CPPD) disorder, with predilection for the TMJ. It is characterized by calcific deposits composed of rhomboid- or rectangular-shaped crystals that exhibit birefringence when examined under polarized light. We hereby present a case of a 65-year-old man with left pre-auricular tenderness and malocclusion. CT imaging was notable for a left TMJ mineralized mass with erosion of the condylar head. Treatment involved excision of the mass with eminectomy, condylectomy, and a stock total TMJ reconstruction. In this report, important considerations for diagnosis, biopsy, and surgical treatment with emphasis on reconstruction for tophaceous pseudogout of the TMJ have been highlighted by the authors. [ABSTRACT FROM AUTHOR]

Objectives: Arthritis is a common clinical manifestation of hereditary hemochromatosis (HH), and HH is one of a handful of conditions linked to calcium pyrophosphate deposition (CPPD) in joints. The connection between these two types of arthritis has not yet been fully elucidated. In light of new pathogenic pathways recently implicated in CPPD involving bone, we reviewed the literature on the etiology of hemochromatosis arthropathy (HHA) seeking shared pathogenic mechanisms. Results: Clinical observations reinforce striking similarities between HHA and CPPD even in the absence of CPP crystals. They share a similar joint distribution, low grade synovial inflammation, and generalized bone loss. Excess iron damages chondrocytes and bone cells in vitro. While direct effects of iron on cartilage are not consistently seen in animal models of HH, there is decreased osteoblast alkaline phosphatase activity, and increased osteoclastogenesis. These abnormalities are also seen in CPPD. Joint repair processes may also be impaired in both CPPD and HHA. Conclusions: Possible shared pathogenic pathways relate more to bone and abnormal damage/repair mechanisms than direct damage to articular cartilage. While additional work is necessary to fully understand the pathogenesis of arthritis in HH and to firmly establish causal links with CPPD, this review provides some plausible hypotheses explaining the overlap of these two forms of arthritis. [ABSTRACT FROM AUTHOR]

Calcium Pyrophosphate Crystal Deposition (CPPD) disease is characterized by the deposition of calcium pyrophosphate crystals in the cartilage. In most cases, it can manifest as a subclinical condition named chondrocalcinosis, often revealed by joint x-ray examination. In other cases, deposition can cause flares of arthritis, known as acute CPP crystal arthritis. In the last few years, many pathogenic pathways have been discovered. Interleukin-1 (IL-1) plays a key role in the pathogenesis of CPPD disease, both as a mediator of inflammatory response to crystals and as a promoter of damage to articular cartilage. In this review, we investigated the role of IL-1R inhibitor, such as Anakinra, as an alternative to the various therapeutic strategies for CPPD disease, especially among patients resistant to traditional treatment with NSAIDs, corticosteroids and colchicine. [ABSTRACT FROM AUTHOR]

Purpose: The authors present a rare case of calcium pyrophosphate deposition disease (CPPD) in the temporomandibular joint (TMJ), successfully treated with complete resection and immediate reconstruction using a custom-made prosthesis. This one-stage approach has prevented the patient from requiring two surgeries and has proven effective in the management of the condition. Methods: Our patient, a 56-year-old male, presented with left TMJ pain and trismus. Investigations showed a destructive left TMJ mass, confirmed by biopsy to be tophaceous pseudogout. The treatment involved excising all the affected tissues followed by condylectomy, coronoidectomy, and a total TMJ replacement. The patient recovered well from surgery with good mouth opening. Results: CPPD disease is a metabolic arthropathy of poorly understood aetiology. Management options vary from conservative non-surgical to surgical treatment depending on the severity. Conclusion: This case demonstrates the feasibility of a one-stage procedure with reconstruction using a custom-made prosthesis and virtual planning. [ABSTRACT FROM AUTHOR]

Calcium pyrophosphate dihydrate crystal deposition (CPPD), also known as pseudogout, can have spinal manifestations in roughly one quarter of patients. We present a rare, intradural manifestation of CPPD requiring surgical intervention, with a review of pertinent differential diagnoses on imaging. A 48-year-old male presented with urinary retention, and was found to have an intradural lesion with peripheral enhancement on gadolinium T1-weighted magnetic resonance imaging. Due to the patient's progressive neurological deterioration, he was taken for a minimally invasive approach for resection of the lesion. Histopathological analysis revealed crystal deposits with rhomboidal birefringence consistent with CPPD. The imaging features of this lesion were atypical for any of the traditional intradural extramedullary lesions. Typically seen extradurally, recognizing CPPD as a potential culprit for intradural compression is helpful to recognize for providers. [ABSTRACT FROM AUTHOR]

The aim of this study was to evaluate the effects of low-level laser therapy using the gallium arsenide laser (λ = 830 nm) on the articular cartilage (AC) organization from knee joint in an experimental model of microcrystalline arthritis in adult male Wistar rats. Seventy-two animals were divided into three groups: A (control), B (induced arthritis), and C (induced arthritis + laser therapy). The arthritis was induced in the right knee using 2 mg of Na4P2O7 in 0.5 mL of saline solution. The treatments were daily applied in the patellar region of the right knee after 48 h of induction. On the 7th, 14th, and 21st days of treatment, the animals were euthanized and their right knees were removed and processed for structural and biochemical analysis of the AC. The chondrocytes positively labeled for the TUNEL reaction were lower in C than in B on the 14th and 21st days. The content of glycosaminoglycans and hydroxyproline in A and C was higher than B on the 21st day. The amount of tibial TNF-α in B and C was lower than in A. The amount of tibial BMP-7 in B and C was higher than in A. The femoral MMP-13 was lower in B and C than for A. The tibial TGF-β for C was higher than the others. The femoral ADAMT-S4 content of A and C presented similar and inferior data to B on the 21st day. The AsGa-830 nm therapy preserved the content of glycosaminoglycans, reduced the cellular changes and the inflammatory process compared to the untreated group. [ABSTRACT FROM AUTHOR]

To test the performance of a billing claims-based calcium pyrophosphate deposition disease (CPPD) algorithm for identifying pseudogout. We applied a published CPPD algorithm at an academic institution and randomly selected 100 patients for electronic medical record review for 3 phenotypes: (1) definite/probable CPPD, (2) definite/probable pseudogout; (3) definite pseudogout. Clinical data were recorded and positive predictive value (PPV) (95% CI) for each phenotype was calculated. We then modified the published algorithm to require ≥ 1 of 4 relevant terms (“pseudogout”, “calcium pyrophosphate crystals”, “CPPD”, or “chondrocalcinosis”) through automated text searching in clinical notes, and re-calculated PPVs. To estimate the percentage of pseudogout patients not identified by the published algorithm, we reviewed a random sample of 50 patients with ≥ 1 of 4 relevant terms in clinical notes who did not fulfill the published algorithm. Among patients fulfilling the published algorithm, 68% had ≥ 1 of 3 phenotypes. The published algorithm had PPV 24.0% (95% CI 19.3-28.7%) for definite/probable pseudogout and 18.0% (95% CI 14.5-21.5%) for definite pseudogout. Requiring ≥ 1 of 4 relevant terms in clinical notes increased PPV to 33.3% (95% CI 26.8-39.8%) for definite/probable pseudogout and 24.6% (95% CI 19.8-29.4%) for definite pseudogout. Among patients not fulfilling the published algorithm, 16.0% had definite/probable pseudogout and 6.0% had definite pseudogout. A billing code-based CPPD algorithm had low PPV for identifying pseudogout. Adding text searching modestly enhanced the PPV, though it remained low. These findings highlight the need for improved approaches to identify pseudogout to facilitate epidemiologic studies. [ABSTRACT FROM AUTHOR]

According to recent hypothesis, the inflammation has a pivotal role in the onset and progression of erosive hand osteoarthritis (EHOA), psoriatic arthritis (PsA) and chondrocalcinosis (CC)/pseudogout. Albeit, it has been recognised for years as an association between EHOA and radiographic evidence of CC, but there are few reports of coexistence of microcrystalline arthritis and PsA. This is the first report that described a clinical experience concerning two consecutive cases of patients presented with EHOA, PsA and pseudogout. Two Caucasian women of 71 and 85 years old with a history of OA and mild psoriasis are presented with tenderness and swelling of first interphalangeal (IP) and wrist joint, respectively. Arthrocentesis performed at the first IP and wrist joint, respectively, showed an inflammatory synovial fluid with presence of calcium pyrophosphate dehydrate crystals. X-rays of hands, feet and knees showed characteristic features of EHOA, PsA and CC. Furthermore, HLA typing evinces the presence of HLA C*06; DRB*01 07 and HLA C*07; DRB*01 *11 alleles, respectively, predisposing factors of these inflammatory diseases. The relationship between these aggressive rheumatic diseases along with their clinical, radiographic, laboratory and genetic features is discussed. [ABSTRACT FROM AUTHOR]

The protein product of the progressive ankylosis gene, known as ANK, is a 492-amino acid multi-pass transmembrane protein. This protein is critical for the regulation of pyrophosphate, and gain of function ANK mutations is associated with calcium pyrophosphate deposition disease. Much about the structure, function, and regulation of ANK remain unstudied. This review of the current literature examines recent contributions to our understanding of ANK. We focus on new work on the function, binding partners, and regulators of ANK. A more complete understanding of this important protein may help to identify future therapeutic targets for the treatment of calcium pyrophosphate deposition disease. [ABSTRACT FROM AUTHOR]

ute pseudogout of the neck, also known as the crowned dens syndrome, is a rare cause of neck pain characterised by crystalline deposition in periodontoid articular tissues. It is typified clinically by severe cervical pain and stiffness, often in conjunction with pyrexia and raised inflammatory markers. As such, it is often misdiagnosed. We report 2 cases of crowned dens syndrome masquerading respectively as meningitis and polymyalgia rheumatica, and review the literature with particular attention to the clinical and radiological aspects of this under-recognised condition. [ABSTRACT FROM AUTHOR]

Calcification of the ligamentum flavum secondary to calcium pyrophosphate dihydrate (CPPD) deposition may be a rare cause of cervical myelopathy. We present a 56-year-old man with spinal stenosis secondary to CPPD disease and subacute cervical myelopathy following minor trauma. The patient had no history of CPPD disease. Posterior C4-C6 decompression and instrumented fusion were performed. Intraoperative findings were densely thickened ligamentum flavum and distortion of the cervical dura mater due to impingement. Histological sections of discs and ligamentum flavum specimens showed CPPD crystals. Postoperatively, neck pain and paresthesias improved immediately. At 2 weeks after spinal surgery, the patient experienced acute right knee swelling. Standard radiographs showed chondrocalcinosis of the knee menisci. [ABSTRACT FROM AUTHOR]

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A report of two patients in which a soft tissue mass, initially regarded as a malignant tumor, was shown to be the result of calcium pyrophosphate deposition disease. The first case, a woman aged 71 years, presented with a mass involving the right fifth finger. In the second case, also a woman aged 71 years, the lesion involved the tissues adjacent to the right hip. Each lesion consisted of a mass of highly cellular tissue containing deposits of calcium pyrophosphate dihydrate crystals. The clinical, radiological, and pathological features of the two cases are compared with those of seven similar cases reported in the literature. [ABSTRACT FROM AUTHOR]

The wrist is a frequent site of arthropathy in the pscudogout syndrome (calcium pyrophosphate dihydrate crystal deposition disease). Three cases of severe wrist arthropathy are presented to emphasize the destructive changes which may occur, including necrosis or collapse of the lunate and navicular bones. The roentgenographic findings characteristic of pseudogout in the wrist are discussed. [ABSTRACT FROM AUTHOR]

We report the case of a 74-year-old woman who presented with acute-onset right groin pain irradiating to the thigh anteriorly after having suffered for a few weeks from slight knee pain. As a CT scan showed multiple herniated intervertebral discs and spinal stenosis at the L3-L4 level, she was referred to a neurosurgical unit with the tentative diagnosis of L2-L3 radicular pain. Investigations (MR, myelography with CT scan) showed severe acquired lumbar canal stenosis. Decompression surgery was finally postponed because of the patient's serious cardiac medical history and she was referred to us for conservative treatment. She was found to have iliopsoas bursitis with chondrocalcinosis of the knee. Local steroid injections of the two sites abolished her symptoms. We draw attention to the possible pitfalls that the radiographic appearance and one of the multiple clinical presentations of this unrare pathology may represent. Whenever a patient comes walking with crutches, avoids putting weight on his or her leg, and radicular pain is suspected, we advise consideration of other extra-spinal causes for the pain. [ABSTRACT FROM AUTHOR]

Proteoglycans are recognized to inhibit calcium pyrophosphate dihydrate (CPPD) crystal formation but the mechanisms are not known. To study the role of carboxylate (-CO2-) ligands, the possible inhibitor effects of sodium acetate, sodium D-glucuronate, disodium malate, and trisodium citrate were studied using solution mixtures containing [Ca2+] = 1.5 mM, [Mg2+] = 0.5 mM, [PPi] = 0.1 mM, [Pi] = 0.1 mM, [Na+] = 140 mM, 37 degrees C, pH 7.4 with or without 9.5 +/- 0.5 mg CPPD (seed) crystals. These studies showed that monocarboxylates (acetate, glucuronate) have little inhibitive effect. Progressively greater inhibition was found with dicarboxylate (malate) and tricarboxylate (citrate) indicating that the arrangement of carboxylate (and sulfate) ligands on proteoglycan is more important than the inhibitory effect of individual ligands. [ABSTRACT FROM AUTHOR]

Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease is conventionally classified into cases that are hereditary, idiopathic (sporadic) or associated with other disorders. In the idiopathic form, the disease usually occurs in middle-aged or elderly patients. An earlier age of disease onset is observed in the hereditary form and the form associated with other disorders. Therefore, the occurrence of CPPD crystal deposition disease in a young patient merits thorough investigation for an underlying cause such as haemochromatosis, hyperparathyroidism, Wilson's disease, hypophosphatasia or hypomagnesaemia and requires a family study to investigate a possible hereditary cause. We report a case of a young female patient who presented with pseudogout at the age of 24 years; no associated diseases or familial occurrence were found despite a follow-up of more than 12 years. [ABSTRACT FROM AUTHOR]

Purpose: We report the unusual case of a 63-year-old man with gout and coexisting pseudogout (calcium pyrophosphate dihydrate crystal deposition disease) affecting the same joint. In this report, we describe the clinical features of the patient in addition to the characteristics of the crystals extracted from his affected joint, examined using compensated polarized microscopy. Methods: Synovial fluid and specimens of the lateral meniscus of the patient's knee joint taken during arthroscopy were analyzed using compensated polarized microscopy to evaluate the presence and type of crystals. Results: Compensated polarized microscopy revealed both monosodium urate (MSU) and calcium pyrophosphate dihydrate (CPPD) crystals extracted from the knee joint. Conclusions: Our findings suggest that the coexistence of gout and pseudogout should be considered in the diagnosis of crystal-induced arthritis. Careful crystal evaluation using compensated polarized microscopy may reveal similar cases with MSU and CPPD crystals in the same joint. [ABSTRACT FROM AUTHOR]

Study design:Retrospective case study.Objectives:Report a rare case of cervical myelopathy induced by calcium pyrophosphate dehydrate (CPPD) deposition in multiple cervical levels.Setting:An area teaching hospital in Taiwan.Method:A patient with cervical myelopathy was evaluated by computerized tomography (CT) scan and magnetic resonance (MR) image. CPPD deposition known as pseudogout was diagnosed and approved by a polarized microscope.Result:A prominent hypertrophy of ligmentum flavum and a retro-odontoid bulging mass induced cord compression were found in CT scan and MR image. CPPD deposition was confirmed by the histological examinations in the ligamentum flavum at the spinal levels of C3–C6. After decompression surgery of spine and comprehensive rehabilitation, the patient's neurological symptoms subsided and her neurological functions improved leading to a good prognosis.Conclusion:CPPD deposition in cervical spine occurring at multiple levels is rare. Image studies with CT scan and MR are complementary in the diagnosis of CPPD-induced myelopathy. Surgical decompression is always required and expected to have a good outcome.Spinal Cord (2006) 44, 692–694. doi:10.1038/sj.sc.3101890; published online 13 December 2005 [ABSTRACT FROM AUTHOR]

Pseudogout is an uncommon complication of arthroplasty, so it is important to distinguish pseudogout from infection. We experienced three patients with the acute onset of pseudogout after unicondylar knee arthroplasty (UKA). Treatment with nonsteroidal anti-inflammatory drugs (NSAID) and cooling improved the symptoms of all three patients, and there have been no further episodes. Because there is a large area uncovered by the UKA prosthesis, it is necessary to keep in mind the possibility of pseudogout affecting the operated knee. [ABSTRACT FROM AUTHOR]

We present an unusual case of tophaceous pseudogout in the atlantoaxial joint with progressive cervical cord compression symptoms and lack of additional clinical manifestations of CPPD crystal deposition disease. This represents only the fourth reported case in the medical literature. [ABSTRACT FROM AUTHOR]

Purpose of Review: This paper covers confusion and challenges in the nomenclature of calcium pyrophosphate deposition disease. Clinicians, investigators, and patients are faced with a variety of terms that are used to describe CPPD and its phenotypes, and clarity is greatly needed to help advance research and patient care. Motivation for the upcoming development of CPPD classification criteria is reviewed. Recent Findings: EULAR proposed recommended terminology for CPPD in 2011. International Classification of Diseases (ICD-9 and ICD-10) billing codes identify definite or probable CPPD with variable accuracy depending on the clinical setting and comparator group. READ diagnostic codes have been employed to identify pseudogout in UK datasets but their accuracy has not been evaluated. CPPD classification criteria will provide a system for identifying a relatively homogenous group of patients to be included in clinical studies, enabling comparison of outcomes across studies. Summary: CPPD nomenclature remains challenging for clinicians, investigators, and patients. A lay-friendly definition of CPPD, using easily accessible terminology, would be welcome. CPPD classification criteria are a necessary step in moving forward CPPD clinical research and may involve a range of clinical, laboratory, and imaging modalities. [ABSTRACT FROM AUTHOR]

Background: Calcium pyrophosphate dihydrate deposition disease (CPDD) is a rare disease in the temporomandibular joint (TMJ) space. It forms a calcified crystal mass and induces a limitation of joint movement.Case presentation: The calcified mass in our case was occupied in the left TMJ area and extended to the infratemporal and middle cranial fossa. For a complete excision of this mass, we performed a vertical ramus osteotomy and resected the mass around the mandibular condyle. The calcified mass in the infratemporal fossa was carefully excised, and the segmented mandible was anatomically repositioned. Scanning electronic microscopy (SEM)/energy-dispersive X-ray spectroscopy (EDS) microanalysis was performed to evaluate the calcified mass. The result of SEM/EDS showed that the crystal mass was completely composed of calcium pyrophosphate dihydrate. This result strongly suggested that the calcified mass was CPDD in the TMJ area.Conclusions: CPDD in the TMJ is a rare disease and is difficult to differentially diagnose from other neoplasms. A histological examination and quantitative microanalysis are required to confirm the diagnosis. In our patient, CPDD in the TMJ was successfully removed via the extracorporeal approach. SEM/EDS microanalysis was used for the differential diagnosis. [ABSTRACT FROM AUTHOR]

A 72-year-old man, treated with a 60-mg intravenous infusion of pamidronate for shoulder hand syndrome, developed after few hours an acute pseudogout arthritis of his right knee. Diagnosis was confirmed by synovial fluid analysis and was associated with hypocalcemia. Only a few cases are reported in the literature. The mechanism of this potential side effect remains speculative. [ABSTRACT FROM AUTHOR]

The article presents a letter to the editor about the acute pseudogout which is characterized by acute monoarthritis.