Diagnosis Of Calcium Pyrophosphate Deposition Disease Research Articles

Calcium pyrophosphate deposition disease.

Calcium pyrophosphate deposition (CPPD) disease is a consequence of the immune response to the pathological presence of calcium pyrophosphate (CPP) crystals inside joints, which causes acute or chronic inflammatory arthritis. CPPD is strongly associated with cartilage degradation and osteoarthritis, although the direction of causality is unclear. This clinical presentation is called CPPD with osteoarthritis. Although direct evidence is scarce, CPPD disease might be the most common cause of inflammatory arthritis in older people (aged >60 years). CPPD is caused by elevated extracellular-pyrophosphate concentrations in the cartilage and causes inflammation by activation of the NLRP3 inflammasome. Common risk factors for CPPD disease include ageing and previous joint injury. It is uncommonly associated with metabolic conditions (eg, hyperparathyroidism, haemochromatosis, hypomagnesaemia, and hypophosphatasia) and genetic variants (eg, in the ANKH and osteoprotegerin genes). Apart from the detection of CPP crystals in synovial fluid, imaging evidence of CPPD in joints by mainly conventional radiography, and increasingly ultrasonography, has a central role in the diagnosis of CPPD disease. CT is useful in showing calcification in axial joints such as in patients with crowned dens syndrome. To date, no treatment is effective in dissolving CPP crystals, which explains why control of inflammation is currently the main focus of therapeutic strategies. Prednisone might provide the best benefit-risk ratio for the treatment of acute CPP-crystal arthritis, but low-dose colchicine is also effective with a risk of mild diarrhoea. Limited evidence suggests that colchicine, low-dose weekly methotrexate, and hydroxychloroquine might be effective in the prophylaxis of recurrent flares and in the management of persistent CPP-crystal inflammatory arthritis. Additionally, biologics inhibiting IL-1 and IL-6 might have a role in the management of refractory disease.

OP0205 ULTRASOUND-DETECTED CALCIUM PYROPHOSPHATE CRYSTAL DEPOSITION: WHICH SITES SHOULD BE SCANNED?

Background:In recent years, ultrasonography (US) has emerged as an accurate and reliable tool for the diagnosis of calcium pyrophosphate (CPP) deposition disease (CPPD) in daily practice. Previous studies analyzed the diagnostic value of US findings in different tissues and joints. However, no studies have investigated the optimal US scanning protocol in the diagnosis of CPPD at patient level.Objectives:To assess the diagnostic value of the combinations of OMERACT-defined US findings of CPPD in the upper and lower limbs and to select the best minimal combination of anatomic structures to be scanned for diagnosing CPPD in inter-critical periods.Methods:Patients with a crystal-proven diagnosis of CPPD and age- and sex-matched disease-controls were prospectively enrolled in this cross-sectional, monocentric, case-control study. All subjects underwent a bilateral US examination of 9 hyaline cartilages (HC), 6 fibrocartilages (FC), 5 tendons, 1 joint recess and 1 ligament as follows: shoulder (glenoid FC, humeral HC and acromioclavicular FC), elbow (humeral HC and triceps tendon), wrist (triangular FC, scapho-lunate ligament, volar recess of the radio-lunate joint), hand (HC of the metacarpophalangeal joints from 2nd to 5th finger), hip (acetabular FC and femoral HC), knee (femoral condyles’ HC, meniscal FC, patellar and quadriceps tendons), ankle (talar HC, Achilles tendon and plantar fascia). US assessment was carried-out by a rheumatologist blinded to clinical data. CPP deposits were identified as presence/absence, according to the OMERACT definitions [1].Results:Ninety-five patients were enrolled: 45 CPPD patients (age: 72±10.6 years, disease duration: 5.6±7.8 years, female/male ratio: 1.3) and 50 age- and sex-matched disease-controls (18 with rheumatoid arthritis, 13 with osteoarthritis, 10 with psoriatic arthritis and 9 with gout).The FC of the medial and lateral meniscus were the most frequently involved targets of CPP deposits in cases (81.8% and 77.3% of patients, respectively), followed by the triangular FC of the wrist (68.2%), the HC of the femoral condyles (54.5%), the scapho-lunate ligament (52.3%) and the acetabular FC (50.0%). In all these anatomical targets, US findings indicative of CPP deposits were detected in a significantly higher percentage of cases than controls (p<0.01).The US scanning protocols that showed the best balance between sensitivity and specificity, the most sensitive and the most specific were shown in Table 1.Table 1.Diagnostic performances of different US scanning protocolsAnatomical targetsSESPLH+LH-Knee meniscal FC and wrist triangular FC0.86(0.76-0.96)0.86(0.73-0.94)6.35(3.17-12.72)0.13(0.06-0.30)Knee meniscal FC, wrist triangular FC and hip acetabular FC0.93(0.82-0.99)0.82(0.69-0.91)5.19(2.85-9.42)0.08(0.03-0.24)Hip acetabular FC, knee femoral condyles’ HC andwrist scapho-lunate ligament0.67(0.51-0.80)0.96(0.86-0.99)16.67(4.22-65.82)0.35(0.23-0.53)LH: likelihood ratio, SE: sensitivity, SP: specificity, US: ultrasonography.In all US scanning protocols, each anatomical target was assessed bilaterally.Figure 1 includes representative pictures showing CPP crystal deposits in different anatomical targets.A: Wrist, longitudinal scan of the triangular FC complex.B: Knee, longitudinal scan of the lateral meniscus.C: Hip, longitudinal scan of the acetabular labrum FC,D: Knee, longitudinal scan of the medial femoral condyle’s HC.Arrows: CPP crystal deposits at FC, arrowhead: CPP crystal deposits at HC.Conclusion:Our results suggest that bilateral US assessment of knee, wrist and hip provided acceptable sensitivity and specificity for diagnosing CPPD.

Age-related features of calcium pyrophosphate deposition disease

Calcium pyrophosphate deposition disease (CPPD) is more common at an old age. The age-related features of the disease have not been studied. Objective : to study age-related features of CPPD. Subjects and methods . A total of 113 patients (54 men and 59 women) who had received inpatient or outpatient treatment at the V.A. Nasonova Research Institute of Rheumatology were retrospectively analyzed. The inclusion criteria were age older than 18 years, crystal-verified diagnosis of CPPD, and a signed informed consent. The patients were divided into two groups: 1) older than 55 years (n=38) and younger than 55 years (n=75). The investigators compared the frequency of clinical symptoms, including phenotypes in accordance with the EULAR recommendations, the intensity of pain with a visual analogue scale (VAS), the need for symptomatic therapy, detection rates for chondrocalcinosis (CC) in the knee and wrist joints by radiography, anthropometric and laboratory (the serum levels of C-reactive protein (CRP), creatinine, uric acid, parathyroid hormone, magnesium, phosphorus, and total calcium) features, the presence of CPPD-associated factors (a history of joint injuries, family CC cases, hypomagnesemia, hyperparathyroidism (HPT), hemochromatosis, hypomagnesemia, rheumatoid arthritis (RA), gout, chronic kidney disease (CKD) Stage >3, and use of diuretics). Results and discussion . Thirty-eight (33.6%) of the 113 examined patients with CPPD were aged less than 55 years. The most common clinical form of CPPD was chronic arthritis (n=54; 47.8%), the less common forms were osteoarthritis (OA) with calcium pyrophosphate (CPP) crystals (n=35; 31%) and acute arthritis (n=24; 21.2 %); their rates did not differ in the formed groups. In patients older than 55 years, the pain intensity according to VAS was higher than that at a younger age (50 [40; 70] mm and 40 [25; 54] mm, respectively; p 5 mg/dL was comparable for both groups. Family CPPD cases were recorded in two patients less than 55 years of age. Knee joint radiography significantly more frequently revealed CC in 65 (57.5%) patients aged older than 55 years than that at a younger age (68 and 36.8%, respectively; p=0.001). Hand radiography detected CC in 21 (18.6%) patients, it was also more common in those aged older than 55 years (25.3 and 5.3%, respectively; p=0.001). The CPPD-associated factors include OA, gout, HPT, RA, and CKD >3 Stage; these diseases detected in this study were seen in 91 (80.5%), 28 (24.8%), 14 (12.4%), 5 (4.4%), and 12 (11%) cases, respectively. There were no significant differences in the detection rates for these diseases in patients younger and older than 55 years of age. Also, one patient had hypomagnesemia and one patient had hemochromatosis; both were younger than 55 years old. Conclusion. The prevalence of CPPD at a young age can be underestimated: 33.6% of the patients with CPPD confirmed by the detection of CPP crystals in synovial fluid were aged less than 55 years. Moreover, the frequency of individual phenotypes and main CPPD-associated factors is identical in different age groups. At the age of older than 55 years, CPPD is characterized by the more frequent detection of radiographic signs of CC, a greater need for NSAIDs and colchicine, and a high level of CRP.

THE PREVALENCE AND CLINICAL FEATURES OF GOUT AND CALCIUM PYROPHOSPHATE DEPOSITION DISEASE IN PATIENTS WITH ACUTE ARTHRITIS

Objective: to study the rate of gout and calcium pyrophosphate deposition disease (CPDD) and to provide their clinical characteristics in patients with acute arthritis. Subjects and methods . The investigation enrolled 150 patients (97 men and 53 women) with acute mono- or oligoarthritis of no more than 2 weeks’ duration who had visited the Outpatient Department of V.A. Nasonova Research Institute of Rheumatology. Their mean age was 60.2±12.0 years (range, 28–76 years). All the patients underwent inflamed joint puncture and synovial fluid (SF) crystal identification by polarized microscopy using an Olympus CX31-P compensator.The diagnosis of CPDD was established in compliance with the criteria elaborated by D. McCarty. Gout was diagnosed only when sodium monourate (SMU) crystals were found in SF. Results and discussion. The investigation revealed gout in 51 (34%) patients, CPDD in 45 (30%), coincidence of gout and CPDD in 15 (14%), and 39 (26%) patients had other diseases: 15 (10%) – osteoarthritis, 6 (4%) – rheumatoid arthritis, 5 (3%) – septic arthritis, 5 (3%) – psoriatic arthritis, 2 (1%) – ankylosing spondyloarthritis, 4 (3%) – injury, and 5 (3%) – undifferentiated spondyloarthritis. There were 40 men and 11 women among the patients with gout and 13 men and 32 women among those with CPDD. The patients with gout were younger than those with CPDD (35.5±8.9 and 58.4±12.8 years, respectively; p < 0.05). In the patients with CPDD, knee, ankle, and first metatarsophalangeal joint arthritis was present in 34 (76.3%), 17 (30.3%), and 4 (9%) cases, respectively. Arthritis developed within a few hours in 90% of the patients with gout and in 33.3% of those with CPDD (p < 0.001). Monoarthritis was significantly more common in gout whereas oligoarthritis – in CPDD (p < 0.05). Visual analog scale pain intensity averaged 78.4±12.5 mm and 54.32±22.02 mm (p < 0.05), duration of arthritis – 10.3±3.8 days and 3.4±2.1 days (p < 0.05) in gout and CPDD respectively. Conclusion. CPDD is the most common (60%) cause of acute arthritis in women and the second most frequent (13%) cause in men (following gout). Acute arthritis develops more slowly and persists for a longer period of time in patients with CPDD than in those with gout.

Imaging of calcium pyrophosphate deposition disease.

Calcium pyrophosphate deposition disease (CPPD) is a common and clinically heterogeneous form of arthritis caused by the deposition of calcium pyrophosphate (CPP) crystals in articular tissues. The diagnosis of CPPD is supported by the presence of radiographic chondrocalcinosis; yet, conventional radiography detects only about 40% of clinically important CPPD. Here, we critically review the recent literature on imaging in CPPD. New studies inform our use of conventional radiographic screening methodologies for CPPD and provide additional evidence for the utility of diagnostic ultrasound. Recent work also highlights the polyarticular nature of CPPD, its association with tissue damage, and the high prevalence of tendon involvement. While dual energy CT and diffraction-enhanced synchrotron imaging remain research tools, they present potential avenues for improved visualization of CPP deposits. Advances in imaging in CPPD will increase diagnostic accuracy and eventually result in better management of this common form of arthritis.

SAT0526 The Frequency of Calcium Pyrophosphate Deposition Disease in Patients with Acute Arthritis

BackgroundMost of the patients with acute arthritis are diagnosed gout. However, the frequency of Calcium Pyrophosphate Deposition Disease (CPPD) in patients with acute arthritis is not enough valuated.ObjectivesTo evaluate the...

AB1057 Osteoporosis in calcium pyrophosphate deposition disease

ObjectivesTo study the frequency of osteoporosis (OP) in patients with calcium pyrophosphate deposition disease (CPPD).Methods85 pts with crystal-proven diagnosis of CPPD and chondrocalcinosis phenomena, confirmed either by conventional radiography (Rg)...

Tumoural calcium pyrophosphate dihydrate crystal deposition disease presenting clinically as a malignant soft tissue mass diagnosed on fine needle aspiration biopsy

Tumoural calcium pyrophosphate dihydrate crystal deposition is a rare manifestation of calcium pyrophosphate deposition disease (CPPD). We present the case of a 75-year-old male with a previously resected rectal adenocarcinoma who developed a 5-cm right-sided mass at the base of his neck. Clinically and radiologically the lesion was suspicious for malignancy, possibly of metastatic origin. A bedside fine needle aspirate was performed and the smears were mildly cellular showing histiocytes with numerous intracellular and extracellular crystals. These colourless crystals were mostly short and rhomboid shaped and demonstrated weakly positive birefringence. A diagnosis of tumoural CPPD was made. This case is only the second in the English literature diagnosed on fine needle aspiration biopsy. Tumoural CPPD is well known to be a clinical, radiological and occasionally pathological mimic of malignancy. Several cases have been reported where unnecessary radical surgery was performed for this condition. Fine needle aspiration biopsy, as in this case, can provide a rapid and accurate diagnosis of CPPD, avoiding the need for invasive procedures. Polarisation microscopy is a vital adjunct to confirm this diagnosis.

Degeneration of the Scaphoid-Trapezium Joint: A Useful Finding to Differentiate Calcium Pyrophosphate Deposition Disease from Osteoarthritis

This study aimed to determine whether osteoarthritis of the scaphoid-trapezium joint (ST osteoarthritis) is associated with calcium pyrophosphate deposition disease (CPDD) in an elderly population with or without concomitant polyarthritis of the finger joints (FIPO). An age- and gender matched case-control study was performed at a university hospital outpatient clinic. Cases and controls were identified from a clinical registry. The case ascertainment process included: (1) chart review for evidence of pyrophosphate crystals from arthrocentesis and/or cartilage calcifications and (2) blinded reading of hand X-rays by three observers for calcification of the triangular fibrocartilage and/or cartilage calcification around the spatium triangulare. Osteoarthritis was graded from 0 to 4 according to the Standard Atlas of Radiographs. The association of ST osteoarthritis with the diagnosis was examined using chi2 tests or the Wilcoxon rank sum test as appropriate. From 65 potential cases, 30 fulfilled the inclusion/exclusion criteria whereas from 185 potential controls, 81 fulfilled the inclusion/exclusion criteria. Thirty controls were matched to cases for gender and age. ST osteoarthritis was much more severe in CPDD (median: 3.0) than in patients with FIPO (median: 0.3) and was strongly associated with the diagnosis (odds ratio 13.8; CI 3.4-59.8). Definite ST osteoarthritis identified CPDD with a sensitivity of 83% and a specificity of 73% with regard to FIPO. It was concluded that the presence of ST osteoarthritis is a helpful diagnostic finding for the diagnosis of CPDD in an elderly, predominantly female population with a high prevalence of FIPO. Especially in cases without radiographic cartilage or fibrocartilage calcification of the wrist, ST osteoarthritis may point to the correct diagnosis.