Pyrophosphate Dihydrate Crystal Deposition Disease Research Articles

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

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

CT imaging features of calcium pyrophosphate dihydrate crystal deposition involving spine

PurposeTo explore the computed tomography (CT) imaging features of the spine involved by calcium pyrophosphate dihydrate crystal deposition disease (CPPD-CDD) in order to improve the understanding of this disease. Materials and methodsAmong 79 patients diagnosed with CPPD-CDD in our hospital, 51 patients with spinal involvement were selected, 9 patients without CT examination were excluded, and 42 patients were finally included as research objects. The CT images of the 42 patients were retrospectively analyzed. The analysis contents mainly included the distribution of lesions and the characteristic imaging findings of intervertebral disc, posterior ligament complex, sacroiliac joint and atlantoaxial joint. ResultsIn this paper, 42 patients with 11 males and 31 females were studied. The onset age ranged from 62 to 95 years. There were 264 lesions in 42 patients with spinal involvement, including 177 lumbar vertebrae, 43 cervical vertebrae, 27 thoracic vertebrae and 17 sacroiliac joints (14 bilateral and 3 unilateral). Characteristic imaging findings include: disc calcification (129 cases), posterior ligament complex calcification (107 cases), sacroiliac articular cartilage calcification (17 cases), and ligaments around the odontoid process calcification (11 cases). ConclusionsThe characteristic images of CPPD-CDD involving the spine are calcification of intervertebral disc, posterior ligament complex, sacroiliac joint surface and ligaments around odontoid process, and the lesions are frequent and coexisting, so it should be differentiated from gout, rheumatoid arthritis, ankylosing spondylitis, ossification of ligamentum flavum, basic calcium phosphate and infectious spondylodiscitis.

Drugs Recommended in Adult Rheumatic Diseases, But Considered for Off-Label Use in Argentina

BackgroundOff-label (OL) drug use is the prescription of a drug for indications other than those authorised in its technical datasheet. The objective of this study was to identify drugs recommended in rheumatology but considered for off-label use in Argentina. MethodsA list of medications for certain selected rheumatic conditions was compiled. A drug was considered recommended if it was endorsed by a) at least one Argentine or Pan-American treatment guideline or consensus, or b) two international treatment guidelines, or c) one international treatment guideline and one selected textbook. Approval of these drugs for any condition in Argentina until December 31st, 2018 was explored, and medicines were divided into those with on-label indications and those considered for OL use. ResultsOne hundred and thirty-six medications were analysed in 13 clinical conditions. Sixty-seven OL recommendations (49%) were found, and several drugs had more than one. All the conditions included the recommendation of at least 1 OL drug except osteoporosis and rheumatoid arthritis. The frequency of OL recommendations for the following conditions was 100%: calcium pyrophosphate dihydrate crystal deposition disease, polymyalgia rheumatica, Sjögren syndrome, and systemic sclerosis. The drugs with the highest number of OL recommendations were methotrexate (in 7 conditions), and glucocorticoids and mycophenolate (in 4). There were 2 OL recommendations for rituximab and 1 for abatacept. ConclusionsAlmost all the rheumatic disorders analysed involved the recommendation of at least 1 OL medication, and in 4 conditions all the recommendations were OL. Most OL drugs recommended in rheumatology are neither biological nor small-molecule therapies.

Drugs Recommended in Adult Rheumatic Diseases, But Considered for Off-Label Use in Argentina.

Off-label (OL) drug use is the prescription of a drug for indications other than those authorised in its technical datasheet. The objective of this study was to identify drugs recommended in rheumatology but considered for off-label use in Argentina. A list of medications for certain selected rheumatic conditions was compiled. A drug was considered recommended if it was endorsed by a) at least one Argentine or Pan-American treatment guideline or consensus, or b) two international treatment guidelines, or c) one international treatment guideline and one selected textbook. Approval of these drugs for any condition in Argentina until December 31st, 2018 was explored, and medicines were divided into those with on-label indications and those considered for OL use. One hundred and thirty-six medications were analysed in 13 clinical conditions. Sixty-seven OL recommendations (49%) were found, and several drugs had more than one. All the conditions included the recommendation of at least 1 OL drug except osteoporosis and rheumatoid arthritis. The frequency of OL recommendations for the following conditions was 100%: calcium pyrophosphate dihydrate crystal deposition disease, polymyalgia rheumatica, Sjögren syndrome, and systemic sclerosis. The drugs with the highest number of OL recommendations were methotrexate (in 7 conditions), and glucocorticoids and mycophenolate (in 4). There were 2 OL recommendations for rituximab and 1 for abatacept. Almost all the rheumatic disorders analysed involved the recommendation of at least 1 OL medication, and in 4 conditions all the recommendations were OL. Most OL drugs recommended in rheumatology are neither biological nor small-molecule therapies.

Hemochromatosis Arthropathy in Heterozygous HFE H63D Mutation Without Iron Overload- An Entity Less Commonly Touched

Human homeostatic iron regulator protein gene (HFE gene) H63D mutations even when homozygous are rarely associated with iron overload. These mutations, independent of iron status, are associated with calcium pyrophosphate dihydrate crystal deposition disease (CPPD) leading to arthropathy even for heterozygotes. The arthropathy does not respond to iron depletion. We report a case of a 62-year-old male with chronic generalized arthralgias with no evidence of iron overload or elevated inflammatory markers with characteristic radiographic hook-like osteophytes suggestive of hemochromatosis arthropathy. Further, he was found to be a carrier of HFE H63D mutation. Recognition of the association can help guide goal directed management.

Pathophysiology and MRI Findings of Infectious Spondylitis and the Differential Diagnosis

MRI에서 추간판의 이상 신호와 위, 아래 척추체 종판의 파괴, 종판 주변의 골수부종 등은 감염성 척추염의 전형적인 소견으로 여겨지나 퇴행성 척추질환, acute Schmorl's node, 척추관절병증, synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO)/chronic recurrent multifocal osteomyelitis, 척추신경관절병증, calcium pyrophosphate dehydrate 결절침착질환 등 다양한 비감염성 척추질환에서도 나타날 수 있다. MRI에서 이러한 비감염성 척추질환과 감별되는 감염성 척추염의 영상 소견은 추간판의 고신호와 농양, 척추 연부조직의 농양, 그리고 T1 강조영상에서 저신호로 보이는 종판의 경계가 불명확해지는 점 등이다. 그러나 이러한 감별점이 항상 적용되는 것은 아니며 감염성, 비감염성 질환의 영상 소견에 유사점이 많기 때문에 정확한 진단을 위해서는 감염성 척추염뿐만 아니라 감염과 감별해야 하는 다양한 질환의 병태생리와 연관된 영상학적 특징을 아는 것이 중요하다.

Calcium Pyrophosphate Dihydrate (CPPD) Crystal Deposition Disease, Crowned Dens Syndrome (CDS) and Its Mimicries: Case Report

Calcium Pyrophosphate Dihydrate (CPPD) Crystal Deposition Disease, Crowned Dens Syndrome (CDS) and Its Mimicries: Case Report

Calcium Pyrophosphate Dihydrate Crystal Deposition Disease Involving the Ligamentum Flavum of the Cervical Spine with Intense Enhancement on MRI: A Case Report

Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease is characterized by chondrocalcinosis, which mainly affects the knees, wrists, pelvis, and rarely, the spine. According to previous reports, CPPD crystal deposits display heterogeneous enhancement on MRI. When combined with inflammation of the surrounding soft tissue, strong enhancement by CPPD crystal deposition may appear similar to imaging features of other conditions such as infectious spondylitis. In these conditions, CT plays an important role in differential diagnosis. Here, we present a case of CPPD crystal deposition disease in the ligamentum flavum of the cervical spine that showed intense enhancement on MRI.

Acute odontogenic infection combined with crowned dens syndrome: a case report

BackgroundCalcium pyrophosphate dihydrate crystal deposition disease is a condition in which calcium pyrophosphate dihydrate crystal is deposited in joint cartilage and ligaments. Calcium pyrophosphate dihydrate crystal deposition disease that involves calcification around the odontoid process of the second cervical vertebra is called crowned dens syndrome. Crowned dens syndrome is accompanied by fever in addition to acute and intense neck, posterior head, and temporal pain; thus, distinguishing crowned dens syndrome may be difficult in the presence of odontogenic infection. To the best of our knowledge, this is the first report describing a patient with crowned dens syndrome with coexisting odontogenic infection.Case presentationA 75-year-old Japanese woman was examined in the Emergency Department of this hospital due to a chief complaint of worsened buccal swelling on the left side. An odontogenic infection was considered, and she underwent her first examination. She presented with a body temperature of 37.4 °C, marked swelling and tenderness of her left lower eyelid through to her left cheek, and pain on the left temporal area. Blood tests revealed a leukocyte count of 6700/μL and a C-reactive protein level of 7.15 mg/dL. There was swelling and pain around the gingiva and acute purulent apical periodontitis of left maxillary second premolar. Cellulitis of the left cheek was diagnosed. After performing drainage of the pus, antibiotic treatment was initiated. Although her clinical symptoms improved, blood tests on day 9 of hospitalization revealed a leukocyte count of 6500/μL and a C-reactive protein level of 25.62 mg/dL, which were indicative of worsening symptoms. Computed tomography was performed to evaluate remote infection and images revealed a calcification around the odontoid process of her second cervical vertebra. When she was referred to the Orthopedic Surgery Department, pseudogout of the cervical spine was diagnosed. Subsequently, oral acetaminophen was initiated, and both her leukocyte count and C-reactive protein improved markedly.ConclusionsIn the presence of persistent fever and abnormally high leukocyte and C-reactive protein indicative of an inflammatory reaction, coexistence of pseudogout should be considered. In particular, when symptoms of temporal pain are present, the possibility of pseudogout of the cervical spine must be considered in the differential diagnosis.

A case of calcium pyrophosphate dihydrate crystal deposition disease suspected the tumor of temporomandibular joint

A case of calcium pyrophosphate dihydrate crystal deposition disease suspected the tumor of temporomandibular joint

Rare case of pseudogout in the scapular region

Calcium pyrophosphate dihydrate crystal deposition disease (CPPD) also referred to as pseudogout is an inflammatory arthritis produced by the deposition of calcium pyrophosphate crystals in the synovium and periarticular soft tissues. This report documents a case of a 41 year old female who presented with a painful swelling in the back. MRI revealed a complex swelling with lobulation of size 7.4×7.4×7 cm in the posterolateral aspect of chest wall just superficial to the ribcage. A surgical excision biopsy of the swelling was done and patient improved symptomatically. Serum uric acid levels were measured and found to be within limits.

A case of calcium pyrophosphate dihydrate crystal deposition disease presenting as an acute polyarthritis

A case of calcium pyrophosphate dihydrate crystal deposition disease presenting as an acute polyarthritis

Spontaneous rupture of extensor tendons of the index finger in calcium pyrophosphate dihydrate crystal deposition disease

Spontaneous rupture of extensor tendons of the index finger in calcium pyrophosphate dihydrate crystal deposition disease

Referral Patterns for Dual-Energy Computed Tomography in Diagnosis and Management of Gout: Ten-Year Experience at a Canadian Institution

PurposeTo analyze the utilization, indications, and outcomes of dual-energy computed tomography (DECT) gout imaging in clinical practice. MethodsThis retrospective study was ethics approved. Radiology reports of DECT gout scans between 2007 and 2016 were analyzed for trends of utilization, referral pattern, indication, and diagnosis. ResultsDECT gout referrals increased substantially (2007: 37; 2008: 72; 2016: 385; total: 1877). The largest number of referrals were from rheumatology (1160), emergency medicine (283), and family medicine (177). Most referrals (92%) were requested to aid an initial diagnosis of gout. Other reasons included estimating the disease burden (6%) or monitoring disease progression and effectiveness of treatment (2%). Rheumatology accounted for most referrals for the latter two reasons (81% and 97%). Imaging findings of urate presence were similar in referrals from rheumatology (62%), family medicine (62%), and other medical specialties (62%). The urate positive rates were slightly lower in referrals from emergency medicine (47%) and surgical specialties (41%). The most common differential diagnoses by referring specialties were calcium pyrophosphate dihydrate crystal deposition disease (CPPD) and other inflammatory or erosive arthritides (rheumatology, family medicine), CPPD and infections (other medical specialties), infections and fractures (emergency medicine), neoplasm and infections (surgical specialties). ConclusionsThe increasing utilization of DECT for gout imaging validates its clinical value. Varying clinical presentation could explain differences of urate positive rates among specialties. Our results support a multispecialty collaborative approach to the diagnosis and management of gout, with direct access to DECT gout imaging provided to various physician specialties.

Chemical Changes Caused by Calcium Deposition in Forestier - Rotes - Querrol�s Disease

Calcium is involved in many biological processes. Hydroxyapatite provides bone mineralization, maintains cellular signaling, enzyme activity, nerve impulse transmission, muscle contraction and regulates blood clotting and membrane permeability. Calcium depositions may be caused by necrosis following trauma, connective tissue diseases, metabolic diseases, sarcoidosis, myeloma, metastases, chondrocalcinosis or calcium pyrophosphate dihydrate crystal deposition disease, cervical spondylosis, ankylosing spondylitis, ossification of the posterior longitudinal ligament of the spine and Forestier�s disease. Diffuse idiopathic skeletal hyperostosis (DISH) or Forestier-Rotes-Querrol�s disease is characterized by calcification of ligaments and entheses of the anterior vertebrae in the thoracic spine and sacroiliac joint bridging caused by abnormal proliferation of osteoblasts. At least four consecutive thoracic vertebrae are affected and the intervertebral disc space is preserved. Peripheral joints of heels, knees, elbows and pelvis may be also affected. Diagnosis is confirmed by radiographic findings with a candlewax-like appearance down the spine, on C2-C6 vertebrae. A total number of 37 patients with a median age of 63 years, of which 24 were male (64.86%) and 13 female (35.14%), were diagnosed with DISH at the Clinic of Geriatry of Galati County Emergency Clinical Hospital Sf. Apostol Andrei between 2006-2016. Differential diagnosis included ankylosing spondylitis and spondylosis deformans. DISH was found in patients aged over 50 years, with obesity and diabetes, based on radiographic findings. Drug therapy and physical therapy successfully provided pain relief, increased axial and peripheral mobility, improved physical function and optimal quality of life. None of our patients required surgery, as there were no cases with compromised airways and digestive tract involvement. Our study confirms that old age, obesity and type 2 diabetes are risk factors for diffuse idiopathic skeletal hyperostosis.

A case of tophaceous pseudogout of the temporomandibular joint extending to the base of the skull

A case of tophaceous pseudogout (i.e., calcium pyrophosphate dihydrate (CPPD) crystal deposition disease) in the temporomandibular joint (TMJ), extending to the base of the skull, is reported. A 38-year-old man was referred to the hospital with mild pain in the right chin and tip of the tongue. Panoramic radiography showed a large calcified mass around the right TMJ. Computed tomography imaging revealed a large, granular, calcified mass surrounding the right condylar head and extending to the base of the skull. The mass was clinically and radiographically suspected to be a pseudogout lesion. A biopsy specimen was collected under general anaesthesia to confirm the diagnosis. On histology, the mass was found to contain deposits of numerous rod-shaped and rhomboid crystals, which suggested tophaceous pseudogout. The deposits were identified as CPPD crystal deposition, based on analysis by X-ray diffraction and Fourier transform infrared spectroscopy. These two crystallography methods were useful in confirming the diagnosis of CPPD crystal deposition disease in the TMJ.

The prevalence of chondrocalcinosis of the symphysis pubis on CT scan and correlation with calcium pyrophosphate dihydrate crystal deposition disease.

Calcium pyrophosphate dihydrate (CPP) crystal deposition in the articular cartilage can often be seen radiographically as chondrocalcinosis (CC). CPP crystals preferentially deposit in fibrocartilages such as the knee menisci and symphysis pubis (SP). We sought to determine the prevalence of CC in the SP on computed tomography (CT) of the abdomen and pelvis. This retrospective study involved readings on 1070 consecutive CTs of the abdomen and pelvis performed over 3 months in patients over 65 years of age. Medical records of 226 patients found to have CC were reviewed to determine age, gender, documentation of CPPD on problem lists or in medical histories, and whether radiology readings of the CTs mentioned CC. SP CC was identified in 21.1 % (226/1070) of consecutive CT scans with the mean age of CT+ patients being 78.6. Of the 226 patients with SP CC, the observation of CC was documented in only 5.3 % (12/226) of the radiology reports. Of the 12 instances in which the radiology reports mentioned CC, this observation was never (0/12) transmitted to the medical history or problem list. The prevalence of SP CC in patients older than 65 was 21.1 %. Since the majority of CTs of the abdomen and pelvis are not ordered for evaluation of musculoskeletal conditions, this is likely a true prevalence without selection bias. When CC of the SP was present on images, radiologists routinely overlooked or chose not to report CC. Even in the rare instances when it was reported, that information was not added to the medical history or problem list. There are several clinical situations (e.g., acute monoarthritis or atypical osteoarthritis) in which recognizing that a patient has CPP deposition would be useful. Taking the time to review images may yield clinically important findings that are not mentioned anywhere on the patient chart.

Acute Pseudogout of the Neck: "Crowned Dens" Revisited

Calcium pyrophosphate dihydrate crystal deposition disease is associated with an acute mono- or pauciarthritis, termed “pseudogout” in elderly patients, involving a large joint (including the knees, ankles) or a chronic arthropathy manifesting as mild joint pain and stiffness. Pseudogout is a crystal-deposition disease of peripheral joints, usually encountered in elderly patients. However, acute presentation of pseudogout around the odontoid process comprises a “crowned-dens” appearance, and requires contemplation of differential diagnoses. We recently experienced a case of pseudogout in the cervical spine presenting with fever and acute neck pain that was successfully treated with a colchicine and low-dose oral steroid. We reported this case with a review of the relevant literature. (J Rheum Dis 2016;23:122-124)

Fibrin glue for olecranon bursitis: a case report.

Olecranon bursitis is a common cause of elbow pain and swelling. Causes of aseptic inflammation of the olecranon bursa include trauma, gout, calcium pyrophosphate dihydrate crystal deposition disease, rheumatoid arthritis, and systemic lupus erythematosus. Most cases occur in men between the ages of 30 years and 60 years. The inflammatory response within a bursa can lead to permanent epithelial damage and recurrent symptoms. There is little literature to guide therapy for recurrent olecranon bursitis. Commonly used conservative modalities include ice, compression, and activity modification with use of elbow padding. Aspiration, corticosteroid injection, and surgery are often reserved for persistent cases. Weinstein and colleagues studied patients with both acute and chronic olecranon bursitis, finding aspiration alone was sufficient for resolution of effusion in 55% of patients at 2 weeks, 77% at 8 weeks, and 91% at 24 weeks. In contrast, patients who received intrabursal steroid injections improved more quickly, but with more subsequent complications. In the group treated with aspiration and triamcinolone hexacetonide injection, 3 out of 25 patients (12%) developed a septic bursitis compared with none with aspiration only [Weinstein et al. 1984]. A recent review by Baumbach and colleagues suggests that chronic, nonseptic olecranon or prepatellar bursitis that does not resolve after initial aspiration and corticosteroid injection should be treated with bursectomy [Baumbach et al. 2014]. For patients who do not desire to proceed with surgery, the literature offers little further guidance. This case report presents a novel treatment for recurrent olecranon bursitis using a fibrin glue sealant. After failure of conventional therapies, a patient with chronic, refractory nonseptic olecranon bursitis was treated with ultrasound-guided injection of fibrin glue, resulting in complete resolution of his refractory bursitis. This therapy provided a safe and minimally invasive alternative to bursectomy. Fibrin sealants have been used for many years during various types of surgeries and procedures. Its unique properties and minimal risk make it an attractive treatment option for persistent nonseptic olecranon bursitis.

Use of ultrasound for diagnosis and monitoring of outcomes in crystal arthropathies.

In the latest recommendations for the diagnosis and management of gout and calcium pyrophosphate dihydrate (CPPD) crystal deposition disease, the diagnostic potential of ultrasound has been recognized. This review highlights the recent advances of research on ultrasound in gout and CPPD crystal deposition disease. Ultrasound allows highly sensitive, noninvasive and quick detection of microcrystal aggregates in multiple anatomic areas. Ultrasound can be used as a safe and reliable guide to aspirate even minimal fluid collections suitable for microscopic analysis, and as a tool for monitoring monosodium urate crystal dissolution induced by urate-lowering therapy. The first metatarsophalangeal joint and the knee should be regarded as the anatomic regions with the highest probability of being respectively positive for monosodium urate and CPPD crystal aggregates. The detection of highly evocative signs in patients with equivocal clinical findings may have a deep impact on the clinical decision-making process, narrowing the differential diagnostic spectrum and avoiding time-consuming and expensive diagnostic procedures. Ultrasound differential diagnosis between gout and CPPD crystal deposition disease is based on the characteristics of crystal aggregates and their preferential localization in different anatomical areas.