Mineral and Bone Disorder in Chronic Kidney Disease: A Case Report from Vietnam.

We report a case of calcium pyrophosphate dihydrate deposition disease (CPDD) involving a patient on maintenance hemodialysis (MHD). The 32-year-old man presented in August 2016 with a complaint of left shoulder swelling of 8 months' duration with no trauma or fever. He was diagnosed with nephrotic syndrome in 1998, which progressed to ESRD. He commenced MHD in 2012. Examination at our hospital revealed a soft nontender swelling of the left shoulder. Blood biochemistry showed elevated serum urate, phosphate, ß2 microglobulin, and parathyroid hormone. Imaging revealed joint effusion and dense heterogenous deposition. Aspirate analysis showed urate crystals 3+, and culture yielded no growth. Following rheumatology review, the working diagnosis was periarticular tissue tuberculosis, after excluding pseudogout and amyloidosis. Following 1 month of colchicine and allopurinol, synovial fluid microscopy showed CPDD crystals. Symptoms gradually resolved over the course of 6 months. In this rare case, a diagnosis of CPDD was made with a multidisciplinary approach that included imaging and biochemical investigations.

Musculoskeletal manifestations of primary hyperparathyroidism.

Primary hyperparathyroidism (PHPT) can be associated with a variety of musculoskeletal complaints, which occasionally can be the leading or presenting manifestation. In this paper, we describe the musculoskeletal manifestations observed in patients with primary hyperparathyroidism. Medical record reviews of a select population of 74 patients with primary hyperparathyroidism are seen in a rheumatology practice. Bone manifestations included back pain in 11 patients (15.2 %), generalized bone pain in 7 patients (9.7 %), rib cage/chest pain in 6 (8.3 %), pseudoclubbing in 3, and a giant cell tumor of the mandible in 2 (2.3 %) patients. Articular manifestations such as chondrocalcinosis with or without apatite deposition disease were seen in 13 (17.7 %), arthralgias in 11 (15.2 %), and non-specific synovitis in 7 (9.7 %). Muscle weakness was observed in six patients (8.3 %) and myalgias in three (4.6 %). Less common manifestations such as Achilles tendon rupture, Jaccoud-like arthropathy, sacral insufficiency fracture, arthritis associated with fever of unknown origin (FUO), meningitis, cervical cord compression, and persistent headache were observed in single patients. Musculoskeletal findings are still a frequent and important presentation in patients with primary hyperparathyroidism seen in rheumatology practice. Some of these manifestations can be quite unusual and may represent diagnostic dilemmas to the practicing rheumatologist and/or endocrinologist.

Calcium metabolism disorders simulating rheumatologic diseases.

Hypoparathyroidism and hyperparathyroidism may lead to spondylarthropathy or spondylarthropathy-like problems and crystal arthropathy, respectively. In this report, we present 2 cases with hypoparathyroidism and 1 case with hyperparathyroidism who developed spondylarthropathy-like disease, rheumatoid arthritis-like disease, and chondrocalcinosis, respectively. We briefly discussed relationship between calcium metabolism disorders and rheumatologic manifestations. As rheumatologists, we should be always open to other diagnosis if the treatment does not work in patients with rheumatologic diseases.

Characterisation and potential diagnostic value of circulating matrix Gla protein (MGP) species.

Matrix γ-carboxyglutamate (Gla) protein (MGP) is an important local inhibitor of vascular calcification, which can undergo two post-translational modifications: vitamin K-dependent γ-glutamate carboxylation and serine phosphorylation. While carboxylation is thought to have effects upon binding of calcium-ions, phosphorylation is supposed to affect the cellular release of MGP. Since both modifications can be exerted incompletely, various MGP species can be detected in the circulation. MGP levels were measured with two commercially available competitive and two novel sandwich assays in healthy controls, in patients with rheumatic disease, aortic valve disease, and end-stage renal disease, as well as in volunteers after vitamin K supplementation (VKS) and treatment with vitamin K antagonists (VKA). Major differences were found between the MGP assays, including significantly different behaviour with regard to vascular disease and the response to VKA and VKS. The dual-antibody assay measuring non-phosphorylated, non-carboxylated MGP (dp-ucMGP) was particularly sensitive for these changes and would be suited to assess the vascular vitamin K status. We conclude that the different assays for particular circulating MGP species allows the assessment of various aspects of the MGP system.

Serum nucleotide pyrophosphohydrolase activity; elevated levels in osteoarthritis, calcium pyrophosphate crystal deposition disease, scleroderma, and fibromyalgia.

OBJECTIVE: Quantification of serum nucleotide pyrophosphohydrolase (NTPPHase) activity in healthy subjects and in patients with various rheumatic diseases or with quad/hemiplegia, hemodialysis, or renal transplant. METHODS: Colorimetric assay of enzyme activity in serum. RESULTS: Serum NTPPHase activity in 85 healthy subjects was independent of age or sex and was highly reproducible in each individual. The biologic and methodologic coefficients of variation were nearly identical. Elevated enzyme levels were found in sera from patients with osteoarthritis/spondylosis, calcium pyrophosphate dihydrate (CPPD) crystal deposition, scleroderma, fibromyalgia, or hemodialysis. Renal transplant patients receiving cyclosporine had the highest enzyme activity of any group, whereas transplant patients not taking this drug had normal levels. Histograms of values in all groups showed a normal distribution. CONCLUSION: Serum NTPPHase activity levels were significantly elevated in patients with degenerative arthritis whether or not CPPD crystals were present, in patients with either scleroderma or fibromyalgia, and in patients receiving hemodialysis therapy or taking cyclosporine.

Prevalence of chondrocalcinosis in patients with primary hyperparathyroidism in Japan.

One hundred and thirty-two consecutive patients with primary hyperparathyroidism were studied preoperatively for the presence of chondrocalcinosis, the roentgenographic marker of calcium pyrophosphate dihydrate (CPPD) crystal deposition disease, by obtaining radiographs of knees, wrists and pelvis. Chondrocalcinosis was found in 8 patients (6.1%), each of whom was over 50 years of age. In 72 of the patients over 50 years of age, the prevalence of chondrocalcinosis in the hyperparathyroid patients (11.1%) was greater than that found in 72 control patients (2.8%) with thyroid nodular disease who were exactly matched for age and sex, but the difference was not significant. The prevalence of chondrocalcinosis in the hyperparathyroid patients sharply increased with age. In the group in their 50's it was 4.4%, rising to 15.8% in patients in their 60's and reaching 37.5% for those over 70 years of age. Patients with chondrocalcinosis were significantly older than those without this finding (p < 0.005). Those with chondrocalcinosis also had significantly higher preoperative serum calcium levels than those without it (p < 0.05). While chondrocalcinosis was detected by taking joint radiographs in all patients with primary hyperparathyroidism, acute arthritis (pseudogout attack) occurred in only 2 of the 132 patients (1.5%) after parathyroidectomy, but this represents 25% (2 of 8) of those who had chondrocalcinosis. An attack of pseudogout may therefore be one of the most common postoperative complications of parathyroid surgery in the elderly. Considering the low incidence of pseudogout attack following parathyroidectomy, preoperative radiological studies of the knee joints are sufficient to screen for chondrocalcinosis and are recommended for patients over 60 years old in Japan.(ABSTRACT TRUNCATED AT 250 WORDS)

Pseudogout attacks after successful treatment of hyperparathyroidism.

Two patients developed pseudogout attacks during treatment for hyperparathyroidism, the first shortly after surgery and the second during medical treatment of her hypercalcaemia. From our observations in these patients and from data in the literature, we conclude that pseudogout attacks following parathyroidectomy in patients with chondrocalcinosis are caused by the decrease in the serum calcium levels and not by the major surgical procedure itself.

Do viruses play an etiologic role in ankylosing spondylitis or psoriatic arthritis?

High venous blood levels of 2-5A, an adenylic acid polymer synthesized in the presence of double-stranded RNA and considered as a viral replication indicator, have been found in blood samples from ankylosing spondylitis and psoriatic arthritis patients, but not from patients with seropositive rheumatoid arthritis or acute chondrocalcinosis. These findings suggest the possibility that ankylosing spondylitis and psoriatic arthritis might be virus-induced diseases.

[Normocalcemic hyperparathormonemia and articular chondrocalcinosis. Study of 69 patients compared to 57 controls]. / Hyperparathormonémie normocalcémique et chrondrocalcinose articulaire. Etude de 69 patients comparés à 57 sujets témoins.

The level of parathyroid hormone was measured by heterologue C terminal radio-immunological assay in 69 patients with clinical or radiological manifestations of the type seen in primary articular chondrocalcinosis. They were divided into three groups: P1 with undetermined clinical arthropathies; P2 with sub-chondral and arthosic arthropathies; P3 with radiologically definite chondrocalcinosis. They were compared with 57 control subjects broken up into four groups: T1 with chronic rheumatic arthritis, T2 with low back pain, T3 with primary hyperparathyroidism due to adenoma, and T4 with secondary hyperparathyroidism with renal insufficiency. A form of normocalcemic hyperparathormonaemia was demonstrated in more than one out of two patients in group P1 (15/29). It was seen in three-quarters of the cases in group P2 (12/16). And it was seen in more than a quarter of the cases in group P3 (7/24). This hyperparathormonaemia was statistically significant only in groups P1 and P2 compared to the normals in groups T1 and T2. The results we obtained in this study seem to be in complete concordance with those we obtained earlier in idiopathic hemochromatosis. This hyperparathormonaemia seems to regress with age and is often only discovered when the characteristic articular lesions have appeared. The discovery of normocalcemic hyperparathormonaemia several years before the appearance of the radiological signs of the disease would appear to be an important argument in favour of the diagnosis of early articular chondrocalcinosis. The existence of raised parathyroid hormone in primary articular chondrocalcinosis as well as in idiopathic hemachromatosis is special etiopathogenic interest even if there remain numerous questions concerning its origin and mode of action.

[Associated and secondary chondrocalcinosis]. / Chondrocalcinoses secondaires et associées.

The authors consider the problems posed by secondary and associated chondrocalcinosis (CCA) on the basis of a study carried out at many French Rheumatology centres (Centres de Rhumatologie Française) and in the light of data in the literature. CCA is associated with gout in 4 percent of cases, with hyperparathyroidism in 3.9 percent of cases, with haemochromatosis in 1.7 percent of cases and with hypothyroidism in 0.8 percent of cases. The existence of secondary CCA in gout, hyperparathyroidism, haemochromatosis, Wilson's disease, ochronosis, hypophosphatasia and perhaps hypothyroidism seems proven. These secondary cases of CCA represent 10.7 percent of the total 1226 : here CCA seems to form one element of a much broader syndrome, namely the metabolic arthropathies. The other associations, with the exception of those which are not considered in this paper (rheumatoid polyarthritis, diabetes mellitus, Paget's disease, osteochrondromatosis, acromegalia), are probably coincidental.