| A 61-year-old man had a tophus on the third finger of his right hand. There was no history of arthritis, no laboratory abnormality, and no chondrocalcinosis. Crystalline material from the tophus was identified as calcium pyrophosphate dihydrate by x-ray diffraction. ( info) |
112/186. Pyrophosphate arthropathy in hypophosphatasia. The association of hypophosphatasia and pyrophosphate arthropathy in an adult patient has been described on 1 previous occasion. We report a further 2 patients with this disease combination. One patient suffers from the type of hypophosphatasia that presents in adult life, with fractures that are either spontaneous or the result of minimal trauma. The other patient suffered from the severe type of hypophosphatasia that presents in infancy but survived longer than is usual; the necropsy findings on this patient are reported. ( info) |
113/186. Idiopathic familial chondrocalcinosis due to apatite crystal deposition. Idiopathic familial chondrocalcinosis was found in five members of a family. The clinical features of the disease were morning stiffness, pain and limitation of motion of the dorsolumbar spine in four of the five members, associated with arthritis of the small joints of the hands in three, shoulder periarthritis in two and costal cartilage pain in one. Radiologically, four of the five patients had multiple intervertebral disk calcifications, mainly located at the nucleus pulposus area. Three of them also had periarticular calcific deposits, associated with costal cartilage calcifications and degenerative changes in the small joints of the hands in two. None of these cases showed cartilage calcification in the knees, public symphysis or triangular ligament of carpus. In the propositus, optical microscopy of a specimen of the second proximal interphalangeal joint obtained by open biopsy showed the presence of multiple calcified areas in the intercellular matrix and chondroid metaplasia with calcification of the matrix in the synovial membrane and capsule. x-ray diffraction studies, energy dispersive analysis, and infrared spectrophotometry of a costal cartilage sample obtained by open biopsy demonstrated the presence of carbonate calcium hydroxyapatite. In this study, no relation was found between hla antigens, loci A, B and C, and this syndrome due to calcium hydroxyapatite crystal deposition. ( info) |
114/186. Further observations on the arthropathy of calcium pyrophosphate crystal deposition disease. The arthropathy of calcium pyrophosphate dihydrate (CPPD) crystal deposition disease is distinctive and may affect lumbar spinal and sacroiliac joints, as well as appendicular joints. Subchondral pseudocysts that are a hallmark of the disease have a variable appearance, but often occur as a typical cluster of subchondral, coalescent lucencies with smudged, sclerotic margins. Structural joint collapse with fragmentation of cartilage and bone may occur and appear to be related, at least in some cases, to antecedent pseudocysts. Characteristic intra-articular osteochondral bodies are often extensive and may affect multiple joints; their pathogenesis is discussed. Articular synovial calcification is common and may be due to calcium hydroxyapatite, as well as CPPD, particularly if advanced degenerative changes are present. Recognition of the radiologic features may be encountered in CPPD crystal deposition disease is important for differential diagnosis. ( info) |
115/186. calcium pyrophosphate dihydrate deposition disease and familial hypomagnesemia. A 40-year-old woman presented with calcium pyrophosphate synovitis and chondrocalcinosis. She was subsequently found to have hypomagnesemia, as did her 22-year-old son. Metabolic studies demonstrated normal gastrointestinal absorption of magnesium, and impaired renal conservation of magnesium without other evidence of renal tubular dysfunction. It seems likely that a genetically determined abnormality of magnesium metabolism was responsible for the occurrence of chondrocalcinosis in this patient. ( info) |
| calcium pyrophosphate dihydrate deposits were found in the lumbar disc fibrocartilage in 4 patients undergoing surgery for spinal cord or nerve root compression. All of the patients had prior surgery at the same lumbar area. None of the patients had the articular or roentgenographic manifestations of calcium pyrophosphate deposition disease (pseudogout). Andres and Trainer have recently reported 7 similar patients. calcium pyrophosphate dihydrate deposition in axial skeleton fibrocartilage may be a common finding in patients undergoing repeat spinal surgery. ( info) |
117/186. Pyrophosphate arthropathy in two Swedish families. A clinical and radiographic survey of 110 members of 2 families with hereditary pyrophosphate arthropathy was performed. The mode of inheritance was autosomal, dominant with a variable penetrance. Twenty-two percent of the family members had joint involvement related to pyrophosphate arthropathy, 47% of those over 50 years of age had experienced acute attacks of arthritis and/or had joint calcifications. The majority of individuals with both arthritis and joint calcifications suffered from chronic pain that resulted in early retirement. A high frequency of back pain was observed, but no ankylosis or deformity. Surgery was performed for parathyroid hyperplasia on the propositus in 1 family, and several members of her family suffered from symptoms that suggested a disturbance of calcium phosphate metabolism. There were several differences between our patients and 50 cases of sporadic pyrophosphate arthropathy from the same area of sweden. Familial cases had an earlier onset, a greater number of involved joints, and peripheral joint involvement more often. back pain was more frequent, and calcifications of intervertebral discs were found only in the hereditary cases. ( info) |
118/186. Massive articular chondrocalcinosis. Its occurrence with calcium pyrophosphate crystal deposits in nucleus pulposus. We studied a case of long-standing chondrocalcinosis that after 40 years affected almost all fibrocartilages, hyaline cartilages, and articular cavities within the subject. Massive deposits of dihydrocalcium pyrophosphate (CPP) were also found in nonfissured nucleus pulposus of the vertebral disks. To our knowledge, this is the first report of CPP deposits in these disks. ( info) |
| A case of olecranon bursitis in an 81-year-old patient is presented. Analysis of the bursal fluid revealed positive birefringent crystals; radiographs showed calcifications in the distal triceps tendon. A bursectomy was performed. x-ray diffraction analyses demonstrated calcium pyrophosphate dihydrate patterns in a subcutaneous "tophus" and in a specimen of the tendon. On histologic examination, there was a bursitis with positive birefringent crystals on the bursa's inner surface; histologic images of "chondrocalcinosis" were observed in and around the tendon. It is concluded that bursitis may be part of the extraarticular manifestations of calcium pyrophosphate dihydrate crystal deposition disease. ( info) |
120/186. Pyrophosphate arthropathy as a late complication of juvenile chronic arthritis. Pyrophosphate arthropathy is an age-related disease rarely seen in those under 45 years of age. We report 2 patients with early onset pyrophosphate arthropathy in joints previously affected by juvenile chronic arthritis. This previously undescribed association supports the hypothesis that joint damage may predispose to calcium pyrophosphate dihydrate crystal deposition and lead to secondary pyrophosphate arthropathy. ( info) |