Crystal Deposition Disease

Rheumatic disease is defined as disease of connective tissue and medical disorders of the musculoskeletal system . The medical discipline concerned with these diseases is referred to as rheumatology. The majority of rheumatic diseases are soft tissue rheumatism and nonspecific low back pain (LBP), autoimmune inflammatory rheumatic diseases, osteoarthritis (OA), osteoporosis, crystal-deposition disease and infectious arthritis. 

The etiologies of the autoimmune inflammatory diseases, OA, osteoporosis and crystal-deposition disease are still not known in exact details. This is in contrast with impressive molecular insights gained recently. However, there is consensus that manifestations of autoimmune diseases are precipitated by either acute and/or chronic interactions of genetic and environmental risk factors. 

Gouty arthritis is an inflammatory response to the deposition of monosodium urate monohydrate crystals secondary to hyperuricemia. It is called monosodium urate crystal deposition disease. Hyperuricemia is a serum urate concentration > 7 mg% in males and >6 mg% in females. Hyperuricemia results from overproduction (10-15% of individuals) or a renal excretion of urate lower than 400 mg uric acid/24 hours (85-90% of individuals). The urate under-excretors have a urate clearance of <6 ml/min or a urate to creatinine clearance ratio of <6%. The combination of a relative excess of dietary purine consumption together with urate under-excretion is often the basis for hyperuricemia. 

Pharmaceutical therapy of acute arthritis of crystal-deposition disease is effective, in particular for gout and hyperuricemia. Treatment is directed towards termination of acute arthritis, prevention of recurring attacks and prophylaxis and reversal of complications of chronic gout. Such complications include tophi, urolithiasis, nephropathy and with hyperuricemia associated medical problems that can be prevented, inhibited, and sometimes reversed. 

Steinbach LC (2004) Calcium pyrophosphate dehydrate and calcium hydroxyapatite crystal deposition diseases imaging perspectives. Radiol Clin North Am 42 185-205 Steiner GM, Sprigg A (1992) The value of ultrasound in the assessment of bone. Br J Radiol 65 589-593 Stieber JR, Dormans JP (2005) Manifestations of hereditary multiple exostoses. J Am Acad Orthop Surg 13 110-120 Stone M, Bergin D, Whelan B et al (2001) Power Doppler ultrasound assessment of rheumatoid hand synovitis. J Rheumatol 28 1979-1982... 

Among the degenerative arthropathies that typically involve the shoulder, there are a variety of conditions related to crystal deposition diseases, including renal osteodystrophy, milk alkali syndrome, hyper-vitaminosis D and the so-called Milwaukee shoulder syndrome . This last condition, which is also known as apatite-associated destructive arthritis, hemorrhagic shoulder or rapid destructive arthritis of the shoulder, consists of massive rotator cuff tear, osteoarthritic changes, hlood-stained noninflammatory joint effusion containing calcium hydroxyapatite and calcium pyrophosphate dihydrate crystals, synovial hyperplasia and extensive destruction... 

Fig. 6.141a,b. Chondrocalcinosis. a Oblique coronal 12-5 MHz US image over the supraspinatus tendon with b radiographic correlation demonstrates a continuum of fine hyperechoic spots (arrows) located in series within the hypoechoic articular cartilage of the humeral head (HH), reflecting calcium pyrophosphate dihydrate crystal deposition disease... 

US demonstrates olecranon bursitis as a localized fluid collection and/or synovial wall hypertrophy within the subcutaneous tissue immediately posterior to the olecranon (Fig. 8.50). Soft-tissue hyperemia is often recognized with color and power Doppler imaging as an accompanying finding (Lin et al. 2000). The hypervascular pattern typically distributes in a rim-like peribursal pattern. In crystal deposition diseases, the US appearance of bursal fluid is more likely hyperechoic and associated with thickened and echogenic bursal walls (Fig. 8.51). In hemorrhagic and septic bursitis, the fluid may result in a complex... 

Ohira, T. Ishikawa, K. Masuda, I. Yokoyama, M. Honda, I. Histologic localization of lipid in the articular tissues in calcium pyrophosphate dihydrate crystal deposition disease. Arthritis Rheum. 1988, 31, 1057-1062. 

Gout is a metabolic disease characterized by recurrent episodes of acute arthritis due to deposits of monosodium urate in joints and cartilage. Uric acid renal calculi, tophi, and interstitial nephritis may also occur. Gout is usually associated with hyperuricemia, high serum levels of uric acid, a poorly soluble substance that is the major end product of purine metabolism. In most mammals, uricase converts uric acid to the more soluble allantoin this enzyme is absent in humans. While clinical gouty episodes are associated with hyperuricemia, most individuals with hyperuricemia may never develop a clinical event from urate crystal deposition. 

Because of nephrotoxicity (oliguria, cylindruria, and reduced creatinine clearance, with crystal deposits in renal tubules and interstitial tissue) (4) phenazopyridine should not be used in patients with suspected renal disease and insufficiency, or in patients with glucose-6-phos-phate dehydrogenase deficiency. Bladder stones have also been described (5). 

Farge D, Turner MW, and Roy DR (1986) Dyazide-in-duced reversible acute renal failure associated with intracellular crystal deposition. American Journal of Kidney Diseases 8 445—449. 

I would like to suggest that involvement of this joint results from unusual degrees of physical stress, that this stress leads to degenerative joint disease, that further use of the damaged joint leads to synovial effusion, and that the nocturnal resolution of these effusions transiently concentrates uric acid. When this sequence of events occurs in a hyperuricemic patient, the increment in urate concentration may surpass its solubility limits and lead to crystal deposition. 

Fig. 5.34a-g. Calcium pyrophosphate deposition disease, a-c Transverse 12-5 MHz US images obtained over a the femoral trochlea, b the posterior aspect of the medial condyle and c the lateral meniscus in a patient with bilateral degenerative osteoarthritis of the knee reveal scattered hyperechoic foci (arrowheads) due to crystal deposition within the hyaline cartilage, the medial meniscus and the joint capsule (arrows). F, femur T, tibia. Note that crystals tend to be deposited in the middle layer of the cartilage, parallel to the subchondral bone, d-f Radiographic correlation, g Schematic drawing illustrates the typical deposition pattern of pyrophosphate crystals within the cartilage... 

Calcification of collateral ligaments of the fingers can follow traumatic injuries or result from crystal pyrophosphate deposition disease. The diagnosis of ligament calcifications relies on standard radiographs and US is not considered the best modality to detect them. However, ligament calcifications can... 

Fig. 11.66a,b. Lateral ligament calcification in a patient with crystal pyrophosphate deposition disease, a Coronal 12-5 MHz US image over the proximal interphalangeal joint with b radio-graphic correlation demonstrates a calcified convex structure (arrows) with posterior acoustic shadowing over the joint line, reflecting a ligament calcification. PP, proximal phalanx MP, middle phalanx... 

Diseases. Liquid crystals have been impHcated in a number of disease conditions in the human body. A complex cholesterol—phosphoHpid—Hpoprotein Hquid crystal phase has been identified in the initiation and maintenance of atheromatous deposits on the aortic intima in dissected human and rabbit arteries (40). The paracrystalHne nature of this precursor to plaque buildup with the resultant loss of arterial elasticity... 

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