Scandinavian Journal of Rheumatology
ISSN: 0300-9742 (Print) 1502-7732 (Online) Journal homepage: http://www.tandfonline.com/loi/irhe20
Pyrophosphate Arthropathy Anders Bjelle To cite this article: Anders Bjelle (1979) Pyrophosphate Arthropathy, Scandinavian Journal of Rheumatology, 8:3, 145-153, DOI: 10.3109/03009747909114447 To link to this article: http://dx.doi.org/10.3109/03009747909114447
Published online: 12 Jul 2009.
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Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=irhe20 Download by: [University of New England]
Date: 18 March 2016, At: 23:13
Scand J Rheumatology 8: 145-153, 1979
PYROPHOSPHATE ARTHROPATHY Anders Bjelle
Downloaded by [University of New England] at 23:13 18 March 2016
From the Department of Rlierimatology, University Hospital, UmeH, Sweden
ABSTRACT. Pyrophosphate arthropathy has a wide spectrum of clinical symptoms and is a common cause of synovitis in the elderly. The acute “pseudogout” attack is the most commonly recognized, but chronic synovitis might be just as prevalent. Familial pyrophosphate arthropathy with a dominant autosomal heredity is a rare form, with an earlier debut and a more severe prognosis. Another rare expression of pyrophosphate arthropathy is severe joint destruction. The cause of pyrophosphate arthropathy is still unknown. Both individual reactivity and crystal properties determine the intensity of the inflammatory response to crystals.
With the development of skeletal radiology during the first half of this century, occasional reports on the occurrence of joint calcifications appeared (cf. Bjelle 1971). In the late ’fifties two separate approaches stimulated further systematic studies. The aggregation of cases of joint calcifications and arthritis in a few Slovakian families thus alerted the local rheumatologists looking for X-ray changes in ochronosis (40, 41). Shortly afterwards, the use of polarized light microscopy of synovial fluids for the identification of urate crystals in gout was introduced (26), leading to the concept of pseudogout and subsequent identification of the crystals of calcium pyrophosphate dihydrate (27). During the last few decades, reports on pyrophosphate arthropathy-deposition of calcium pyrophosphate dihydrate crystals in joints-have appeared from most parts of the world (4, 1 1 , 28) including Scandinavia, where sporadic (cf. Bjelle and SundCn 1971) and familial (7) cases have been observed. The present report summarizes clinical and laboratory experience of pyrophosphate arthropathy with the aim of familiarizing clinical expressions and diagnostic procedures and to stimulate work on the elucidating of etiologic and pathogenetic factors. CLINICAL PICTURE For didactic reasons, the clinical expressions of pyrophosphate arthropathy are categorized as presented in Table I. 10- 791 866
The acute pseudogout attack may appear suddenly, with severe joint inflammation, pus-like synovial fluid, elevated body temperature, and a general state of malaise, but is often not so dramatic. Synovitis in one large joint is usual, but several and also smaller joints and bursae may be involved. The affection sometimes follows an operation or a severe cardiovascular illness, appearing on the second or third day. The synovitis subsides spontaneously within 1-3 weeks and the interval between attacks varies from weeks to years. In some patients, attacks appear with increasing frequency, ending in chronic synovitis with or without intervening acute exacerbations. Chronic pyrophosphate synovitis is usually associated with moderate joint swelling, stiffness and pain, frequently interpreted as osteoarthritis. This clinical picture is not as well documented as the acute attack (9). In Table 11, clinical findings are correlated to previous symptoms, illustrating the high prevalence of chronic joint complaints in pyrophosphate arthropathy. Farnilial pyrophosphate arthropathy has an autosomal, dominant inheritance (7, 34, 41). As in the sporadic cases, both acute and chronic cases as well as asymptomatic calcifications are found. Years of diffuse arthralgia may precede the first acute attack of synovitis. It often runs a more severe course with the development of disability, than the sporadic type of disease. Ankylosis of joints and spine has been observed. Some characteristics in sporadic versus non-familial disease within the same Scandinavian population are listed in Table 111. Destnrctive pyrophosphate arthropathy has so far only been found in a few elderly patients (30, 35). It develops rapidly and cannot be distinguished radiologically from neuropathic destruction of the Charcot type. No signs of nerve or vascular involvement have been demonstrated, however. Asymptomatic pyrophosphate arthropathy is a common d i c t i o n , judging by the high prevalence figures shown below. Calcifications are most Sctind J Rlrriitnotolog~8
146
A . Bjelle
Type of pyrophosphate arthropathy Acute
Chronic
Familial
Destructive
Asymptomatic
Occurence ,4ge
Common
Common
365
365
Rare Old
Common Old
Sex
d3P Good Gout, sepsis, trombosis
bcP Good Osteo-arthr., rheum. aithr.
Large joints
Large joints
Monoarticular
Mono- & oligoarticular Osteolytic lesions in wrists or large joints
Rare From 20, usually a40 d=P Poor Rheum. arthr., reactive arthr., osteo-arthr., ankyl.. spondyl. Large and small joints, spine Oligo- & polyarticular Ankylosis
Downloaded by [University of New England] at 23:13 18 March 2016
Prognosis Differential diagnosis Common joint involvement Number ofjoints involved Particular features
Post-op. or after acute illness
easily detectable in the knee menisci, but pyrophosphate deposition may be widely distributed in both hyaline and fibrous articular cartilages as well as in para-articular structures, without giving rise to symptoms such as illustrated by findings in family studies (7. 34. 41) and on radiographs of asymptomatic joints in cases of pyrophosphate synovitis ( I I ) .
d70 years) has revealed the surprising prevalence figures of 7 ( 13) and even 27 ( 16) R. Hereditary disease has so far only been found in a few countries (cf. Bjelle et al.. 1978). In Slovakia (41) and on the Chiloe Islands (34) it occurs among minorities who have immigrated and is thus possibly attributable to a founder effect. This genetic mechanism has not been proven in other countries
DIAGNOSIS .S~tioi,icil j l i i i d e.viitniimtiot1 The use of polarized light equipment in good routine microscopy facilitates the screening for
Previous Present Acute Subacute or chronic Total
Acute only
Chronic only
Acute + chronic
38
17
6
I2
16 I1
70 29
44
29
21
I00
Total (%)
Downloaded by [University of New England] at 23:13 18 March 2016
Pyrophosphci te rirtliropri thy
Fig. 1. Typical finding in wet preparation of synovial fluid. Note the great variation in crystal size and shape in a fluid rich in crystal material. The typical rhomboid shape of
crystal material considerably on wet preparations of synovial fluids (Fig. 1). The use of a quartz compensator plate and a rotating specimen stage is a further advantage as pyrophosphate crystals are weakly birefringent. It allows the differentiation of positively birefringent pyrophosphate crystals from other material such as negatively birefringent urate crystals. Other birefringent materials in the specimens are common (cholesterol, collagen, organic debris, steroid crystals). The use of non-birefringent (dust free) equipment is essential. X-ray diffraction techniques (3, 27) and electron microscopic and X-ray spectrometry procedures (1,8, 15) for precise crystal identification will remain research tools of importance for the future recogni-
pyrophosphate is seen in the lower right-hand corner. Small, irregular intracellular crystals are often the only finding.
tion of “ultra-micro crystal arthritides” (8) and for the more precise study of crystal structures. The cell count of synovial fluid can reach very high levels, the synovial fluid resembling thick whitish-yellow pus with innumerable leukocytes as in septic arthritis. In chronic cases the cell count is usually higher than in rheumatoid arthritis, though it may be low in some patients (
ISSN: 0300-9742 (Print) 1502-7732 (Online) Journal homepage: http://www.tandfonline.com/loi/irhe20
Pyrophosphate Arthropathy Anders Bjelle To cite this article: Anders Bjelle (1979) Pyrophosphate Arthropathy, Scandinavian Journal of Rheumatology, 8:3, 145-153, DOI: 10.3109/03009747909114447 To link to this article: http://dx.doi.org/10.3109/03009747909114447
Published online: 12 Jul 2009.
Submit your article to this journal
Article views: 4
View related articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=irhe20 Download by: [University of New England]
Date: 18 March 2016, At: 23:13
Scand J Rheumatology 8: 145-153, 1979
PYROPHOSPHATE ARTHROPATHY Anders Bjelle
Downloaded by [University of New England] at 23:13 18 March 2016
From the Department of Rlierimatology, University Hospital, UmeH, Sweden
ABSTRACT. Pyrophosphate arthropathy has a wide spectrum of clinical symptoms and is a common cause of synovitis in the elderly. The acute “pseudogout” attack is the most commonly recognized, but chronic synovitis might be just as prevalent. Familial pyrophosphate arthropathy with a dominant autosomal heredity is a rare form, with an earlier debut and a more severe prognosis. Another rare expression of pyrophosphate arthropathy is severe joint destruction. The cause of pyrophosphate arthropathy is still unknown. Both individual reactivity and crystal properties determine the intensity of the inflammatory response to crystals.
With the development of skeletal radiology during the first half of this century, occasional reports on the occurrence of joint calcifications appeared (cf. Bjelle 1971). In the late ’fifties two separate approaches stimulated further systematic studies. The aggregation of cases of joint calcifications and arthritis in a few Slovakian families thus alerted the local rheumatologists looking for X-ray changes in ochronosis (40, 41). Shortly afterwards, the use of polarized light microscopy of synovial fluids for the identification of urate crystals in gout was introduced (26), leading to the concept of pseudogout and subsequent identification of the crystals of calcium pyrophosphate dihydrate (27). During the last few decades, reports on pyrophosphate arthropathy-deposition of calcium pyrophosphate dihydrate crystals in joints-have appeared from most parts of the world (4, 1 1 , 28) including Scandinavia, where sporadic (cf. Bjelle and SundCn 1971) and familial (7) cases have been observed. The present report summarizes clinical and laboratory experience of pyrophosphate arthropathy with the aim of familiarizing clinical expressions and diagnostic procedures and to stimulate work on the elucidating of etiologic and pathogenetic factors. CLINICAL PICTURE For didactic reasons, the clinical expressions of pyrophosphate arthropathy are categorized as presented in Table I. 10- 791 866
The acute pseudogout attack may appear suddenly, with severe joint inflammation, pus-like synovial fluid, elevated body temperature, and a general state of malaise, but is often not so dramatic. Synovitis in one large joint is usual, but several and also smaller joints and bursae may be involved. The affection sometimes follows an operation or a severe cardiovascular illness, appearing on the second or third day. The synovitis subsides spontaneously within 1-3 weeks and the interval between attacks varies from weeks to years. In some patients, attacks appear with increasing frequency, ending in chronic synovitis with or without intervening acute exacerbations. Chronic pyrophosphate synovitis is usually associated with moderate joint swelling, stiffness and pain, frequently interpreted as osteoarthritis. This clinical picture is not as well documented as the acute attack (9). In Table 11, clinical findings are correlated to previous symptoms, illustrating the high prevalence of chronic joint complaints in pyrophosphate arthropathy. Farnilial pyrophosphate arthropathy has an autosomal, dominant inheritance (7, 34, 41). As in the sporadic cases, both acute and chronic cases as well as asymptomatic calcifications are found. Years of diffuse arthralgia may precede the first acute attack of synovitis. It often runs a more severe course with the development of disability, than the sporadic type of disease. Ankylosis of joints and spine has been observed. Some characteristics in sporadic versus non-familial disease within the same Scandinavian population are listed in Table 111. Destnrctive pyrophosphate arthropathy has so far only been found in a few elderly patients (30, 35). It develops rapidly and cannot be distinguished radiologically from neuropathic destruction of the Charcot type. No signs of nerve or vascular involvement have been demonstrated, however. Asymptomatic pyrophosphate arthropathy is a common d i c t i o n , judging by the high prevalence figures shown below. Calcifications are most Sctind J Rlrriitnotolog~8
146
A . Bjelle
Type of pyrophosphate arthropathy Acute
Chronic
Familial
Destructive
Asymptomatic
Occurence ,4ge
Common
Common
365
365
Rare Old
Common Old
Sex
d3P Good Gout, sepsis, trombosis
bcP Good Osteo-arthr., rheum. aithr.
Large joints
Large joints
Monoarticular
Mono- & oligoarticular Osteolytic lesions in wrists or large joints
Rare From 20, usually a40 d=P Poor Rheum. arthr., reactive arthr., osteo-arthr., ankyl.. spondyl. Large and small joints, spine Oligo- & polyarticular Ankylosis
Downloaded by [University of New England] at 23:13 18 March 2016
Prognosis Differential diagnosis Common joint involvement Number ofjoints involved Particular features
Post-op. or after acute illness
easily detectable in the knee menisci, but pyrophosphate deposition may be widely distributed in both hyaline and fibrous articular cartilages as well as in para-articular structures, without giving rise to symptoms such as illustrated by findings in family studies (7. 34. 41) and on radiographs of asymptomatic joints in cases of pyrophosphate synovitis ( I I ) .
d70 years) has revealed the surprising prevalence figures of 7 ( 13) and even 27 ( 16) R. Hereditary disease has so far only been found in a few countries (cf. Bjelle et al.. 1978). In Slovakia (41) and on the Chiloe Islands (34) it occurs among minorities who have immigrated and is thus possibly attributable to a founder effect. This genetic mechanism has not been proven in other countries
DIAGNOSIS .S~tioi,icil j l i i i d e.viitniimtiot1 The use of polarized light equipment in good routine microscopy facilitates the screening for
Previous Present Acute Subacute or chronic Total
Acute only
Chronic only
Acute + chronic
38
17
6
I2
16 I1
70 29
44
29
21
I00
Total (%)
Downloaded by [University of New England] at 23:13 18 March 2016
Pyrophosphci te rirtliropri thy
Fig. 1. Typical finding in wet preparation of synovial fluid. Note the great variation in crystal size and shape in a fluid rich in crystal material. The typical rhomboid shape of
crystal material considerably on wet preparations of synovial fluids (Fig. 1). The use of a quartz compensator plate and a rotating specimen stage is a further advantage as pyrophosphate crystals are weakly birefringent. It allows the differentiation of positively birefringent pyrophosphate crystals from other material such as negatively birefringent urate crystals. Other birefringent materials in the specimens are common (cholesterol, collagen, organic debris, steroid crystals). The use of non-birefringent (dust free) equipment is essential. X-ray diffraction techniques (3, 27) and electron microscopic and X-ray spectrometry procedures (1,8, 15) for precise crystal identification will remain research tools of importance for the future recogni-
pyrophosphate is seen in the lower right-hand corner. Small, irregular intracellular crystals are often the only finding.
tion of “ultra-micro crystal arthritides” (8) and for the more precise study of crystal structures. The cell count of synovial fluid can reach very high levels, the synovial fluid resembling thick whitish-yellow pus with innumerable leukocytes as in septic arthritis. In chronic cases the cell count is usually higher than in rheumatoid arthritis, though it may be low in some patients (
