The arthropathy of cystic fibrosis.

Dixey, Josh; Redington, A. N.; Butler, Robin; Smith, Michael J.; Batchelor, J. R.; Woodrow, D F; Hodson, M E; Batten, John; Brewerton, D. A.

doi:10.1136/ard.47.3.218

Cited by 61 publications

(31 citation statements)

References 22 publications

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“…By this means we have found a total of 90 reported cases of HPOA in CF1" [5][6][7]. In these reports the diagnosis of HPOA was made by three different sets of criteria:…”

Section: Clinical Featuresmentioning

confidence: 99%

Joint Disorders in Cystic Fibrosis

Turner¹,

Baildam²,

Patel³

et al. 1997

J R Soc Med

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“…By this means we have found a total of 90 reported cases of HPOA in CF1" [5][6][7]. In these reports the diagnosis of HPOA was made by three different sets of criteria:…”

Section: Clinical Featuresmentioning

confidence: 99%

Joint Disorders in Cystic Fibrosis

Turner¹,

Baildam²,

Patel³

et al. 1997

J R Soc Med

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“…Nonetheless, there are some differences. HOA is relatively common in carcinoma of the bronchus (about 10% of cases [IOS]), and in some other lung conditions, such as cystic fibrosis [31,32]; yet it is manifestly much less common and much less florid in cyanotic congenital heart disease. One difference could be in megakaryocyte function.…”

Section: Hypertrophic Osteoarthropathy (Hoa)mentioning

confidence: 99%

The aetiology of clubbing and hypertrophic osteoarthropathy

Dickinson

1993

Eur J Clin Investigation

132

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Abstract. The evidence is reviewed for the hypothesis that clubbing and hypertrophic osteoarthropathy are due to the peripheral impaction of megakaryocytes and platelet clumps in the fingers and toes, to which this particulate matter has passed in an axial stream. The normal pulmonary vascular bed retains these large particles, which fragment before entering the systemic circulation. A right-to-left shunt allows them to bypass the pulmonary vascular bed. A preliminary histological report of platelet clumps seen at necropsy in nail bed capillaries of clubbed fingers supports the hypothesis. Platelets contain and release platelet-derived growth factor, whose known effects could explain all the pathological changes in clubbing. In addition to explaining why clubbing should occur in cyanotic congenital heart disease, clubbing in sub-acute bacterial endocarditis and distal to infected arterial grafts and aneurysms can be understood in terms of platelet clumps breaking off valves or arterial walls, and passing distally. Clubbing in liver disease is associated with multiple small pulmonary arteriovenous anastomoses which allow large particles through. Hypertrophic osteoarthropathy probably shares the same mechanism, and is mainly attributable to PDGF release; but there may also be altered platelet function and an additional growth factor derived from the lungs.

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“…Symptoms related to the joints occur in up to 12% of CF patients [53,54] and more commonly affect adults in the second decade of life. It was thought that there was no relationship between the severity of pulmonary disease and the occurrence of arthritis [53] until a recent study suggested an association with infection with P. aeruginosa and A. fumigatus [54].…”

Section: Arthropathiesmentioning

confidence: 99%

“…It was thought that there was no relationship between the severity of pulmonary disease and the occurrence of arthritis [53] until a recent study suggested an association with infection with P. aeruginosa and A. fumigatus [54]. The most common arthropathies are episodic arthritis (EA) and hypertrophic pulmonary arthropathy (HPOA).…”

Section: Arthropathiesmentioning

confidence: 99%

Adult cystic fibrosis care in the 21st century

Garattini

Bilton

Cremona

et al. 2015

Monaldi Arch Chest Dis

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Cystic fibrosis (CF) is the most common autosomal recessive inherited disease of Caucasian populations. As a result of a variety of diagnostic and therapeutic strategies there has been a dramatic increase in the life expectancy of patients with CF in the last decades and 50% of patients are now adults. This review will focus on the disease in adults and the provision of appropriate care. The complex care required to improve the survival and quality of life in the adult patients can best be provided in a dedicated adult cystic fibrosis unit. These units currently exist in many European countries, but more are needed in Italy.

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The arthropathy of cystic fibrosis.

Cited by 61 publications

References 22 publications

Joint Disorders in Cystic Fibrosis

Joint Disorders in Cystic Fibrosis

The aetiology of clubbing and hypertrophic osteoarthropathy

Adult cystic fibrosis care in the 21st century

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