Malignancy in scleroderma patients from south west England: a population-based cohort study.
Affiliation
Department of Medicine, Great Western Hospital, Swindon, SN3 6BB, UK, keith@siau.org.Issue Date
2010-01-08
Metadata
Show full item recordCitation
Malignancy in scleroderma patients from south west England: a population-based cohort study. 2010: Rheumatol. Int.Journal
Rheumatology internationalDOI
10.1007/s00296-009-1348-yPubMed ID
20058012Abstract
The pathophysiological relationship between scleroderma and malignancy remains poorly understood. Although some previous studies have demonstrated an increased malignancy risk in patients with scleroderma, others have been inconclusive. We aimed to determine if patients with scleroderma had an increased risk of malignancy compared to an age- and sex-matched local South West England population, and if there were any important differences between scleroderma patients with and without malignancy. Methods of this study are as follows. Notes were obtained on all local scleroderma patients (n = 68) locally, and those diagnosed with malignancy verified by contacting each patient's general practitioner. Expected malignancy figures were obtained from age- and sex-stratified regional prevalence data provided by the South West Cancer Intelligence Service registry. Among the patients, 22.1% with scleroderma were identified with concurrent malignancy. Affected sites were of the breast (n = 5), haematological system (n = 5), skin (n = 4), and unknown primary (n = 1). Overall, malignancy risk was found to be increased in scleroderma (RR = 3.15, 95% CI 1.77-5.20, p = 0.01). In particular, this risk was the highest for haematological malignancies (RR = 18.5, 95% CI 6-43, p = 0.03), especially for non-Hodgkin's lymphoma (RR = 25.8, 95% CI 5-75, p = 0.10). The majority of patients (86.7%) developed malignancy after the onset of scleroderma (mean = 6.9 years). Age of >70 and patients with limited scleroderma were significant risk factors for a patient with scleroderma to have a concurrent malignancy; however, no increased risk was found in patients with any particular pattern of organ involvement, cytotoxic usage or serology. To conclude, in this small patient cohort, we have found that scleroderma is associated with an increased risk of malignancy. This risk is statistically significant in patients with limited scleroderma. Patients who are elderly and those with limited disease should be closely scrutinized at follow-up appointments.ISSN
1437-160Xae974a485f413a2113503eed53cd6c53
10.1007/s00296-009-1348-y
Scopus Count
Collections
Related articles
- Scleroderma in South Australia: further epidemiological observations supporting a stochastic explanation.
- Authors: Roberts-Thomson PJ, Walker JG, Lu TY, Esterman A, Hakendorf P, Smith MD, Ahern MJ
- Issue date: 2006 Aug
- Systemic sclerosis sine scleroderma: a multicenter study of 1417 subjects.
- Authors: Diab S, Dostrovsky N, Hudson M, Tatibouet S, Fritzler MJ, Baron M, Khalidi N, Canadian Scleroderma Research Group
- Issue date: 2014 Nov
- Italian cancer figures--Report 2015: The burden of rare cancers in Italy.
- Authors: AIRTUM Working Group, Busco S, Buzzoni C, Mallone S, Trama A, Castaing M, Bella F, Amodio R, Bizzoco S, Cassetti T, Cirilli C, Cusimano R, De Angelis R, Fusco M, Gatta G, Gennaro V, Giacomin A, Giorgi Rossi P, Mangone L, Mannino S, Rossi S, Pierannunzio D, Tavilla A, Tognazzo S, Tumino R, Vicentini M, Vitale MF, Crocetti E, Dal Maso L
- Issue date: 2016 Jan-Feb
- Risk of cancer in patients with scleroderma: a population based cohort study.
- Authors: Hill CL, Nguyen AM, Roder D, Roberts-Thomson P
- Issue date: 2003 Aug
- Increased malignancies in our Waikato cohort of patients with systemic sclerosis.
- Authors: Rees MS, Frampton C, White DHN, Solanki KK
- Issue date: 2021 Apr