Arthritis & Rheumatism, Volume 63,
November 2011 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Chicago, Illinois November 4-9, 2011.

Cancer in Systemic Sclerosis: Results From a Single Centre Cohort Report.

Abignano,  Giuseppina, Evans,  Hannah Lee, Emery,  Paul, Del Galdo,  Francesco, Buch,  Maya H.


Malignancy has been reported in 3.6–10.7 % of patients with systemic sclerosis (SSc) (1) with lung and breast being the most commonly reported types. The aim of this study was to determine the incidence of cancer in our SSc cohort, describe their demographic and clinical features and any cancer risk factors.


The medical records of 191 patients admitted to our centre and diagnosed with SSc between 1985 and 2010, all fulfilling the ACR classification criteria, were retrospectively reviewed. Patients with overlap syndrome and mixed connective tissue disease were excluded from the analysis. The epidemiological and clinical information and any risk factors of the patients with cancer history were compared to those of a gender-matched control group, randomly selected from our scleroderma database. Continuous variables were expressed as mean and standard deviation. Unpaired two-tailed t-test was used to compare groups. Qualitative variables were compared using Fisher's exact test. Data were analysed using GraphPad Prism software.


Of 191 SSc patients, 18 (9.4%) were found to have a history of cancer. Twenty-one primary cancers were identified. Breast cancer was the most frequent (7 cases, 3.6 %), followed by non-melanoma skin cancer (4 cases, 2.1 %; 3 basal cell carcinoma, 1 squamous cell carcinoma), lung cancer (3 cases, 1.6%), non-Hodgkin's lymphoma (3 cases, 1.6 %), melanoma (1 case, 0.5 %), myeloma (1 case, 0.5.%), colon (1 case, 0.5 %) and parotid cancer (1 case, 0.5%). In 2 patients cancer preceded the SSc onset (1 breast cancer, 1 non-Hodgkin's lymphoma). One patient had 2 primary cancers, breast and colon, before and after the SSc onset, respectively. Comparing the 2 groups with gender-matched controls, we found that cancer diagnosis occurred at a younger age in patients that developed SSc after the malignancy (p = 0.02). Patients with cancer developed SSc at an older age compared to controls (p = 0.0123 ). No difference was found in any risk factors. The risk associated to the cyclophosphamide (CYC) therapy in SSc patients with cancer was nearly significant (p = 0.075) (table).

ParametersPost-SSc cancer (n=15)Pre-SSc cancer (n=2)Post-SSc cancer vs pre-SSc cancer (p value)Pre- and post SSc cancer (n=1)SSc no cancer (n=18)All SSc-cancer (n=18) vs control (p-value)
Gender (M/F)2/130/210/12/16
Subset, L/D12/32/011/013/50.69
ANA +14 (93.3%)2 (100%)11 (100%)17 (94.4%)1.5
ACA +7 (46.6%)0 (0%)0.481 (100%)5 (27.7%)0.49
AntiScl-70 +3 (20%)1 (50%)0.4306 (33.3%)0.71
Age at SSc onset (mean ± sd)52.9 ± 1747.5 ± 3.50.676537.9 ± 18.20.0123
Age at cancer dx (mean ± sd)63.4 ± 11.342 ± 4.20.0241, 78
Family malignancy history1 (6.6%)1 (50%)0.2303 (16.7%)1
ILD7 (46.6%)0 (0%)0.4905 (27.7%)0.72
tobacco5 (33.3%)1 (50%)104 (22.2%)0.71
alcohol0 (0%)0 (0%)01 (5.55%)1
CYC8 (53.3%)1 (50%)103 (16.6%)0.075
MMF2 (13.3%)1 (50%)0.3302 (11.1%)1
AZA0 (0%)0 (0%)00 (0%)
MTX0 (0%)1 (50%)0.1202 (11.1%)1


In line with previous reports, we found breast and lung cancer among the most common types of malignancy in our SSc patients. High incidence of cases of non-Hodgkin's lymphoma cases was also observed. Although not mostly associated in SSc patients, a relatively high incidence of non-melanoma skin cancers was noted in in our cohort; the small studied population may underlie these findings. With the limitations of a case-control study, no risk factors were identified, except for the nearly significant risk associated to the CYC therapy.


1.Wooten, M. Southern Medical journal 2008

To cite this abstract, please use the following information:
Abignano, Giuseppina, Evans, Hannah Lee, Emery, Paul, Del Galdo, Francesco, Buch, Maya H.; Cancer in Systemic Sclerosis: Results From a Single Centre Cohort Report. [abstract]. Arthritis Rheum 2011;63 Suppl 10 :667

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