Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.

An Analysis of Agreement of Guidelines for Management in SSc from a Large Database (CSRG: Canadian Scleroderma Research Group).

Pope3,  Janet E., Harding4,  Sarah, Khimdas4,  Sarit, Bonner2,  Ash, Baron1,  Murray

Jewish General Hospital, Montreal, QC, Canada
McMaster University
St Joseph Health Care London, London, ON, Canada
University of Western Ontario


We determined congruence with recently published guidelines from EULAR/EUSTAR, for SSc investigations and treatment practices within the Canadian Scleroderma Research Group (CSRG)


Investigations and medication use for SSc complications were obtained from SSc patients in the CSRG to determine adherence to guidelines.


The CSRG database of 938 SSc patients demonstrated that annual echocardiograms for PAH screening were done in 86.5%; for PAH treatment one quarter were receiving warfarin which has no recommendations, all diagnosed with PAH Class III/IV were on PAH specific treatment (9% of total CSRG patients had PAH). For Raynaud's: 47% were on calcium channel blockers and some sites used PDE5 inhibitors more than others. Iloprost is not approved in Canada, so its use for severe RP and digital ulcers was low. In the guidelines it is recommended that all SSc patients with GERD be recommended for PPIs. In the CSRG database, PPIs were used in 86%, and one quarter with GI symptoms were on prokinetic drugs. In those with SRC ever: 77% were on current ACE inhibitors, one third were on dialysis and 10% had received kidney transplants. It is recommended that ACE inhibitors not be discontinued post SRC. 19% of MRSS >10 were on methotrexate whereas only 3% were using D-penicillamine which was not recommended in the guidelines. In severe ILD, there was significant variability in use of cyclophosphamide. There was also variation between centers with >50 patients for treatment for inflammatory arthritis (with hydroxychloroquine and D-penicillamine). The rate of ordering some investigations such as HRCT was different at some centers, but obtaining echocardiograms annual did not differ and was at a rate of >90%.

Eular guidelineMedication% of CSRG patients
Raynaud's phenomenon  
1. Calcium channel blockers should be considered for first-line therapy for SSc-related RP and intravenous iloprost, or other available i.v. prostanoids, should be considered for severe SSc-related RP.Calcium channel blockers47.3
Digital ulcers (active)  
2. Intravenous prostanoids, (particularly iv iloprost) should be considered for the treatment of active digital ulcers.Iloprost<1%
3. Bosentan should be considered in treatment of diffuse SSc patients with multiple DU, after failure of CCBs and, usually, prostanoid therapy.Bosentan9.2
4. Bosentan should be strongly considered in the treatment SSc-related PAH.Bosentan19
5. Sitaxentan may be considered in treatment of SSc-related PAH.Sitaxentan11.9
6. Sildenafil may be considered in treatment of SSc-related PAH.PDE5 inhibitors14.3
7. Intravenous epoprostenol should be considered for the treatment of severe SSc-related PAH.Epoprostenol6
Skin involvement (mRSS>10)  
8. Methotrexate may be considered in the treatment of skin involvement in early diffuse SSc.Methotrexate18.8
9. Cyclophosphamide should be considered in the treatment of SSc-related ILD.Cyclophosphamide)16.2
Renal crisis  
10. Angiotensin converting enzyme inhibitors should be used in the treatment of scleroderma renal crisis.ACE inhibitors77.1
Gastrointestinal involvement  
12. Proton pump inhibitors should be used for prevention of SSc-related GERD, esophageal ulcers and strictures.PPIs (GERD)85.5
13. Prokinetic agents should be used for the management of SSc-related symptomatic motility disturbances (dysphagia, GERD, delayed gastric emptying, bloating, pseudoobstruction, etc.)Promotility agents (GERD)24.6
 Promotility agents (delayed gastric emptying)27.8
 Promotility agents (pseudo-obstruction)40.3
 Promotility agents26.8

Treatment and investigation practice frequency and variation among centers with n>50

 A N=52B N=65C N=86D N=98E N=154F N=185 
N=640Frequency (%)Frequency (%)Frequency (%)Frequency (%)Frequency (%)Frequency (%)P-value
Raynaud's phenomenon       
N (%) with RP52 (100)65 (100)86 (100)91 (94.8)152 (100)183 (98.9)0.002
Calcium Channel Blocker25 (48.1)25 (38.5)41 (47.7)50 (54.9)62 (40.8)85 (46.4).280
Iloprost0 (0)0 (0)0 (0)0 (0)1 (0.7)0 (0).678
PDE5 Inhibitors5 (9.6)2 (3.1)1 (1.2)0 (0)8 (5.3)6 (3.3).036
Digital ulcers (DU) (ever)       
N (%) with DU ever28 (53.8)31 (47.7)56 (65.1)47 (48.0)56 (36.4)60 (32.4)0.000
Calcium Channel Blocker15 (53.6)11 (35.5)28 (50)28 (59.6)28 (50.0)39 (65.0).131
Bosentan2 (7.1)3 (9.7)2 (3.6)1 (2.1)2 (3.6)4 (6.7).650
Skin involvement (MRSS>10)       
N (%) with MRSS>1021 (40.4)42 (64.6)54 (62.8)47 (48.0)76 (49.4)88 (47.6)0.020
Corticosteroids3 (14.3)8 (19.0)8 (14.8)15 (31.9)16 (21.3)23 (26.1)0.299
D-penicillamine0 (0)1 (2.4)6 (11.1)0 (0)1 (1.3)1 (1.1)0.004
Methotrexate2 (9.5)9 (21.4)8 (14.8)8 (17.0)6 (8.0)15 (17.0)0.387
Cyclophosphamide3 (14.3)0 (0)1 (1.9)5 (10.6)3 (4.0)8 (9.1)0.062
N (%) with dysphagia31 (59.6)41 (63.1)55 (64.0)54 (55.1)94 (61.0)94 (50.8)0.236
Esophageal Dilatation7 (22.6)11 (26.8)8 (14.5)12 (22.6)35 (37.2)4 (4.3)0.000
Inflammatory arthritis       
N (%) with inflammatory arthritis19 (37.3)23 (35.4)71 (83.5)42 (44.7)32 (21.2)39 (21.1)0.000
Methotrexate1 (5.3)7 (30.4)10 (14.1)9 (21.4)4 (12.5)8 (20.5)0.259
Hydroxychloroquine5 (26.3)0 (0)10 (14.1)11 (26.2)7 (21.9)13 (33.3)0.023
D-penicillamine0 (0)0 (0)7 (9.9)0 (0)0 (0)0 (0)0.008
Corticosteroids4 (21.1)8 (34.8)10 (14.1)16 (38.1)6 (18.8)13 (33.3)0.039
Inflammatory myositis       
N (%) with inflammatory myositis3 (5.9)11 (17.2)42 (50.6)11 (11.7)6 (3.9)18 (10.1)0.000
Methotrexate1 (33.3)4 (36.4)8 (19.0)2 (18.2)1 (16.7)5 (27.8)0.826
N (%) with PAH1 (2.2)2 (3.3)1 (1.3)4 (4.2)10 (7.4)24 (14.4)0.001
N % with ILD13 (25.0)28 (44.4)36 (43.4)35 (36.1)38 (25.5)83 (45.4)0.001
Corticosteroids3 (23.1)7 (25.0)4 (11.1)12 (34.3)11 (28.9)25 (30.1)0.284
Cyclophosphamide4 (30.8)1 (3.6)2 (5.6)3 (8.6)1 (2.6)10 (12.0)0.037
Axathioprine2 (15.4)6 (21.4)4 (11.1)2 (5.7)3 (7.9)2 (2.4)0.037
* P-values measure variation among centers     


Although the guidelines were not published until after our standard of care was established, the SSc experts were mostly practicing in accordance with EULAR/EUSTAR recommendations. Methotrexate use for skin was only 19% for current use, but we did not study ever use so it may have been higher if studied in that way. CSRG practices were generally comparable to recently published guidelines; however, use of iloprost for digital ulcers and severe RP differed from guidelines as this drug is not approved in Canada. The CSRG adherence is a best case' scenario as these are centers with an interest in SSc and queries for non-adherence to recommended tests occur in the database. The adherence to EULAR/EUSTAR SSc guidelines in general rheumatology practice is likely far less than in SSc centers.

To cite this abstract, please use the following information:
Pope, Janet E., Harding, Sarah, Khimdas, Sarit, Bonner, Ash, Baron, Murray; An Analysis of Agreement of Guidelines for Management in SSc from a Large Database (CSRG: Canadian Scleroderma Research Group). [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1202
DOI: 10.1002/art.28968

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