Interstitial Lung Disease Remains the leading Cause of Death in Patients with Systemic Sclerosis, Despite Aggressive Treatment with Cyclophosphamide

Background/Purpose:

Studies suggest that despite aggressive treatment of interstitial lung disease (ILD) secondary to systemic sclerosis with cyclophosphamide, over one third of patients will experience a decline in lung function, death or require a lung transplant. The objective of this retrospective cohort is to document the causes of mortality in a UK based population with systemic sclerosis based in a tertiary centre in the North East of England over a period of seven years, and to review the causes of death in those patients treated aggressively with cyclophosphamide.

Methods:

All patients attending a North East of England Tertiary Medical Centre with a diagnosis of systemic sclerosis were identified using the departmental database. From this group, all patients who died between 2003 and 2010 were identified. Medical records were reviewed and government death certificates were obtained for these patients. Patients were excluded from the study if either of these resources were not available.

Results:

Of the twenty patients identified, five were male and fifteen were female. Four patients had a diagnosis of diffuse systemic sclerosis. Sixteen had a diagnosis of limited disease.

More patients died of non-systemic sclerosis related (NSR) causes (55%) than systemic sclerosis related (SR) causes. These included cancer (not related to cyclophosphamide) (25%), infection (15%), and other chronic disease (15%). Of the eleven patients treated with cyclophosphamide therapy, nine (82%) died of their underlying lung disease. The leading scleroderma related cause of death in our cohort was ILD (30% of all deaths), followed by pulmonary hypertension (10% of all deaths).

Conclusion:

This highlights the importance of screening for organ complications of systemic sclerosis. Patients are, however more likely to die of non-systemic sclerosis related diseases. Physicians should therefore also remain vigilant for NSR disease such as malignancy, which accounted for 25% of deaths.