Scleroderma and Related Disorders [202–212]

Background: The objective of this multicentre regional audit was to determine: (a) clinical presentations leading to treatment with ivCYC, (b) time between diagnosis of ILD and starting treatment with ivCYC, (c) outcome of treatment with ivCYC and (d) proportion of patients with SSC-ILD whose care involved respiratory physicians. Methods: A retrospective case note audit was carried out in patients who had iv CYC for SSc-ILD between 2000 and 2009. This was approved by audit committees at Queen’s Medical Centre, Derby Acute Hospitals and Kings Mill Hospital NHS Trusts. Paired t test was used to assess the effect of i.v. CYC on lung function tests. All statistical analyses were carried out using SPSS v14. Results: 30 patients with SSc and 4 patients with mixed connective tissue disease (MCTD) were identified. There were 24 women and 10 men. 11 had limited SSc, 19 diffuse SSc and 4 MCTD. The mean age at onset of Raynaud’s phenomenon, diagnosis of SSc/MCTD and treatment with iv CYC was 40, 46 and 50 years, respectively. Among patients with DCSSc, 7(37%)and 3(16%) were positive for anti-Scl70 and anti-Centromere antibodies, respectively. Among LCSSc patients 4(36%) and 2(18%) were positive for Anti SCl70 and anti-Centromere antibodies, respectively. The clinical presentation leading to treatment was asymptomatic decline in serial lung function; decline in serial lung function plus shortness of breath; and symptomatic shortness of breath in 16, 5, 15 patients, respectively. The median interval between HRCT chest and treatment with iv CYC was 4.75 (0.5–18) month. A respiratory opinion was obtained for 23 out of 34 patients. The cumulative iv CYC dose was 5.6 g (0.7-12) over 5.4 (1-12) months. Fifteen patients received PCP prophylaxis and 17 did not. 22 patients received steroids: the median peak daily prednisolone dose was 14 mg/d. One patient received 1g of Methylprednisolone on one occasion. 23 patients were able to complete the course. The known reasons for not completing the course of treatment was due to: neutropaenia (3) and other side effects (3) patients declining further treatment. Eight patients had transient side effects: confusion (1), nausea (5) and vomiting (3), diarrhoea (2), fatigue and arthralgia (1). This resulted in a change in dose of iv CYC in 1. No patient developed haemorrhagic cystitis. At 12 months post commencing treatment with iv CYC, the TLC increased from 3.87 l (s.d. 1.22) to 3.94 l (0.86) (P = 0.24), FVC increased from 2.62 l (s.d. 0