Systemic sclerosis therapy with iloprost: a prospective observational study of 30 patients treated for a median of 3 years.
Iloprost is useful in the short-term treatment of severe Raynaud's phenomenon and ischaemic ulcers in patients with systemic sclerosis (SSc), but its long-term effects are largely unknown. The aim of this study was to report long-term outcome (median follow-up 36 months) in a prospective observational study of a cohort of 30 consecutive patients with SSc who received iloprost therapy with maintenance infusions every 3 weeks after an initial cycle of 5 consecutive days. At the end of the observation, compared to the pretreatment point, we observed complete healing of digital ulcers in 19/21 patients (90%), a decrease of the Raynaud's phenomenon visual analogue score from 10/10 (25th-75th percentile 7-10) to 5/10 (4-6.75) ( P <0.001) and, in patients with diffuse cutaneous involvement, of the modified Rodnan skin thickness score from 25.5 (16.5-31.5) to 16 (13.5-20) ( P = 0.02), minimal improvement of the Health Assessment Questionnaire from 0.87 (0.68-1.37) to 0.75 (0.62-1.25), which was neither statistically nor clinically significant. The forced vital capacity was not significantly changed, but the diffusion capacity corrected for the alveolar volume decreased from 71% (54-76.7) of the expected value to 62% (51.5-71) ( P = 0.02). In one patient with limited SSc a positive effect on pulmonary hypertension was observed. Six patients, after a median of 25 months of treatment and healing of digital ulcers, discontinued the therapy; after a median of 10 months ulcers did not recur in five of these six. Other reasons for discontinuation were: tolerability (1), disease progression (normotensive renal crisis: 1), and death due to intracranial haemorrhage (1). This same patient had previously suffered a central retinal vein thrombosis. In conclusion, long-term therapy with iloprost in patients with SSc has a durable effectiveness on ischaemic ulcers and Raynaud's phenomenon, but it is not possible to conclude that the natural history of the disease was modified.
Bettoni
L
L
Servizio di Reumatologia ed Immunologia Clinica, Spedali Civili and University, I-25124 Brescia, Italy.
Geri
A
A
Airò
P
P
Danieli
E
E
Cavazzana
I
I
Antonioli
C
C
Chiesa
L
L
Franceschini
F
F
Grottolo
A
A
Zambruni
A
A
Radaeli
E
E
Cattaneo
R
R
D000328:Adult; D000368:Aged; D015331:Cohort Studies; D005260:Female; D005385:Fingers / Q000098:blood supply; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology; D016285:Iloprost / Q000009:adverse effects / Q000627:therapeutic use*; D007511:Ischemia / Q000150:complications; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D010975:Platelet Aggregation Inhibitors / Q000009:adverse effects / Q000627:therapeutic use*; D011446:Prospective Studies; D011928:Raynaud Disease / Q000188:drug therapy / Q000209:etiology; D012129:Respiratory Function Tests; D012595:Scleroderma, Systemic / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D012867:Skin / Q000473:pathology; D014456:Ulcer / Q000188:drug therapy / Q000209:etiology; D014665:Vasodilator Agents / Q000009:adverse effects / Q000627:therapeutic use*
D010975:Platelet Aggregation Inhibitors; D014665:Vasodilator Agents; D016285:Iloprost