Scleroderma renal crisis as a possible complication of cyclosporin a withdrawal

Abstract

The pharmacological treatment of systemic sclerosis (SSc) has been discouraging. In view of this, therapeutic trials with cyclosporine A (CyA) are outstanding particularly in patients with a rapid cutaneous involvement. We report the case of a patient whose SSc cutaneous manifestations dramatically improved with CyA therapy. Its subsequent withdrawal led to a quick onset of a fatal sclerodermal renal crisis. We believe that in our patient there could have been a cause and effect relationship between the suspension of CyA and kidney sclerodermal complication.

Inflammatory myopathy associated with mixed connective tissue disease and scleroderma renal crisis

“…It has been postulated that the ability of corticosteroids to inhibit prostacyclin production, which may result in increased ACE activity, contributes to the development of SRC. Scleroderma renal crisis has been reported in both hypertensive and nonhypertensive patients, 5,9,10,12,13,27 in patients already on ACE inhibitors, in patients without prior steroid treatment, in association with cyclosporine initiation, 28 in association with cyclosporine withdrawal, 4 after use of essential (volatile) oils, 17 and without antecedent factors. The vessel changes on kidney biopsy are similar to the microangiopathic abnormalities found on muscle biopsy in patients with MCTD and necrotizing myopathy.…”

Calcineurin inhibitors in systemic sclerosis – a systematic literature review

“… 24 Finally, 27 review articles were excluded, and the remaining 37 identified primary research articles were included into the analysis. 24 – 60 The 37 articles consisted of 19 case reports, 15 case series, 1 open-label, randomized controlled trial with a no-treatment arm (group I iloprost vs group II iloprost with low-dose CsA), one open-label, prospective study with a historical control group (CSA group vs historical placebo group of the chlorambucil trial), 61 and one retrospective study without control (patients received prednisolone (0.5 mg/kg/d) and tacrolimus; Table 1 ).…”

“…Altogether, hypertension was noticed in 13 patients. 26 , 31 – 33 , 36 , 42 , 48 , 59 Pathological renal biopsy was reported in 11 patients. 26 , 31 , 32 , 36 , 40 , 48 , 58 , 59…”

“…Ando et al 27 Basso et al 29 Basso et al 30 Casoli et al 31 Clements et al 33 Davies and Dunn 35 Number Pat. 1 9 1 1 10 1…”

“…Amor and Dougados 26 Casoli et al 31 Chen et al 32 Clements et al 33 Denton et al 36 Francès et al 38 Gisslinger et al 39 Heickendorff et al 40 Patients Ippoliti et al 42 Ishida et al 43 Morton and Powell 47 Vayssairat et al 53 Wörle et al 54 Zachariae et al 58 Zentilin et al 60 Patients 3…”