Cyclosporine in erythema nodosum leprosum (ENL) reaction not responding to treatment

 Leprosy Mailing List –    February 26^th, 2011

Ref:    Cyclosporine in erythema nodosum leprosum (ENL) reaction not responding to treatment.From:  Naafs B., Munnekeburen, The Netherlands

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Dear Salvatore,

In response to the question of Dr. Antonio Salafia, LML 23-02-2011,  I have included 3 papers*, one in vitro and two clinical trials (one positive and one negative).  I'm inclined to go with van Gompel, more than with Rea.  I hope the latter will comment.

I myself treated 5 patients with Cyclosporine A, similar period as above-mentioned papers:

-         Two with mild ENL.  They improved, but I expected it to be the normal course of ENL.

-         Three with severe recurrent ENL.  In none of them it proved possible to prevent the recurrence.  It did not show effect in reversing acute nerve damage (acute nerve damage in ENL is most likely partly oedema driven).  I never used it again.  It may be that I used a too low dose, 5mg/kg.

ENL is episodic as you know.  I'm afraid the steroid treatment schedule in use (low dose and for long periods - three months - and repeated when needed, on advice of WHO/Novartis) is partly responsible for the chronic ENL we see at the moment. Additionally, I guess you have no access to thalidomide.

My present treatment is 2mg/kg prednisolone tapering off to zero within a month. If there is a new occurrence while the patient is still on prednisolone, I double the dose and  taper off within a month again (a kind of pulse treatment).  I try not  to give steroid maintenance. Instead, I use methotrexate, sometimes together with clofazimine.  I think it works.  I now even use lower dose of Clofazimine.  I still have to instigate a proper trial.

With kind regards,

Ben

*
 - Uyemura K, Dixon JF, Wong L, Rea TH, Modlin RL. Effect of cyclosporine A in erythema nodosum leprosum. J Immunol. 1986 Dec 1;137(11):3620-3.

- Miller RA, Shen JY, Rea TH, Harnisch JP.  Treatment of chronic erythema nodosum leprosum with cyclosporine A  produces clinical and immunohistologic remission.Int J Lepr Other Mycobact Dis. 1987 Sep;55(3):441-9.

- van Gompel A, van den Enden E, van den Ende J.  Cyclosporin A is not very effective in erythema nodosum leprosum (ENL).  Trop Geogr Med. 1994;46(5):331. No abstract available.