Systemic sclerosis treated with dexamethasone pulse

Introduction

Various drugs used in the treatment of systemic sclerosis include corticosteroids, D-penicillamine, colchicines, azathioprine and cyclosporin-A with or without physiotherapy. None of these measures were able to halt the progress of the disease. Pulse therapy with corticosteroids (intermittent high dose) has been used with gratifying success in several skin diseases[1],[2],[3],[4] with relatively mild or no side effects as compared to prolonged daily corticosteroids therapy. The patient being reported had progressive disease for the last 8 years, which showed remarkable improvement in all her symptoms and signs with repeated, intermittent high dose of dexamethasone.

Case Report

A 52-year-old woman having picture of systemic sclerosis of 8-years duration presented on May 4, 1994 with purse string mouth, beaked nose, expressionless face, bound down skin of face, forearms, dorsa of hands, feet and toes along with depigmented skin on the forehead. Her fingers were spindle-like with ulcers in few of them. There was history of Raynaud′s phenomenon through oui the year which was more in winter season. She was having dyspnoea which was continuous throughout the day. There was no dysphagia but she was having diarrhoea off and on. Various combinations and dosages of drugs like systemic corticosteroids, methotrexate, colchicines, nifedipin and cizapride have not helped her much. She was having severe dyspnoea and was not able to open her mouth. Investigation showed haemoglobin 10.4gm%, total leukocyte count 1 6,900/cmm with polymorph 77%, lymphocytes 14%, monocytes 8% and eosinophils 1 %, ESR was 59mm in first hour, platelets 2,40,000/cmm. Routine urinalysis was normal. Serum bilirubin, alkaline phosphatase, SGOT, SGPT, CPK, LDH, blood urea and serum creatinine were within normal limits. Lung function test done in September, 1993 showed diffused interstitial pulmonary involvement with peribronchial fibrosis at the level of the small airways. On May 25′h, 1994 dexamethasone pulse therapy which comprised intravenous infusion of 100mg dexamethasone in 500ml of 5% glucose over 1-11/2 hour on 3 consecutive days repeated on every 4 weeks was started. One month after first pulse, her dyspnoea was less and she felt better. After third pulse she stopped having diarrhoea, dyspnoea improved by 40%, ulcer in fingers healed, the skin on hands, forearms and face started loosening she was able to open her mouth by 20% and stopped getting Raynaud′s phenomena but was still getting swelling of her fingers on exposure to cold water. She continued improving with successive pulses. After 9th pulse there was no dyspnoea, no swelling of the fingers, opening of mouth improved further with improvement in binding down of skin. Investigation done before 9th pulse on February 21, 1995 showed negative ANF, ESR 60mm in first hour and random blood sugar was 111 mg%. Lung function test done after 17th pulse showed marginal improvement in the airway obstruction. The diffusion coefficient was normal, indicating normal gas exchange in the functioning alveoli. She noticed irritation, weakness and loss of appetite 3′d day after 17, 18 and 19 pulse which disappeared in 2-3 days with 0.5 mgdexame-thasone given orally. She continued receiving dexamethasone pulses 4 weekly interval and thus receiving 21 pulses. At the time of last pulse she was completely free from dyspnoea, Raynaud′s phenomenon, was able to open her mouth almost completely, skin on face, hands, fingers, forearms was almost 80% softer and one could pinch the skin with fingers.

After 21St pulse she was lost to follow-up.

Discussion

Dexamethasone pulse therapy consists of 100mg dexamethasone dissolved in 500m1 of 5% glucose transfused over an hour on three consecutive days, which is repeated every month. We used dexamethasone pulse therapy first time in 1981 for treating a case of Reiter′s diseases where almost all the known method of treatment virtually failed. Encouraged with this subsequently we used it to treat pemphigus[6] with addition of cyclophosphamide in the dexamethasone pulse and we called it dexamethasone-cyclophosphamide pulse (DCP) therapy.

In about 367 patients of pemphigus[7] we were able to control the disease completely and were able to withdraw the treatment completely. Majority of these patients are without any medicine and with no relapse in symptoms and signs varying from 5-10 years.[7] In 1990, Pasricha et al[8] used dexamethasone pulses to reverse the systemic sclerosis. Subsequently, few more workers[9],[10] have used dexamethasone pulse in systemic sclerosis with significant symptomatic and clinical improvement. Sharada et al[10] used dexame-thasone 100mg once in a month for 6 months in 18 patients in a double blind placebo controlled study and reported definite improvement by the pulse though it was confined to skin only. It is possible that if they would have used dexame-thasone pulse 100mg for 3 consecutive days repeated every month for prolonged period and would have not terminated the therapy at 6 months, their patients could have been benefited as of the present and other workers who used full dose for prolonged period.

In the present patient, the dexamethasone pulses were not only able to arrest the progress of the disease which was going on for 8 years but were also able to reverse significantly all signs and symptoms to almost normal. At the end of the therapy patient was free from Raynaud′s phenomenon, dyspnoea, binding down of the skin, was able to open mouth almost fully softer and normal skin.

It is also to be noted that with dexamethasone pulse or DCP side effects commonly seen with prolonged corticosteroids therapy like electrolyte imbalance, acne, diabetes mellitus, weight gain, hypertension, osteoporosis, etc have not been encountered. The main side effect of the pulse therapy is increased susceptibility to infection or reactivation of tuberculosis.[7]