International Journal of Dermatology, Vol. 34, No. 10, October 1995 PHARMACOLOGY AND THERAPEUTICS EFFICACY OF DEXAMFTHASONE PULSE THERAPY IN PROGRESSIVE SYSTEMIC SCLEROSIS BALLAMBAT SATHISH PAI, M.D., D.V.D., CHAKRAVARTHI R. SRINIVAS, M.D., LAKSHMINARAYANAN SABITHA, M.D., SHRUTAKIRTHI D. SHENOI, M.D., CHANDRASEKHARAN N. BALACHANDRAN, M.D., AND SANDHYA ACHARYA, M.D. Abstract Background. Systemic sclerosis is a disease of unknown etiology for which no specific treatment is effective. Pulse therapy with corticosteroids has been tried for various au- toimmune disorders with minimal side effects. We under- took this study to determine the efficacy of dexamethasone pulse therapy in progressive systemic sclerosis (PSS). Methods. Five women with PSS between the ages 30 and 60 years, received TOO mg dexamethasone in 500 mL of 5% dextrose by slow intravenous infusion over 3 hours for 3 consecutive days, once a month. Results. All patients had symptomatic and clinical improve- ment. The vital capacity improved in three and posttreatment histopathologic regression was seen in two patients. Conclusions. Dexamethasone pulse therapy may provide an additional option for treating systemic sclerosis. IntJ Dermatol 1995; 34:726-728 Progressive systemic sclerosis (PSS) is a generalized dis- ease of connective tissue, involving mainly the skin, gastrointestinal tract, lungs, heart, and kidneys. Treat- ment of systemic sclerosis (scleroderma) presents a challenge to both the patient and the physician. Estab- lished approaches include long-term physiotherapy, disease-modifying agents, such as D-penicillamine, and treatment of any organ involvement.' Pulse therapy in- volves intravenous administration of "suprapharmaco- logic doses" of prednisolone (0.5 g-2 g) or its equiva- lents.^ This dose is usually given on 3 consecutive days and possibly once a month thereafter.^ The indications are pemphigus vulgaris,^ pyoderma gangrenosum,"* sys- temic lupus erythematosus (SLE),^ rheumatoid arthritis,® lupus nephritis,'' and polyarteritis nodosa.*' From the Department of Dermatology, Kasturha Medical College & Hospital, Manipal, India. Address for correspondence: Dr. B. Sathish Pai, Lecturer, De- partment of Dermatology, Kasturba Medical College & Hos- pital, Manipal 576 119, India. The common side effects are psychologic distur- bances, seizures, hypertension, sinus bradycardia, and increased blood glucose levels.'' Steroids are indicated in inflammatory and immunologically active forms of sys- temic sclerosis.'" This study was undertaken to deter- mine the efficacy of dexamethasone pulse therapy in l'SS. Materials and Methods The patients were five women with PSS, between the ages 30 and 60 years, who were hospitalized for pulse therapy after giving informed consent. The pretreatment investigations performed were as follows: hemoglobin, total and differen- tial leukocyte counts, platelet counts, urine examination, pulmonary function tests, electrocardiogram, and weight charting. The patients also underwent complete blood counts with differential and platelet count once a month be- fore each infusion. At the time of each physical examination, the patients were evaluated for general cushingoid appear- ance. Blood pressures were recorded before, during, and after each infusion. Patients' complaints about side effects were recorded, but not actively elicited. All patients received 100 mg dexamethasone In 500 mL of 5% dextrose by slow intravenous infusion over 3 hours for 3 consecutive days once a month. The total number of pulses ranged from 6 to 20. The patients were evaluated every 4 weeks using the following subjective criteria: im- provement in Raynaud's phenomenon, dysphagia, dysp- nea, itching, and overall patients' opinion. Changes in the induration of skin, erythrocytic sedimentation rate (ESR), and pigmentation were also noted. After six pulses, pul- monary function tests and skin biopsies were repeated and compared with pretreatment findings. RESULTS Symptomatic relief as well as clinical improvement was seen in all five patients (Table 1). The pretreatment pul- monary function tests showed restrictive lung disease in four patients and had not been done in one. The vital capacity improved in three patients after 6 pulses, whereas in one it remained static. The ESR that was raised in three decreased after receiving six pulses. In the other two patients the pretreatment ESR was normal.