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Dermatomyositis: Observations on the Use of Cairo-Glasgow Study Group Postgrad Med J: first published as 10.1136/pgmj.54.634.516 on 1 August 1978. Downloaded from Postgraduate Medical Journal (August 1978) 54, 516-527. Dermatomyositis: observations on the use of immunosuppressive therapy and review of literature Cairo-Glasgow Study Group AHMED EL- GHOBAREY GEZA BALINT M.R.C.P. M.D. (Budapest) KAREL DE CEULAER W. CARSON DICK M.D. (Leuven) M.D., M.R.C.P. W. W. BUCHANAN M.D., F.R.C.P. TAHSIN HADIDI* T. A. HASSAN* F.R.C.P. M.R.C.P. The Centre for Rheumatic Diseases, University Department ofMedicine, Royal Infirmary, Protected by copyright. Glasgow, Scotland, and *Maadi Armed Forces Hospital, and Azhar University, Cairo, Egypt Summary The response to therapy and the subsequent Seven young adults, six of whom were male, all clinical course are briefly described as follows. suffering from dermatomyositis unassociated with Patient N.D. was treated with corticosteroids. The malignancy are described. These patients were not course of therapy, clinical response and changes in adequately controlled with high doses of corti- serum muscle enzymes are summarized in Fig. 1. costeroids but all responded when immunosuppressive Intravenous dexamethasone was discontinued at the therapy was also given. nineteenth week and by the end of the twenty-first week the dose of prednisolone was reduced to 10 mg/ day. Introduction http://pmj.bmj.com/ Dermatomyositis (as the name suggests) is a The patient was discharged at the end of the syndrome consisting of polymyositis associ- twenty-fourth week. clinical One year later, the patient had a relapse, the ated with skin lesions (Pearson, 1966a; Currie and subsequent course and response to treatment are Walton, 1971). The disease is predominantly found summarized in Fig. 2. Methotrexate was discon- in females and is of unknown aetiology, although tinued after 14 weeks and the daily dose of predniso- disordered immunological mechanisms have been lone gradually reduced to 2 5 mg/day at the end of suggested (Dawkins and Mastalgia, 1973). A clear the seventeenth week. At this time the was patient on September 28, 2021 by guest. association with malignancy has been documented clinically very much improved. in patients aged 40 years or more (Vanderploeg, Patient M.B. had a cervical sympathectomy for 1977; Curtis, Blaylock and Harrell, 1952; Bat- severe Raynaud's phenomenon of the hands but schwarov and Minkov, 1968). showed no improvement. One month after surgery, The effects of immunosuppressive drug therapy in the characteristic skin rash and progressive general- a group of patients with dermatomyositis whose ized muscle weakness of dermatomyositis developed. disease had proved resistant to corticosteroids are The diagnosis was confirmed by laboratory tests as now described and the literature is reviewed. outlined in Table 2. The course of the disease and the response to treatment are illustrated in Fig. 3. Patients Two years later, the patient had muscle aches, The clinical and laboratory data on the seven tired easily and had a recurrence of facial dis- patients are summarized in Tables 1 and 2. coloration and fever. Muscle enzyme levels were 0032-5473/78/0800-0516 $02.00 © 1978 The Fellowship of Postgraduate Medicine Postgrad Med J: first published as 10.1136/pgmj.54.634.516 on 1 August 1978. Downloaded from Dermatomyositis: use of immunosuppre-ssive therapy 517 b0 u0 b0 bt 0e X0 c bo o ° to > -Co0._0 -~ ~~~~- V: ~~~- .- C CO C C C C C CZ C rC r C r n C r C r C r bo Q YO YQ O cd a) ct W cQ Q~~~~ ~ ~~~ Cd C0dQ0 sQ° Qe° ; QQ° cl F- _ . 0 WC bo a to _) 6. m m N NC_m .NC_ C c r X wo Cd0-cX- r C wo .XX X . X r c r. x Sl .> C w ce 0 _ e 0 C a)~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~W r A C >. r . Protected by copyright. 0 *. 0 c ;E 0~~~~~~~.. -0 0 3d >~~~~~~~~~~c >: - >, L 04V) R. C) Ca, .C P£oo ._ cl N N .... N N N~~~bN ^ m. toCd < on C). r= r http://pmj.bmj.com/ cn~ 0 C m Zd0 0 CZ 0 Z S <CZ L S C) on September 28, 2021 by guest. o34 Qr ': *a I 0- 0, l0:;I-4 Postgrad Med J: first published as 10.1136/pgmj.54.634.516 on 1 August 1978. Downloaded from 518 Ahmed El-Ghobarey et al. TABLE 2. Laboratory data for seven patients with dermatomyositis Muscle Muscle enzymes ESR Pulmonary Electro- Skin Creatine Lactate Aspartate (mm/ function Patient Biopsy myography biopsy kinase dehydrogenase transaminase WBC first test (jm/ml) (gm/ml) (pm/ml) (cells/i) hour) N.D. Positive Positive Positive 182 255 67-2 114 x 1021 30 M.B. Positive Positive Positive 390 380 96 12-6 56 A.M.H. Positive Positive Not done 1 15-7 255 18-3 12-0 37 M.N.S. Positive Positive ,, , 120 240 19-2 5-3 20 E.H. Positive Positive , 9, 2000 420 76-8 12-4 64 M.A.I. Positive Positive 97 5 120 48-0 11-6 22 M.M. Pos:tive Positive 655 72 5 75 10-2 24 Restrictive defect Pulse/minm 5C 250k Protected by copyright. 200 Serum muscle enzymes (j.m/ml) ..0* 50 Muscle power 6- Dexamethosone 40- (mg 20v0 150 http://pmj.bmj.com/ Prednisolone 100 (mg) 50 0 2 4 6 8 10 12 14 16 18 20 22 24 Weeks FIG. 1. (Patient N.D.) Changes in the muscle power, on September 28, 2021 by guest. pulse rate and serum muscle enzymes during the first course of treatment with oral prednisolone and intra- venous dexamethasone. Creatine kinase ----; Lactate dehydrogenase - ; SGOT aspartate transaminase-. normal. Prednisolone was increased to 60 mg/day. returned to normal in three weeks and the tem- The patient developed back pain and an X-ray perature settled after 5 weeks. Despite high doses of showed collapsed lumbar vertebrae. Prednisolone prednisolone over a period of eight weeks, muscle was stopped and cyclophosphamide in daily doses of power showed only slight improvement. Because of 100 mg produced symptomatic relief. this, intravenous injections of 50 mg methotrexate Patient A.M.H. was begun on daily oral doses of were given every 5 days for 12 weeks and subsequent 60 mg prednisolone. Serum muscle enzyme had improvement of muscle power enabled the patient Postgrad Med J: first published as 10.1136/pgmj.54.634.516 on 1 August 1978. Downloaded from Dermatomyositis: use of immunosuppressive therapy 519 150 Pulse/mi 00 50_ 0 275 250 225 - 200 Serum muscle 175 enzymes (,mr/ml) 150 - 125 100 _ 75 - 50 25 Muscle power 4 Methotrexate 40 (mg v) 208 Prednisolone 100 (mg) 50' Protected by copyright. 0 2 4 6 8 10 12 14 16 18 20 22 Weeks FIG. 2. (Patient N.D.) Changes in the muscle power, pulse rate and serum muscle enzymes during the second course of treatment with intravenous methotrexate, after relapse. Creatine kinase ---- ; Lactate dehydrogenase SGOT aspartate transaminase ..... 100__ Pulse/min 50 400 http://pmj.bmj.com/ 300 Serum muscle enzymes (Mm/ml) 200 \" 200 100 , \ 50 -... C on September 28, 2021 by guest. Muscle power 4 (grade) 2 -, 7 77 Methotrexote 40 (mg i.v.) 20 75 Prednisolone 50 (mg) 25 0 2 4 6 8 10 12 14 16 18 20 22 24 26 28 Weeks FIG. 3. (Patient M.B.) Changes in the muscle power, pulse rate and serum muscle enzymes during the course of treatment with prednisolone and intravenous methotrexate. Postgrad Med J: first published as 10.1136/pgmj.54.634.516 on 1 August 1978. Downloaded from 520 Ahmed El-Ghobarey et al. cardiomegaly and early heart failure in addition to other features of dermatomyositis. After 8 weeks, there was no significant improvement with 80 mg prednisolone, and cyclophosphamide 400 mg/day was begun. Four weeks later, muscle power had improved and myalgia had disappeared. The heart size had returned to normal and there were no signs of heart failure. Four months later the patient's condition relapsed. At that time, the patient was receiving 10 mg of prednisolone and 100 mg cyclophosphamide/day. Increasing the dose of the latter to 300 mg/day brought about an immediate improvement. Two years after admission the patient's condition was satisfactory and he continued to take 10 mg pred- nisolone alone. Patient M.A.I. was initially thought to have myasthenia gravis until he developed skin lesions typical of dermatomyositis. Laboratory tests con- firmed the diagnosis (Table 2). When the patient failed to respond to 80 mg prednisolone daily after 10 weeks, intravenous injection of methotrexate was begun, beginning with Protected by copyright. ... ... ... FIG. 4. Extensive skin ulceration due to intravenous methotrexate. to walk. During this time, the patient also received prednisolone which was continued, after metho- trexate was stopped, in a maintenance dose of 10 mg/day. http://pmj.bmj.com/ Patient M.N.S. started oral corticosteroid therapy in a daily dose of 80 mg prednisolone. Since there was only slight improvement in the patient's clinical condition, methotrexate was begun after 2 months in gradually increasing doses of 5 to 50 mg/week by intravenous injection. Five weeks after beginning methotrexate, the patient developed necrotic skin on September 28, 2021 by guest. ulceration (Fig. 4) which healed when methotrexate was discontinued over a period of 3 weeks, by which time the muscle power had improved considerably. For maintenance immunosuppressive therapy, cyclo- phosphamide 100 mg orally/day was substituted for intravenous methotrexate which had caused recur- rence of the skin ulceration when it was given again to the patient after the ulcers had healed.
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