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ORIGINAL ARTICLE
Year : 2011  |  Volume : 2  |  Issue : 1  |  Page : 10-12  

Dexamethasone- cyclophosphamide pulse in collagen vascular diseases: An observation


Department of Dermatology and Venerology, NRS Medical College, Kolkata, India

Date of Web Publication21-Apr-2011

Correspondence Address:
Sudip Das
Room No. 18 & 19, OPD Building, Skin STD & Leprosy Department, NRS Medical College, 138, AJC Bose Road, Kolkata - 700 014
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2229-5178.79858

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   Abstract 

Background: Treatment of collagen vascular diseases like systemic sclerosis, dermatomyositis, systemic lupus erythematosus (SLE) and even overlap syndromes has been difficult since long. Monumental success of dexamethasone-cyclophosphamide pulse (DCP) in pemphigus has prompted many a dermatologist to try it in other autoimmune diseases. Materials and Methods: DCP was given as per standard regimen for six to nine pulses. Immunosuppressives were given for 12-18 months in dermatomyositis, SLE, and overlap syndrome, and for 12 months in systemic sclerosis. Daily dose of steroid was tapered off gradually. Results: The treatment resulted in 90% improvement in skin binding in systemic sclerosis, 80% in exertional dyspnea, 40% in dysphagia, but minimum improvement was seen in Raynaud"s and digital tip ulcerations. No improvement in pigmentation was noted. In SLE, malar rash cleared in 70%, joint pain in 80%, oral ulcerations reduced in 80%, fever in 98%, and photosensitivity improved in one-third of patients. In dermatomyositis, improvement in muscle tenderness was seen in 100%, improvement in proximal myopathy and heliotrope rash in 80%, and improvement of shawl sign was observed in 80% of the patients. Some flattening of Gottron papules and plaques was noted in some patients. Both overlap patients improved significantly. Out of 24 patients, three were lost to follow-up, one resorted to homeopathic medicine and two expired (one dermatomyositis, one SLE). Side effects like hypertension, hyperglycemia, pyoderma, fungal infections, obesity, psychosis, etc. were seen in 25-30% of patients. Conclusions: We conclude that DCP is relatively safe, effective as well as cheap compared to methylprednisolone pulse. Side effects are also less compared to daily regimen of steroids. We also observed that patients who reported early and put on pulse early responded better.

Keywords: Collagen vascular disease, dexamethasone-cyclophosphamide pulse


How to cite this article:
Das S, Giri PP, Roy AK. Dexamethasone- cyclophosphamide pulse in collagen vascular diseases: An observation. Indian Dermatol Online J 2011;2:10-2

How to cite this URL:
Das S, Giri PP, Roy AK. Dexamethasone- cyclophosphamide pulse in collagen vascular diseases: An observation. Indian Dermatol Online J [serial online] 2011 [cited 2019 Jun 27];2:10-2. Available from: http://www.idoj.in/text.asp?2011/2/1/10/79858


   Introduction Top


Since time immemorial, treatment of collagen vascular diseases has been difficult with varied clinical response. Monumental success of dexamethasone-cyclophosphamide pulse (DCP) in autoimmune bullous diseases has prompted many a dermatologist to use DCP in various collagen vascular diseases and other autoimmune diseases, e.g., Reiter's disease, generalized lichen planus, alopecia subtotalis, etc. [1]

Dhabhai et al., [2] in an Indian study reported significant improvement in Quality of Life (QOL) of systemic lupus erythematosus (SLE) patients only after three to four pulses. Methylprednisolone pulses were routinely given in SLE by physicians, but are expensive in Indian context.

Pemphigus, a common immunobullous disease of skin and mucous membrane affecting both sexes of all ages was almost fatal before the use of corticosteroids. Use of pulse corticosteroid therapy had benefited many patients, with mortality presently dropping to negligible levels as evident in a majority of the hospital data. The reason of clubbing SLE, dermatomyositis, and systemic sclerosis is that these diseases share a common clinical condition involving abnormality in structure, synthesis, or degradation of collagen, although the predominant morphology and etiopathogenesis may vary from disease to disease.

Parsricha et al. reported improvement in Indian patients of systemic sclerosis and Reiters disease with DCP therapy. [3]

Our aim was to evaluate the efficacy of DCP in collagen vascular diseases in Indian patients.


   Materials and Methods Top


Twenty four patients of collagen vascular disease were enrolled in the study, after screening for clinical and hematologic parameters. Detailed history was taken for each of the patients. Patients with history of diabetes, hypertension, and altered renal parameters were not enrolled in study. Fasting blood sugar, routine urinalysis, blood urea, serum creatinine, and ECG were done in all patients. Serum antinuclear antibody (ANA), double stranded deoxyribose nucleotide antibody (dsDNA), creatinine phospokinase-muscle antibody (CPK-MM) were estimated in all the patients. Clinical and biochemical features were assessed to see whether they fit into the ARA criteria and the patients were accordingly grouped into PSS, SLE, dermatomyositis or overlap syndrome. Written consent was taken from each patient.

Patients were treated with DCP which includes the following. Injection dexamethasone, 100 mg, was dissolved in one unit of 5% dextrose and run over 3-4 hours slowly for 3 consecutive days. On the 2 nd day, 500 mg of Injection cyclophosphamide was added to 5% dextrose. Pulse was repeated every 28 days and a total of six to nine pulses were given in each patient. In addition, patients with dermatomyositis received azathioprine 50 mg and also daily steroid prednisolone in a dose of 0.5 mg/kg body weight, till the erythema subsided. Patients with systemic sclerosis were given diltiazem for Raynauds phenomenon, and patients of SLE were given hydroxychloroquine 200 mg per day.


   Results Top


There were 14 patients of systemic scelorosis, 4 each of dermatomyositis and SLE, and 2 of overlap syndrome. We lost three patients in the course of treatment. One patient of PSS resorted to homeopathic mode of treatment and two other patients of dermatomyositis and SLE died of cardiac arrhythmia and renal failure, respectively, which may be due to causes unrelated to it. Twenty-one patients completed the study. Many of the patients showed significant improvement after three to four pulses.

Improvement status

The improvement status of PSS and SLE patients and also the side effects are given in [Table 1], [Table 2] and [Table 3].
Table 1: Improvement in PSS patients (13)

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Table 2: Improvement in systemic lupus erythematosus patients (4)

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Table 3: Side effects

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In two dermatomyositis patients, there was complete improvement in myopathy, heliotrope rash and Shawl sign is a poikilodermic rash surrounding the shoulder,upper anterior part of chest and posterior part and upper part of arm as if a reddish shawl is applied on the area. Gottron papules also improved in one patient. There was significant improvement in both overlap patients. In DM + PSS patients, there was improvement in myopathy, heliotrope rash, skin binding, dysphagia and poikiloderma. We noticed significant improvement in oral ulcers, myopathy, photosensitivity and poikiloderma in DM + SLE overlap patients.


   Conclusions Top


Many studies are done to assess the safety and efficacy of pulse therapy in dermatologic disorders like pemphigus vulgaris, systemic sclerosis, dermatomyositis, Reiter's disease, and even vitiligo. [4] DCP is an accepted modality of treatment in many dermatologic and non-dermatologic diseases and we tried the same in collagen vascular diseases. Side effects like obesity, peptic ulceration, osteoporosis are much less compared to conventional daily dose steroid therapy. The only absolute contraindications are pregnancy and lactation. Relative contraindications are severe hypertension, stroke, myocardial infarction, peptic perforation, etc.

It was possible to induce remission in majority of our patients with PSS. Skin binding improved significantly in 90% of our patients although Raynauds phenomenon, pigmentary changes improved the least. In SLE, oral ulceration, fever, joint pain and malar rash improved significantly, whereas photosensitivity tended to persist even with pulse therapy.

Therefore, we conclude the following about DCP regime:

  • safe and effective new weapon to treat collagen vascular diseases;
  • patient compliance was good;
  • cheap compared to methylprednisolone pulse;
  • safer than oral steroid regime;
  • no significant long-term side effect.


 
   References Top

1.Parsricha JS. Introduction. Pulse therapy in pemphigus and other diseases. Parsricha Js Editor, New Delhi. Pemphigus foundation:2000.  Back to cited text no. 1
    
2.Dhabhai R, Kalla G, Singhi MK, Ghiya BC, Kachhwa D. Dexamethasone-cyclophasphamide therapy in systemic lupus erythematosus. Indian J Dermatol Venereol Leprol 2005;71:9-13.   Back to cited text no. 2
[PUBMED]  Medknow Journal  
3.Parsricha JS, Thamzam J, Khan UK. Intermittent high-dose dexamethasone-cyclophosphamide therapy for pemphigus. Br J Dermatol 1988;119:73-7.  Back to cited text no. 3
    
4.Pai BS, Srinivas CR, Sabithal L, Shenoi SD, Balachandran CN. Efficacy of Dexamethasone pulse therapy in progressive systemic scelorosis. Int J Dermatol 1995;34:726-28.  Back to cited text no. 4
    



 
 
    Tables

  [Table 1], [Table 2], [Table 3]


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[Pubmed] | [DOI]



 

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