INTRODUCTION :
Collagen vascular diseases like Systemic Lupus Erythematosus (SLE) and
Systemic Sclerosis (SSc) are always a challenge to the treating dermatologists. Though
complete cure remains an enigma, therapeutic advances have aimed at inducing quick
remission and improving the quality of life of the patients.
Pulse therapy is administration of single or multiple daily infusions of
suprapharmacological doses of drugs to achieve a desired therapeutic effect. It is
otherwise called as the “big shot". The history of pulse therapy began in 1969 when
Kountz and Cohn introduced methylprednislone pulse therapy to prevent renal graft
rejection.
The monumental success of Dexamethasone Cyclophosphamide pulse therapy
(DCP) in autoimmune bullous diseases (eg.Pemphigus) reported by Pasrischa et al., has
prompted dermatologists to use DCP in collagen vascular diseases.
Collagen vascular disorders are a group of diseases characterized by
generalised alteration in connective tissue and have certain common features like
autoimmunity, disordered cell mediated immunity, vascular abnormalities, arthralgia
and skin disease.
The diseases grouped under this category include lupus erythematosus,
systemic sclerosis, localised and generalised morphoea, dermatomyositis, rheumatoid
arthritis and Sjogren’s syndrome.
AIMS & OBJECTIVES :
To study the efficacy of Dexamethasone –Cyclophosphamide pulse therapy in
collagen vascular disorders.
1. To evaluate the degree of clinical and serological remission in Systemic Lupus
Erythematosus and Systemic Sclerosis.
2. To evaluate the duration required to induce clinical remission.
3. To evaluate side effects of pulse therapy.
MATERIALS AND METHODS :
TYPE OF STUDY :
This is a type of open labelled non randomised prospective therapeutic study.
Here the patients act as their own control.
SAMPLE SIZE :
A sample size of 36 patients was selected. Out of these, 20 patients had
systemic lupus erythematosus and the other 16 had Systemic Sclerosis.
STUDY PLACE :
This study was conducted at the Department of Dermatology, Stanley Medical
College in association with other departments for evaluation of systemic involvement.
STUDY PERIOD :
The study was conducted over a period of 2 years from November 2009 to
October 2011.
PATIENT SELECTION CRITERIA :
INCLUSION CRITERIA :
1. Systemic lupus erythematosus and systemic sclerosis patients diagnosed as per
ARA criteria.
1. Age: 13 to 60 years.
2. Severe skin lesions not responding to high dose daily steroids
3. Presence of systemic involvement.
EXCLUSION CRITERIA :
1. Pregnancy.
2. Lactating mothers.
3. Children <12 years.
4. Ischemic heart disease.
5. Uncontrolled hypertension.
6. Active infections except Minor upper respiratory tract infections, acute
gastroenteritis and skin infections.
CONCLUSION :
The following are theconclusions derived from this study :
• Dexamethasone cyclophosphamide pulse therapy is an effective therapeutic
option for treatment of SLE and Scleroderma.
• Rapid induction of and prolonged remission is achieved with pulse therapy.
• Pulse therapy is safer than oral steroids with respect to decreased HPA axis
suppression and decreased osteopenia.
• Patient compliance is much better with pulse therapy because of the rapid
induction of remission and relative absence of side effects.
• Though the duration of phase I of pulse therapy is arbitrary depending upon
the degree of remission achieved in individual patients, the mean duration
required would be 9 pulses in SLE while in Scleroderma, a longer phase I of up
to 12-15 months is required.
• Unlike in bullous disorders where clinical signs of activity are clear, it is not so
in collagen vascular disease. Hence an extended phase I and phase II should be
given to attain remission.
• The earlier the initiation of pulse therapy in scleroderma the better is the
response.
• Precipitation of scleroderma renal crisis was not observed in any of our
patients and hence high dose corticosteroid pulse is safe in scleroderma
provided blood pressure is maintained within normal limits by anti
hypertensives.
• Scope for future improvements in pulse therapy for collagen vascular disease
is ample with respect to clear guidelines on the duration of therapy and the
necessity of daily cyclophosphamide
