Dexamethasone Pulse Therapy Research Articles

Pulse dexamethasone therapy versus pulse methylprednisolone therapy for treatment of Graves′s ophthalmopathy

Pulse methylprednisolone therapy is the recommended therapy for moderate to severe and active ophthalmopathy, but high dose pulse methylprednisolone therapy is marred by the chances of fulminant hepatic failure and the high cost of therapy. Dexamethasone pulse therapy can be considered as an alternative to pulse methylprednisolone therapy. A prospective randomized control trial was carried out in 21 patients comparing pulse dexamethasone therapy versus pulse methyprednisolone therapy in Graves's ophthalmopathy. This study proved that pulse dexamethasone therapy is a cheaper and equally effective therapy for Graves's ophthalmopathy and the cost of therapy is reduced to at least 1/8th s. Furthermore, dexa had a better effect on reduction of exophthalmos. The dreaded complication of fulminant hepatic failure, associated with high dose of methylprednisolone, is not seen with dexa therapy.

Immunoglobulin G4–related sclerosing disease of the paranasal sinus

Immunoglobulin G4 (IgG4)-related sclerosing disease is a systemic disease characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs. We present a case of a 69-year-old man with maxillary sinus IgG4 sclerosing disease, with orbital invasion treated with rituximab and dexamethasone pulse therapy. Surgery was used as well to debulk the disease and to obtain tissue for diagnosis. A PubMed search using the key phrase "IgG4-related Sclerosing Disease" was performed. There were 304 different articles regarding the disease for a multitude of different organ sites. Of the 304 articles, there were 3 articles that reported this disease in the paranasal sinuses. IgG4-related sclerosing disease is a rare entity in the head and neck. There are documented reports of steroid therapy for this disease, but the patient presented here demonstrated clinical progression of disease with steroids alone. The use of combination therapy of surgery, dexamethasone, and rituximab provided clinical improvement and stable disease determined by radiographic means.

Skleromyxödem

Scleromyxedema is a rare connective tissue disease that may affect numerous internal organs in addition to the skin. The disease is almost exclusively associated with monoclonal gammopathy. This retrospective study summarizes the clinical characteristics of four patients with scleromyxedema. In all of the patients a systematic serological and apparative check-up was performed. The mean age of the four patients (three women and one man) was 51 years. In all cases, monoclonal gammopathy (3 cases of IgG lambda and 1 case of IgG kappa) was involved. In one patient, skin lesions were restricted to the upper part of the body and three patients had generalized disease. The internal organs of all patients were affected with fibrosis of the lungs, myositis and arthritis, peripheral polyneuropathy and hypomotility of the esophagus. The most effective forms of treatment in this patient collective were dexamethasone-pulse therapy, intravenous immunoglobulins and bortezomib. All patients had recurrences after finishing therapy. The mean observation period after the initial diagnosis of scleromyxedena was 6.25 years (range 2-11 years). Scleromyxedema is a rare multisystemic disease. The heterogeneous affection of internal organs necessitates a comprehensive check-up. The response to recently published treatment strategies is low and recurrences after finishing therapy are frequent.

Pemphigus foliaceus masquerading as IgA pemphigus and responding to dapsone

A 14-year-old male presented with seven years history of recurrent episodes of fluid filled, itchy and eroded lesions over the body not responding to oral corticosteroids and azathioprine. Dermatological examination revealed crusted plaques and erosions in a seborrheic distribution. Histopathology of skin lesions and direct immunofluorescence were characteristic of pemphigus foliaceus. He was treated with dexamethasone pulse therapy with inadequate response. However, relapsing skin lesions revealed a circinate arrangement with a predilection to trunk and flexures. In view of clinical features suggestive of IgA pemphigus, he was started on dapsone, to which he responded dramatically in four weeks. However, repeat biopsy continued to reveal features of pemphigus foliaceus and ELISA for anti-desmoglein 1 antibodies was positive.

Childhood pemphigus vulgaris successfully treated with rituximab

Pemphigus is a potentially fatal autoimmune epidermal bullous disorder. Rituximab is a novel therapy for the treatment of refractory pemphigus. However, there is limited clinical data on safety and efficacy of rituximab in pediatric age group. Herein, we report an 11-year-old boy of childhood pemphigus vulgaris who failed to respond to dexamethasone pulse therapy and was subsequently treated with rituximab and achieved complete remission.

Another promising treatment option for neuroblastoma-associated opsoclonus–myoclonus syndrome by oral high-dose dexamethasone pulse: Lymphocyte markers as disease activity

A one-year-old boy with neuroblastoma (NBoma)-associated opsoclonus–myoclonus syndrome (OMS) was treated by oral high-dose dexamethasone (DEX) pulses (20mg/m2/day of DEX for three consecutive days) every 28days for 6months after resection of the tumor. All OMS symptoms improved after the first course of DEX pulse therapy and disappeared after the last course. No adverse effects were observed. Minor deterioration of his developmental quotient was noted 33months after the onset of the disease. NBoma remission has been maintained since treatment. Before DEX pulse therapy, frequency of T lymphocyte, in particular CD4-positive cell decreased markedly resulted in low CD4/8 ratio in the peripheral blood (PB). The frequency of B lymphocyte increased, especially in cerebrospinal fluid. These aberrant values in PB were reversed by DEX pulse therapy and correlated well with the neurological symptoms. A prospective study that assesses the efficacy of this promising and inexpensive treatment for OMS is warranted.

Pyoderma gangraenosum as rare complication of incisional hernia repair in a patient with Werlhof’s disease

On the 3rdday following surgery to repair an incisional hernia, a 67-year-old male patient with Werlhof's disease (idiopathic thrombocytopenic purpura) was diagnosed with a histologically confirmed pyoderma gangraenosum (PG), a rare complication of wound healing. Dexamethasone pulse therapy resulted in rapid remission of the skin lesions. Further improvement was slowed when the patient suffered multiple organ failure in the intensive care unit, delaying his transfer to rehabilitation for 8weeks. Postoperative PG is always a differential diagnostic possibility in patients with sterile, progressive, painful, ulcerative skin lesions at or near the surgical wound. Unlike most wound healing complications, which are treated by debridement or antibiotics, the treatment of choice for PG is high-dose steroid therapy.

Oral dexamethasone pulse therapy versus daily prednisolone in sub-acute onset myositis, a randomised clinical trial

To determine if high-dose pulsed dexamethasone is more effective and safer than daily high-dose prednisolone in treatment-naive adult patients with inflammatory myopathies (sporadic inclusion body myositis excluded) we performed a multicenter, double-blind randomised controlled clinical trial with 18 months follow-up. Sixty-two patients were randomised into 28-day cycles of oral high-dose dexamethasone or daily high-dose prednisolone. Primary outcome measures included (1) seven point composite score of six clinically relevant outcomes and (2) (time-to) remission and (time-to) relapse. No difference between both treatment groups on the composite score was found. Side-effects occurred significantly less frequently in the dexamethasone group. Median time to relapse was 60 (2.9) weeks in the prednisolone and 44 (4.7) weeks in the dexamethasone group (log-rank test p = 0.03). In conclusion, pulsed high-dose oral dexamethasone is not superior to daily prednisolone as first-line treatment of idiopathic inflammatory myopathies, but is a good alternative by causing substantially fewer side-effects.

Dexamethasone pulse therapy in patients of systemic sclerosis: Is it a viable proposition? A study from kashmir

Background:Systemic sclerosis is a multisystemic autoimmune disorder. Intravenous dexamethasone pulse therapy has been used since 1998.Aim:The aim wasto report the beneficial effects of dexamethasone pulse in patients of systemic sclerosis vis-à-vis the side effects.Materials and Methods:Forty-seven patients of systemic sclerosis were included. After looking at the history and physical examination, the patients were submitted to various relevant investigations. Clinical scoring of the patient was done at baseline and 6-month interval according to Furst's organ indices score.Results:A total of47 patients of systemic sclerosis were included (45 females, 2 males). In majority, acrosclerosis was seen. Severe sclerosis and contractures were seen in two patients. Moderate proteinuria, restrictive lung disease, dysphagia, and valvular heart involvement were seen.A total of13 patients on dexamethasone pulse therapy developed tuberculosis. Improvement in skin scoring and decreased severity of Raynaud's phenomenon was seen. No improvement in dysphagia, severe vascular symptoms, or restrictive lung disease was seen.Conclusion:Thus, beneficial effects of dexamethasone pulse therapy seem to be merely cosmetic.

Assessment of bone mineral density by dual x-ray absorptiometry in dermatological patients treated by corticosteroids

Background:Corticosteroids are mainstay of dermatological therapy and they are also a well known cause of osteoporosis. The objective of the present study was to find out the influence of the systemic intake of corticosteroids, either by the oral route or by IV pulse administration, on bone mineral density in dermatological patients using dual X-ray absorptiometry (DXA).Materials and Methods:This study was carried on 100 patients and 55 controls. The first group of patients included 55 patients undergoing long-term oral corticosteroid therapy daily and the second group included 45 patients who received IV dexamethasone pulse therapy. DXA was measured once for both the controls and patients in group 1. DXA was measured twice for patients in group 2, before starting pulse therapy (baseline DXA) and six months after regular treatment with pulse therapy (follow-up DXA).Results:The results show that significant reduction in BMD occurs in both groups, however, oral corticosteroids produce significantly more reduction in BMD in the lumbar spine. BMD was not found to be affected by the cumulative doses of corticosteroids, the duration of daily oral corticosteroid intake, or the number of IV dexamethasone pulses.Conclusion:Corticosteroid treatment causes significant BMD loss in patients treated by either route. Prophylactic treatment against osteoporosis is mandatory in patients receiving either form of corticosteroids.

Side Effects of Oral Dexamethasone Pulse Therapy for Idiopathic Sudden Sensorineural Hearing Loss

To the Editor: In reaction to clinical practice article by Steven D. Rauch (1) in the August 21 issue of the New England Journal on sudden deafness and the following correspondence in Otology Neurotology on intratympanic treatment of this disease, we would like to comment on the use of a dexamethasone 3-day pulse therapy as studied by Westerlaken et al. (2) in comparison to a 7-day prednisolone taper in a double-blind, randomized, controlled trial as mentioned on page 836 by Rauch (1). Rauch comments rightly on the possible severe side effects of corticosteroid treatment, including elevated blood sugar levels, elevated blood pressure, mood change, sleep disturbances, flushing, gastritis, and weight gain. As we implemented the 3-day dexamethasone pulse therapy treatment in our hospital after the study by Westerlaken, a former colleague of ours, we would like to stress the importance of utter care in following the exclusion criteria, like diabetes mellitus, cardiac history, hypertension, pregnancy, stomach ulcer, renal failure, use of oral anticoagulant or corticosteroids, and Cushing syndrome. We have experienced patients with raising blood pressures, mood disturbances, sleep disturbances, and flushes and observed that this seems to be more often the case in the 3-day dexamethasone treatment than in the prednisolone taper therapy. Also, 1 patient treated with the 3-day dexamethasone course developed a myocardial infarction while his previously existing diabetes mellitus derailed, without regaining the lost hearing. Obviously, that is too high a price to be paid, especially for a disease from which 45 to 65% show spontaneous improvement (3,4). Perhaps because of his preexisting (well-regulated) diabetes mellitus, our patient should not have started the treatment in the first place; however, only badly regulated insulin-dependent diabetes mellitus is one of our exclusion criteria for this type of treatment. We would like to urge physicians to take the severe risks of oral dexamethasone pulse therapy and prednisolone taper therapy seriously and refrain from prescribing this treatment to the group of patients known with exclusion criteria as previously mentioned. A hopeful but expensive alternative could be the intratympanic corticosteroid treatments that are currently being studied and discussed (5,6). However, also doing nothing might be an interesting type of treatment, although difficult for physicians; as the Cochrane Review of Wei pointed out, the efficacy of corticosteroid therapy for sudden sensorineural hearing loss remains unproven (3). Rolien H. Free, M.D., Ph.D.* Noor D. Smale, M.D.* Emile de Kleine, Ph.D.† Bernard F. A. M. van der Laan, M.D., Ph.D.* *Department of Otorhinolaryngology-Head and Neck Surgery and †University Audiological Center Groningen University Hospital Groningen The Netherlands

T.O.5 Oral dexamethasone pulse therapy versus daily prednisolone in subacute inflammatory myopathies: A randomised clinical trial

Background. Long-term high-dose prednisolone is standard treatment for subacute inflammatory myopathies. Pulsed dexamethasone may have fewer side effects than daily prednisolone. We compared effectiveness and safety of prednisolone and pulsed dexamethasone in newly diagnosed adult patients with dermatomyositis or non-specific myositis. Methods. Patients were assigned to 28-day cycles of oral high-dose dexamethasone or daily high-dose prednisolone in a long-term, prospective, multicentre, double-blind randomised controlled clinical trial.

Intravenous dexamethasone vs methylprednisolone pulse therapy in the treatment of acute endothelial graft rejection

To study the outcome of therapy for acute endothelial graft rejection with an intravenous (i.v.) pulse of dexamethasone vs methylprednisolone, in addition to topical corticosteroids. Records of 98 eyes of 99 patients treated for endothelial graft rejection with a single i.v. pulse of dexamethasone or methylprednisolone in addition to topical steroids, between January 1999 and June 2004, were retrospectively reviewed. Baseline characteristics such as surgery-rejection interval, time taken to consult after onset of symptoms, history of failed grafts, extent of stromal vascularization, best-corrected visual acuity (BCVA) and corneal thickness at the time of presentation were noted. Main outcome measures following treatment for rejection included improvement in BCVA, change in corneal thickness, and reversal of graft rejection. Fifty-one patients were treated with i.v. methylprednisolone and 47 with i.v. dexamethasone, in addition to topical steroids. Both groups were found to be comparable with respect to baseline parameters, that is, time taken to present, history of failed grafts, extent of stromal vascularization, BCVA, and graft thickness. Graft rejection could be successfully reversed in 72.3% cases in the dexamethasone group and 49% in the methylprednisolone group (P=0.018). A significant improvement in visual acuity was recorded following treatment in both groups, with a better outcome in the dexamethasone group (P=0.012). Post-treatment pachymetry values were lower than pretreatment values in both groups, with significantly lower final pachymetry in the dexamethasone group (P=0.017). No adverse effects were observed. I.v. pulse therapy with dexamethasone may be used as an effective alternative to methylprednisolone in reversing acute endothelial graft rejection.

Increased incidence of tuberculosis in patients of systemic sclerosis on dexamethasone pulse therapy: A short communication from Kashmir

Background:Systemic sclerosis is a multi-systemic autoimmune disorder affecting predominantly the skin, lungs, gut and kidneys.Purpose:To report the increased incidence of tuberculosis in patients of systemic sclerosis on dexamethasone pulse (DP) therapy.Methods:Forty-seven patients of systemic sclerosis were included in the study. After taking a complete history and doing a detailed physical examination, the patients were submitted to a battery of investigations including complete hemogram (CBC) with erythrocyte sedimentation rate (ESR(F)), Chest X-ray CXR (PA view) Mantoux test and urine analysis. CBC, ESR and urine examination was done monthly and CXR were repeated six-monthly.Findings:Seven patients on DP therapy developed genitourinary tuberculosis. Four had pulmonary tuberculosis. One patient developed tubercular lymphadenitis, one patient succumbed to miliary tuberculosis.Conclusion:There is an increased incidence of tuberculosis amongst patients of systemic sclerosis on DP therapy.Limitation of the Study:There was no control group of systemic sclerosis patients not on DP therapy to rule out the confounding effect of the disease per se predisposing to tuberculosis as all our patients as a matter of routine were put on steroid pulse. Also, the increased incidence of tuberculosis was detected incidentally while on monthly follow-up.

Chronic relapsing opsoclonus-myoclonus syndrome: Combination of cyclophosphamide and dexamethasone pulses

Opsoclonus-myoclonus syndrome (OMS) is a rare and debilitating disorder of unknown etiology affecting children and adults. Outcome is unfavourable; approximately 80% of children with OMS suffer from mild to severe neurological handicaps, mainly cognitive impairment. A standard therapy does not exist. Due to the possible immune-mediated mechanisms, treatment with steroids, ACTH, plasmapheresis and immunoglobulins can be successful. However, some children become steroid dependent and symptoms may reoccur after treatment has been finished. We present two girls with OMS, who had a prolonged clinical course lasting 4 and 9 years with many relapses. Both children developed symptoms around the age of two years. Diagnostic work-up to exclude neuroblastoma was negative. Several treatment modalities including oral steroids, dexamethasone pulses, immunoglobulin and cyclosporine were used without lasting success. In addition, cognitive impairment developed in both children. In order to prevent further clinical and mental deterioration, 6 pulses of cyclophosphamide in combination with dexamethasone pulses every 4 weeks were administered. Both children showed significant improvement of OMS symptoms. One girl is still symptom free 18 months after treatment, mild ataxia developed in the other after 12 months. Both children are mentally handicapped and in special need schools. We conclude that combination of cyclophosphamide pulses and dexamethasone pulse therapy is a therapeutic option even after a long clinical course to improve symptoms of OMS.

The use of oral cyclophosphamide with dexamethasone pulse therapy in the treatment of pemphigus vulgaris

Background: Pemphigus vulgaris (PV) is a severe acquired autoimmune disease. During the past 15 years we used dexamethasone pulse therapy (DP) and oral cyclophosphamide for treatment of our patients with PV. Objectives: To evaluate the efficacy and side effects of this treatment. Method: The study was a retrospective study. Medical records of 50 known cases of PV, who were treated with dexamethasone pulse therapy and oral cyclophosphamide were evaluated and the patients were re‐examined and questioned about their current situations. Results: Thirty‐five females and 15 males were treated, out of which 38 finished the study. At the end of study, 21 patients were in remission, 10 patients were in the healing stage, five patients were partially healed and two patients died. The side effects of this method, especially the adverse effects of cyclophosphamide, were less than previously reported. Conclusion: Dexamethasone pulse therapy and oral cyclophosphamide is an effective regimen with few side effects in the treatment of mild to moderate PV.

Dexamethasone Pulse Therapy for Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis

Mortality in Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) is high. Apart from intensive supportive therapy, no generally accepted specific treatment regimen exists. The role of corticosteroids in SJS/TEN is controversial. It is possible that high-dose pulse therapy with corticosteroids might be an improvement on long-term lower dose therapy, by combining higher efficacy with a diminished risk both of infection and of delayed wound healing. The aim of this study was to evaluate the efficacy of dexamethasone pulse therapy with respect to mortality and healing time of patients with SJS/TEN. A small, uncontrolled series of consecutive inpatients with SJS/TEN was treated with dexamethasone pulse therapy. The efficacy of this treatment was assessed retrospectively using SCORTEN. Twelve patients were included over a period of 10 years. One patient died, while SCORTEN predicted a fatal outcome of 4 patients. Stabilization was reached after 2.3 days on average, total re-epithelialization after 13.9 days. The results of this study bear no statistical relevance due to the small number of patients. In conclusion, short-term dexamethasone pulse therapy, given at an early stage of the disease, may contribute to a reduced mortality rate in SJS/TEN without increasing healing time. A larger controlled trial is warranted to investigate further the use of dexamethasone pulse therapy in SJS/TEN.

Oral Pulsed Dexamethasone Therapy in Early Rheumatoid Arthritis

Pulse therapy with high-dose glucocorticoids (GCs) is widely used as "bridging therapy" for the treatment of patients with active rheumatoid arthritis (RA). Oral pulsed dexamethasone therapy has never been used for this purpose. We determined the clinical efficacy of oral pulsed dexamethasone treatment in patients with early active RA, concomitantly starting with disease-modifying anti-rheumatic drugs (DMARDs). Fourteen early RA patients, glucocorticoid-naive and with active disease for less than 1 year were included. Ten patients were treated with oral pulsed dexamethasone therapy for 4 days in a row. Of this group, four patients received 10 mg dexamethasone/day, three patients 20 mg/day, and three patients 40 mg/day. As controls, four patients were treated with intramuscular methylprednisolone injections. Disease activity (ascertained by disease activity score [DAS]) and biochemical variables were measured at base line, and biweekly thereafter for up to 4 weeks, and monthly thereafter for up to 3 months. A decrease in disease activity, similar in all subgroups, was observed. Nine of 10 patients responded favorably (decrease in DAS of >1.2) 4 weeks after the start of the study. This response was sustained in the months thereafter. One patient did not respond at all, and disease progression during treatment was observed in one patient. No side effects were reported. Only once was a decrease in cortisol level observed; this was at 2 weeks after the start of the study (0.03 micromol/L, reference value 0.18-0.70 micromol/L). Oral pulsed dexamethasone therapy seems to be effective and safe as bridging therapy in early rheumatoid arthritis. The results of the present study justify a long-term controlled trial to compare oral pulsed dexamethasone treatment (10 mg dexamethasone, once weekly for 4 weeks) with the standard GC regimes in the near future.

Randomized Controlled Trial of Adjuvant Oral Dexamethasone Pulse Therapy in Pemphigus Vulgaris

To determine the therapeutic effect of adjuvant dexamethasone pulse therapy when given in addition to conventional treatment of pemphigus vulgaris. A randomized, placebo-controlled trial. International European, multicenter outpatient and inpatient study. Of the 20 enrolled patients, 11 were randomized to the dexamethasone pulse (DP) group and 9 to the placebo pulse (PP) group. Oral dexamethasone in 300-mg pulses or PPs 3 days per month. During the intervention, the DP and PP groups received conventional treatment with prednisolone, 80 mg/d, which was tapered across 19 weeks, and azathioprine sodium, 3 mg/kg per day, until the end of the study. Monthly pulses were continued until prednisolone treatment was tapered to 0 mg. Number of patients in remission, time to and duration of remission, cumulative prednisolone dose, and occurrence of adverse events during 1 year of follow-up. Eight of the 11 DP-treated patients and all 9 PP-treated patients achieved remission. Mean time to remission was 173 days with DP and 176 days with PP. The mean duration of remission within the first year was 151 days for DP and 141 days for PP. Mean cumulative prednisolone dose was 5300 mg for DP and 4882 mg for PP. Weight gain (>5% of baseline) occurred in 8 DP-treated patients compared with 1 PP-treated patient (P<.01). We found no statistically significant difference (P>.05) of an adjuvant effect of DP on remission of pemphigus vulgaris. In patients with new pemphigus vulgaris disease activity, there was no benefit of oral DP therapy given in addition to conventional treatment. clinicaltrials.gov Identifier: NCT00127764.

Anti‐laminin 5 mucous membrane pemphigoid

A 70-year-old patient presented erosions on the oral mucosa and skin, predominantly in perianal and inguinal areas. Histopathologically, subepidermal blistering was observed. By direct immunofluorescence microscopy, linear deposits of IgG and C(3) were seen at the dermal-epidermal junction. By indirect immunofluorescence microscopy, binding of IgG autoantibodies to the dermal side of NaCl-split skin was detected. These autoantibodies were shown to target both the non-processed and processed alpha(3)- as well as the beta(3)-subunit of laminin 5 by immunoblot analysis using extracellular matrix of cultured human keratinocytes. Based on these results, the diagnosis of an anti-laminin 5 mucous membrane pemphigoid was established. Oral treatment with dapsone, combined with intravenous dexamethasone pulse therapy, led to rapid improvement.