Maintenance Dose Of Corticosteroids Research Articles

Real-World Effectiveness of Dupilumab for Patients with Severe Asthma: A Retrospective Study.

BackgroundTreatment with dupilumab, an anti-interleukin (IL)-4 receptor α monoclonal antibody that blocks both the IL-4 and IL-13 pathways, has demonstrated efficacy for the treatment of severe asthma (SA) with type 2 inflammation. However, few studies have focused on the efficacy of this biologic for the treatment of SA in a real-world setting.MethodsFrom April 2019 to December 2021, 26 Japanese patients with SA received dupilumab at Jikei University Hospital. We retrospectively evaluated the number of moderate-to-severe exacerbations, pulmonary function, maintenance dose of corticosteroids, biomarkers, and adverse events.ResultsDuring a mean follow-up period of 12.6 months, 10 patients received dupilumab as the first biologic, and 16 switched to dupilumab from other biologics. Dupilumab treatment significantly reduced the number of annual exacerbations from 3.4 ± 4.1 to 1.6 ± 2.7 (/person-year, p < 0.01) at the last follow-up regardless of previous biologic use. The Asthma Control Test score significantly improved in all patients by six months after administration but tended to worsen by 24 months in patients with previous biologic use. On the other hand, blood eosinophil counts (BECs) transiently increased and peaked three to six months after administration. The peak timing can be affected by previous biologic use. Adverse events included wheezing immediately after injection, hypereosinophilia, mild conjunctivitis, and relapse of chronic eosinophilic pneumonia in the patient switched from benralizumab.ConclusionDupilumab treatment was useful for patients with SA in a real-world setting. However, the BEC should be monitored carefully, especially in patients who previously received anti-IL-5/IL-5 receptor antibody.

Anti-IL-5 Agents for the Treatment of Idiopathic Chronic Eosinophilic Pneumonia: A Case Series.

PurposeIdiopathic chronic eosinophilic pneumonia (ICEP) is a rare, chronic respiratory disease. Corticosteroid therapy is effective for ICEP, but relapse is frequent after its tapering, which leads to chronic use and corticosteroid-related adverse effects. Currently, biological agents targeting interleukin 5 (IL-5) are considered alternatives for treating ICEP patients with frequent relapse, but the detailed effects are not fully understood.Patients and MethodsThe clinical characteristics of 30 patients with ICEP, especially 12 patients with ICEP who experienced relapse after corticosteroid dose tapering, were evaluated retrospectively. In addition, 4 ICEP patients with frequent relapse treated by IL-5-targeted biological agents were reviewed.ResultsOf the 30 patients diagnosed with ICEP, 12 patients (40.0%) recurred after corticosteroid dose tapering, and 9 (30.0%) were treated with maintenance doses of corticosteroid. Of ICEP patients who experienced recurrence, 6 (50.0%) had frequent relapses (2 or more times). All 4 patients treated with anti-IL-5 agents had their corticosteroid dose reduced without any relapses; in 3 patients, corticosteroids were withdrawn.ConclusionAnti-IL-5 agents might be alternatives for treating ICEP patients with frequent relapses.

Early corticosteroid dose tapering in patients with acute exacerbation of idiopathic pulmonary fibrosis

BackgroundAlthough corticosteroid therapy with dose tapering is the most commonly used treatment for acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF), there is no consensus on the tapering regimen. This study aimed to investigate the association between early corticosteroid dose tapering and in-hospital mortality in patients with AE-IPF.MethodsIn this retrospective cohort study, we analyzed the data of a cohort from eight Japanese tertiary care hospitals and routinely collected administrative data from a cohort from 185 Japanese hospitals. Patients with AE-IPF were classified into the early and non-early tapering groups depending on whether the maintenance dose of corticosteroids was reduced within two weeks of admission. Propensity score analysis with inverse probability weighting (IPW) was performed to estimate the effect of early corticosteroid dose tapering.ResultsThe multi-center cohort included 153 eligible patients, of whom 47 (31%) died, whereas the administrative cohort included 229 patients, of whom 51 (22%) died. Patients with early tapering tended to have a better prognosis than those without it (unadjusted hazard ratio [95% confidence interval] 0.41 [0.22–0.76] and 0.65 [0.36–1.18] in the multi-center and administrative cohorts, respectively). After IPW, the early tapering group had a better prognosis than the non-early tapering group (IPW-adjusted hazard ratio [95% confidence interval] 0.37 [0.14–0.99] and 0.27 [0.094–0.83] in the multi-center and administrative cohorts, respectively).ConclusionEarly corticosteroid dose tapering was associated with a favorable prognosis in patients with AE-IPF. Further studies are warranted to confirm the effects of early corticosteroid dose tapering in patients with AE-IPF.

Combining maintenance therapy with hydroxychloroquine increases LLDAS achievement rates in individuals with stable systemic lupus erythematosus.

Hydroxychloroquine (HCQ) has been positioned as an anchor drug for systemic lupus erythematosus (SLE). There is evidence supporting the benefits of HCQ; however, the effect of additional HCQ in maintenance therapy remains unclear. Thirty patients with SLE who visited Juntendo University Hospital were receiving maintenance therapy before HCQ treatment and were able to complete more than 104 weeks of HCQ treatment were analyzed. Anti-DNA antibody titers, IgG and CH50 levels, the maintenance dose of corticosteroids, the SLE disease activity index (SLEDAI), and the achievement of the Lupus Low Disease Activity State (LLDAS) were evaluated at baseline and at 12, 24, 52, and 104 weeks after HCQ initiation. We observed improvements in the anti-DNA antibody titers, IgG and CH50 levels, maintenance dose of corticosteroids, and SLEDAI at week 104 relative to baseline. Moreover, the LLDAS achievement rate increased over time from 10% at baseline to 43% and 80% at week 52 and week 104, respectively. Two years of continuous HCQ treatment led to improvements in SLE disease activity and corticosteroid dose and an increase in LLDAS achievement, thereby demonstrating the significance of the maintenance dose of HCQ for the management of SLE.

The feasible maintenance dose of corticosteroid in Takayasu arteritis in the era of biologic therapy

Objective: Although biologic agents are used in Takayasu arteritis (TAK), corticosteroids are still the mainstay of treatment. This study aimed to investigate the feasible maintenance dose of prednisolone (PSL) in the biologic therapy era. Method: We enrolled 93 patients with TAK who satisfied the criteria of the American College of Rheumatology and visited our department from 2008 to 2018. The clinical characteristics and PSL dose of the patients were retrospectively evaluated. Results: The mean ± sd maintenance dose of PSL was 5.0 ± 3.0 mg/day. In patients having TAK for > 20 years, PSL discontinuation and drug-free status were achieved in 27.2% and 18%, respectively. Although tapering the PSL dose to 10 mg/day was achieved within 12 months, tapering to 5 mg/day required 10 years. Relapse significantly interfered with the PSL dose reduction. The clinical characteristics of patients with relapse included a lower rate of combination therapy using immunosuppressants. Moreover, biologics were used in > 60% of patients with relapse. Tapering of PSL was significantly possible in patients receiving biologics and additional relapse was observed in 6.3% and 50% of patients with and without biologics, respectively. Such PSL-sparing effect enabled the reduction of the median PSL dose from 10 to 5 mg/day. Steroid discontinuation was achieved in some patients. Conclusions: The use of biologics significantly reduced the PSL dose in relapsed patients. A PSL dose of ≤ 5 mg/day is a feasible target for TAK, especially when biologic agents are used. Nevertheless, corticosteroid discontinuation may also be the target in some patients.

Experience in managing patients with Churg-Strauss syndrome.

Our goal was to analyze the possibilities of impro­ving the diagnostics of CSS and to improve the effectiveness of treatment according to the existing literature and our own experience of long-term care for patients with eosinophilic granulomatosis with polyangiitis or Churg-Strauss syndrome (CSS). The medical histories of three female patients aged 26 to 46-years and a 20-year-old male patient were considered. The duration of the disease before the established diagnosis was 5-17 years. Anamnesis and medical documents analysis showed a typical CSS debut in the form of allergic rhinitis, nasal polyps, which were recurrent after polypectomy, and respiratory disorders, which were regarded as bronchitis or bronchial asthma – corresponding to the first phase, also called the prodromal or allergic stage of CSS. The prodromal period lasts up to 10 years or more and is characterized by various allergic manifestations, more often – pollinosis or bronchial asthma, that is difficult to control. But CSS can be suspected because of low effectiveness of the therapy with inhaled steroids, lack of effect of antibiotics and eosinophilia more than 10% that occurs periodically. Even in the third stage of CSS in systemic manifestations of vasculitis and severe secondary lesions of organs and tissues with functional impairment, constant intake of maintenance doses of corticosteroids and cytostatics allows to achieve stabilization of the process in patients with CSS.

An Enigma of Female Psychosis: Case Report and Literature Review of Therapeutic Strategies in Paraneoplastic Encephalitis Associated with Ovarian Teratoma

Introduction: Paraneoplastic Encephalitis (PNE) is the neuropathological disorder of central nervous system, associated with a remote tumor, which presents with diverse of neuropsychological symptoms. There is still a deficiency of an aptly formulated management protocol. Case report: We report the first case of anti- NMDA-R PNE secondary to ovarian teratoma from Pakistan, in a 17 year old girl that presented with acute psychosis. She underwent immediate tumor excision with plasmapheresis followed by a combination of corticosteroids and IV immunoglobulin therapy. The patient relapsed after a month despite the treatment presenting with mild psychosis, memory loss and cognitive impairment. Provided along with the case report is an extensive literature review (2010-2017) on previously reported cases of PNE with ovarian teratoma to improve understanding of current management of this form of encephalitis. Conclusion: Paraneoplastic encephalitis is a critical yet reversible illness. Our comprehensive review and experience can be summarized in the following guidelines signifying the current clinical practices regarding PNE with ovarian teratoma. (I) PNE should be suspected in female patients presenting with an acute history of neuropsychiatric symptoms. (II) Tumor resection should be performed earliest to improve patient outcomes.(III) Administration of plasma exchange therapy in immediate postoperative period is recommended. (IV) Pulse therapy preceding intravenous immunoglobin therapy improves patient prognosis. (V) Patients should be discharged on maintenance dose of corticosteroids. (VI) Long term follow up is recommended to monitor for relapses.

Acquired hemophilia in the patient suffering from rheumatoid arthritis

Acquired hemophilia is a severe bleeding diathesis caused by autoantibodies against a coagulation factor VIII (FVIII inhibitor). Massive bleeding diathesis, often life threatening are observed in almost 90% of patients. In 50-60% of cases, inhibitor emerges spontaneously. However, there are some conditions like pregnancy, puerperium, autoimmune disorders or cancers that seem to induce acquired hemophilia. We report a case of a 49-year-old woman suffering from rheumatoid arthritis (RA) for several years, who was diagnosed with acquired hemophilia in September 2011. The patient had been treated by steroids and leflunomide during the last few months. At the time of diagnosis, diffuse bruising of the forearms and the trunk was observed. The patient was treated with recombinant activated factor VII, and the first-line immunosuppressive therapy was introduced (cyclophosphamide and prednisone). We observed the elimination of symptoms and the disappearance of diathesis. Significant reduction of the titer of inhibitor was achieved, but only partial remission was obtained. It lasted until the beginning of December 2011, when the titer of the inhibitor increased again and massive bleeding to the left lower limb occurred. It was necessary to administer recombinant factor VIIa together with the second-line immunosuppressive therapy based on the Budapest protocol. The rapid reduction of the diathesis and improvement of the patient's general condition was achieved as previously. However, still there was no complete remission. After 2 weeks of treatment, the titer of inhibitor diminished, and factor VIII activity increased slightly. Because of RA, the patient was treated with methylprednisolone in maintenance doses during the next few weeks. Unfortunately, after over a month, the increase of inhibitor titer and the decrease of FVIII level were observed again. Some bruises appeared. It was necessary to increase doses of corticosteroids to therapeutic levels and add cyclophosphamide in low doses to prevent the appearance of more hemorrhagic diathesis. Partial remission was achieved a second time at the end of April 2012. The patient was given methylprednisolone with chloroquine as a maintenance treatment and the control of RA. The titer of the inhibitor increased again in June 2012, but there were no signs of diathesis. In August 2012, some bruises were detected, and we decided to add cyclophosphamide again instead of escalating the doses of methylprednisolone to prevent the occurrence of side-effects of corticosteroids. Cyclophosphamide was given with intervals only depending on activated partial thromboplastin time. No further diathesis was observed in spite of the lack of remission. We were forced to withdrawn cyclophosphamide completely in October 2012 because of signs of hematuria. Fortunately, right nephrolithiasis and urinary tract infection were the cause of that condition. These symptoms vanished after standard supportive treatment. Maintenance doses of corticosteroids and chloroquine were continued as the main treatment. The patient's condition was good, but the titer of inhibitor increased over the value that had been detected at the time of diagnosis, and some bruises appeared again at the end of January 2013. The decision to use rituximab as the next-line therapy was made. This anti-CD20 monoclonal antibody is primarily used in the management of lymphomas. However, it has been successfully applied in the management of various autoimmune conditions. The usual treatment regime involves four separate intravenous infusions of 375 mg/m each, administered at weekly intervals. At the time of admission to the hospital in the second half of February 2013, the titer of inhibitor was dangerously high, almost three times more than the initial level. Fortunately, only a few bruises were observed, and no bypassing agents were needed. The patient was given the whole-planned therapy. Concomitant continuation of maintenance doses of corticosteroids was necessary to enforce the effect of eradication of inhibitor because of high levels of its titer during rituximab administration. It prevented the patient from massive diathesis that might occur. The laboratory tests were improving during the next subsequent weeks after the last dose of rituximab. Over a month later, a significant decrease of the titer of inhibitor and an increase of factor VIII activity was observed. Probably, the laboratory tests will be improving during the next few weeks. The patient is in outpatient care now. She is treated with maintenance doses of corticosteroids and chloroquine as the main treatment of RA. We will try to withdraw corticosteroids unless it is not feasible to achieve complete remission. We will have to introduce another kind of immunosuppressive agent in case of recurrence.

The Effect of Postoperative Corticosteroid Administration on Free Vascularized Fibular Grafting for Treating Osteonecrosis of the Femoral Head

Free vascularized fibular grafting (FVFG) has been reported to be an effective method of treating osteonecrosis of the femoral head (ONFH). This study evaluated whether postoperative maintenance doses of corticosteroids had an adverse effect on FVFG outcomes in patients with corticosteroid-induced ONFH. We retrospectively reviewed the records of 39 patients (67 hips) who had received maintenance doses of corticosteroids following FVFG. This group was matched to a group of patients who had not received corticosteroids treatment after operation. The mean follow-up duration was 5.4 years for the postoperative corticosteroid administration group (PCA group) and 5.0 years for the control group. At the latest follow-up, the average increase in Harris hip score was 11.1 ± 8.7 points for all hips in the PCA group and 12.6 ± 7.4 points for all hips in the control group (P > 0.05). In the PCA group, through radiographic evaluation, 49 hips were improved, 10 hips appeared unchanged, and 8 hips appeared worse. In the control group, 47 hips were improved, 13 hips appeared unchanged, and 7 hips appeared worse. The results suggested that postoperative maintenance doses of corticosteroids do not have an adverse effect on FVFG outcomes in patients with corticosteroid-induced ONFH.

Clinical Relevance of Autoantibodies in Patients with Autoimmune Bullous Dermatosis

The authors present their experience related to the diagnosis, treatment, and followup of 431 patients with bullous pemphigoid, 14 patients with juvenile bullous pemphigoid, and 273 patients with pemphigus. The detection of autoantibodies plays an outstanding role in the diagnosis and differential diagnosis. Paraneoplastic pemphigoid is suggested to be a distinct entity from the group of bullous pemphigoid in view of the linear C3 deposits along the basement membrane of the perilesional skin and the “ladder” configuration of autoantibodies demonstrated by western blot analysis. It is proposed that IgA pemphigoid should be differentiated from the linear IgA dermatoses. Immunosuppressive therapy is recommended in which the maintenance dose of corticosteroid is administered every second day, thereby reducing the side effects of the corticosteroids. Following the detection of IgA antibodies (IgA pemphigoid, linear IgA bullous dermatosis, and IgA pemphigus), diamino diphenyl sulfone (dapsone) therapy is preferred alone or in combination. The clinical relevance of autoantibodies in patients with autoimmune bullous dermatosis is stressed.

慢性関節リウマチにおける股関節破壊の検討

Hip radiographs were examined in forty patients with rheumatoid arthritis who had total hip arthroplasties. The patients' ages ranged from 37 to 83 years.Radiological appearances of hip involvement was calssified into five types as described by Lowe et al. Radiographic evaluation showed that protrusio acetabuli and loss of joint space were seen in 45% and 28%, respectively. Types of hip involvement correlated with the serum level of CRP and type of joint destruction (LES, MES, MUD), but not with disease duration, maintenance dose of corticosteroids and JOA score. 70% of the patients with protrusio acetabuli were MUD type and most with loss of joint space were LES or MES type.

Treatment of Children with Steroid Refractory Idiopathic Nephrotic Syndrome: The Kuwaiti Experience

Data on the treatment and outcome of Kuwaiti children with steroid refractory idiopathic glomerulonephritis (SRIGN), i.e. nephrotic syndrome who failed an eight-week course of prednisone, were collected retrospectively from the records of children attending the two renal centers of Kuwait between January 1, 1990 to December 31, 1996. During those seven years, a total of 34 Kuwaiti children were diagnosed to have SRIGN Histologically, 22 (65%) of those patients had minimal change, 5 (15%) focal segmental GN, 2 (6%) non-IgA mesangioproliferative GN and one membranous GN. Twenty-two patients had manifested frequent relapses, six were steroid-dependent and six were steroid-resistant. Treatment options were in the following order: (a) small maintenance-dose of corticosteroids (< 0.5 mg/kgalternate days); (b) cyclophosphamide and or chlorambucil for a single eight week-course or eight then 12 week courses (c) cyclosporin A for three months. The response to therapy was as follows: nine children were cured with low-dose corticosteroids; 17 with chlorambucil and/or cyclophosphamide; and five with cyclosporin A. At the end of study only three children failed such drug therapy, two of who had focal segmental glomeruloscierosis.

Hormonal modulation in systemic lupus erythematosus. Preliminary clinical and hormonal results with cyproterone acetate.

We prospectively studied the effects of hormonal modulation using the antigonadotropic drug, cyproterone acetate (CA), in 7 female patients who had moderately active systemic lupus erythematosus. CA was taken orally at a mean daily dose of 50 mg for 21-33 months by 6 patients (9 months by the seventh patient) without any side effects. The number of clinical lupus exacerbations during CA treatment was lower than that during the corresponding pretreatment period (15 of 170 patient-months versus 27 of 156 patient-months; P less than 0.05), despite a reduction in the daily maintenance dose of corticosteroids or antimalarial drugs. Mean plasma testosterone levels were low initially and remained unchanged (0.66 +/- 0.31 to 0.59 +/- 0.23 nmoles/liter), whereas plasma estradiol decreased markedly (from 0.6 +/- 0 38 to 0.11 +/- 0.03 nmoles/liter), resulting in a significant reduction in the estradiol:testosterone ratio (from 1.19 +/- 0.68 to 0.23 +/- 0.12) and in the plasma concentration of the sex hormone-binding protein. Thus, cyproterone acetate induced improvement in clinical lupus activity in parallel with the expected lower estradiol:testosterone balance.

HLA-A28 and Steroid-Induced Diabetes in Renal Transplant Patients

Between July 1971 and March 1977, steroid-induced diabetes (SD) developed postoperatively in 31 (10.8%) of 286 nondiabetic adult kidney transplant recipients who were observed for a total of 410 patient transplant years. There was a highly significant association between HLA-A28 and SD. There was no significant association between SD and BB or Bw 15. Age was also associated with SD. However, A28 was associated with SD even when corrected for age. A28 occurred more frequently in blacks than whites, 25.4% and 6.3%, respectively, and accounted for the higher incidence of SD in black adults. There were no significant associations between SD and patients' sex, donor source, number of short-term rejection treatments, or maintenance dose of corticosteroids. Steroid-induced diabetes had no adverse effects on graft or patient survival.

Reversible Adrenocorticol Insufficiency in Fulminant Meningococcemia

A 13-year-old girl who had been well previously was admitted with fulminant meningococcemic purpura. Her plasma cortisol level was only 3 micrograms/dL and failed to rise after cosyntropin administration. Maintenance doses of corticosteroid were administered. As her infection cleared her adrenal glands were restimulated and shown to have near normal response. Replacement doses of corticosteroids should be given to patients with fulminant meningococcemia until adrenal cortical insufficiency can be excluded because of the high incidence of adrenal failure in these patients and the possibility that excessive adrenal stimulation might contribute to adrenal abnormality.

CYCLOPHOSPHAMIDE IN THE ADRENALECTOMIZED ANIMAL.

IN RECENT years there has been an increasing use of chemotherapeutic agents in the treatment of various forms of cancer. The hormonal ablation by operative means has steadily gained a place in the therapy of patients strickened with cancer. That of patients stricken with cancer. That these areas of therapy should meet was inevitable. It has been previously established that caution must be taken when administering a chemotherapeutic agent to an adrenalectomized animal. This is true even when that animal is in good physiological balance with maintenance doses of corticosteroids. The evidence that appears to point toward death, from the chemotherapeutic agents, seems to be one of adrenal insufficiency. If this is true for one cytotoxic agent, will it also be true for other cytotoxic agents? This has been studied previously with 5-fluorouracil and reported. 3-4 The present study is with a cyclophosphamide ester of nitrogen mustard. Material and Methods