Steroid Pulse Treatment Research Articles

Thrombotic thrombocytopenic purpura associated with everolimus use in a renal transplant patient

Thrombotic microangiopathy (TMA) in renal transplantation (RTX) generally develops during treatment with calcineurin inhibitors. We present a RTX case that developed TMA under everolimus treatment. A 40-year-old woman received a kidney allograft from her 77-year-old mother. She initially received tacrolimus, mycophenolate mofetil and steroids. She was discharged with a creatinine level of 2.2 mg/dl after treatment for a cellular rejection attack within the first two weeks after transplantation. Later on, tacrolimus was replaced with everolimus. One year later, she presented with fever and increased creatinine level (4 mg/dl), anemia and thrombocytopenia. Her peripheral blood smear revealed signs of microangiopathic hemolysis. Bone marrow examination revealed an increased number of megakaryocytes. We diagnosed the case as TMA and initiated plasma exchange, I.V. pulse steroid treatment and stopped everolimus. This approach improved laboratory and clinic abnormalities. The development of TMA after treatment with everolimus and the exclusion of other possible causes suggested TMA associated with proliferating signal inhibitors (PSIs) in our case.

Successful Treatment of Necrotizing Scleritis after Conjunctival Autografting for Pterygium with Amniotic Membrane Transplantation

Purpose: To report a case of necrotizing scleritis after pterygium surgery with conjunctival autografting (CA) and intraoperative Mitomycin C (MMC) and its treatment with amniotic membrane transplantation (AMT) and systemic steroid.Methods: A 41-year-old woman who had recurrent nasal pterygium underwent pterygium excision with CA and intraoperative MMC. Two weeks after surgery, graft necrosis and necrotizing scleritis associated with severe pain and irritation were observed on her operated eye.Results: Pulse steroid treatment (intravenous 1 g methylprednisolone for 3 days) was initiated and AMT was performed for ocular surface reconstruction. Over the next 2 weeks, the graft vascularization was complete and there was no complication which required further treatment.Conclusions: Necrotizing scleritis is a rare complication of CA with MMC. Systemic steroid therapy is necessary for subsiding ocular inflammation and AMT can be performed for ocular surface reconstruction.

Pulmonary renal syndrome associated with Wegener’s granulomatosis: a case report and review of literature

Pulmonary renal syndromes are rare but serious complications of systemic vasculitis. The majority of these cases are related to ANCA-associated vasculitis. These syndromes represent a combination of diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. Here we report a case of 21-year-old female with a clinical picture of community-acquired pneumonia that rapidly evolved to pulmonary hemorrhage and acute renal failure. Combined pulse steroid treatment, immunosuppressive treatment, and plasmapheresis were instituted and resulted in the improvement of the patient. The diagnosis of Wegener's granulomatosis was established based on the clinical and serological findings (positive cANCA). Here we review the relevant literature on pulmonary renal syndromes associated with Wegener's granulomatosis and discuss their diverse clinicopathologic features.

P197 Efficacy and safety of an oral steroid pulse treatment scheme in children with epilepsy

P197 Efficacy and safety of an oral steroid pulse treatment scheme in children with epilepsy

A case of peripheral neuropathy and skin ulcer in a patient with rheumatoid arthritis after a single infusion of tocilizumab

We report a case of peripheral neuropathy and skin ulcer in a patient with rheumatoid arthritis (RA) who received tocilizumab. A 65-year-old woman with a 20-year history of RA participated in a tocilizumab clinical trial. She received a single dose of 8 mg/kg tocilizumab intravenously. The following day the patient started to experience numbness and purpura in all four extremities. The purpura of her left lower limb became necrotic, and a skin ulcer appeared 3 weeks later. Steroid-pulse treatment was initiated 12 weeks after tocilizumab administration, with the result that the numbness improved, and the skin ulcers showed complete epithelialization.

Analysis of sFas IL-6 levels in thyroid-associated ophthalmopathy: Pre- or poststeroid pulse treatment

To investigate whether serum levels of soluble Fas (sFas) and interleukin-6 (IL-6) could function as an index of the efficacy of steroid pulse treatment, we examined the serum level of these proteins before and after steroid pulse treatment in thyroid-associated ophthalmopathy patients. We gathered the blood of thyroid-associated ophthalmopathy patients before or after steroid pulse treatment, obtained serum with a centrifuge, and measured the serum levels of sFas and IL-6 by enzyme-linked immunosorbent assay (ELISA). There was no difference in serum IL-6 value between pre- and poststeroid pulse treatment. Serum sFas value was significantly decreased in both pre- and poststeroid pulse treatment. Furthermore, there was a recognizable improvement in the degree of the extraocular muscle thickening after treatment in cases in whom the serum sFas value was lower than 3 ng/ml prior to the beginning of the therapy. In patients who had a serum sFas value of more than 3 ng/ml, there was no improvement in the degree of thickening of the extraocular muscle. Serum level of sFas is an accurate index of the outcome of steroid pulse treatment in thyroid-associated ophthalmopathy and may become a useful index to gauge the status of convalescence.

Effect of Tonsillectomy Plus Steroid Pulse Therapy on Clinical Remission of IgA Nephropathy

Few well-designed investigations have examined how tonsillectomy plus steroid pulse therapy affects IgA nephropathy. A prospective, controlled study therefore was performed to compare the effects of combined therapy with those of steroid pulse alone in patients with IgA nephropathy. Fifty-five patients were followed up for 54.0 +/- 21.2 mo. Thirty-five of them underwent tonsillectomy and steroid pulse therapy (group C), and 20 received steroid pulse monotherapy (group M). Both groups received methylprednisolone intravenously, followed by oral prednisolone (initial dosage 0.5 mg/kg per d) for 12 to 18 mo. Primary evaluation items were a 100% increase in serum creatinine from baseline levels or the disappearance of urinary protein (UP) and/or occult blood (UOB) indicating clinical remission. At 24 mo after the initial treatment, the ratios of the UP and UOB disappearance were higher in group C than in group M, and the therapeutic effect persisted until the final observation. None of group C achieved a 100% increase in serum creatinine from the baseline level, whereas one patient in group M developed ESRD during the observation period. The histologic findings of repeated biopsy specimens from 18 patients revealed that mesangial proliferation and IgA deposition were significantly more reduced in group C than in group M. The Cox regression model showed that the combined therapy was approximately six-fold more effective in causing the disappearance of UP than steroid pulse monotherapy. Tonsillectomy combined with steroid pulse treatment can induce clinical remission in patients with IgA nephropathy.

Epidemic acute interstitial pneumonia in children occurred during the early 2006s

Purpose : This study was aimed to analyze the clinical characteristics of patients with acute interstitial pneumonia who had presented similar clinical patterns from March to June, 2006 and to describe our experience of treatment and to identify risk factors associated with prognosis. Methods : The clinical characteristics, radiologic and histopathologic findings and response to steroids of 15 patients (non-survival group [n=7] and survival group [control, n=8]) with acute interstitial pneumonia were investigated through the review of medical records. Results : The mean age of the patients was 26 (range: 3-48) months. Cough, cyanosis and fever were frequent symptoms. The most frequent radiologic findings on admission were pneumomediastium and extensive ground glass opacity. Surgical lung biopsy was performed on 8/15 (53.3%) patients and diffuse alveolar damage was found. Mechanical ventilation was applied for 9/15 (60.0%) patients for 40 (range: 1-99) days. Five patients in survival group received steroid treatment and 7 patients in non-survivial group (P=0.20). One patient in survival group received steroid pulse treatment and 4 patients in non-survival group (P=0.12). Seven patients died all of respiratory failure. The survival rate was 53.4%. Conclusion : The patients with acute interstitial pneumonia which occurred on spring 2006 showed high mortality because of rapidly and extensively progressing pulmonary fibrosis and air leakage. Therefore, we should consider surgical lung biopsy and steroid application earlier. We should recognize this acute interstitial pneumonia occurring on spring in domestics and need to investigate the cause and treatment in large scale.

Very Early Onset Steroid-Induced Avascular Necrosis of the Hip and Knee in a Patient with Idiopathic Thrombocytopenic Purpura

Avascular necrosis (AVN) is a devastating adverse effect of steroid therapy rarely reported in idiopathic thrombocytopenic purpura (ITP). We describe a 64-year-old woman with progressive left knee and hip pain for 30 days resulting with the inability to ambulate. After she had been diagnosed ITP pulse steroid treatment was started in the department of Hematology. Two weeks later she described left knee and left hip pain. On physical examination, motion in her left knee and hip was severely painful without inflammation but range of motion was not limited. Magnetic resonance imaging showed multiple bony infarcts in the proximal femur, distal femur and proximal tibia, consistent with AVN. This case report is the first to define concurrent hip and knee AVN at a very early stage due to steroid use in a patient with ITP.

成人において敗血症を伴った細菌性髄膜炎治療中に急性散在性脳脊髄炎 (ADEM) の合併が疑われた1症例

A 39-year-old man with a high fever, headache, and stiff neck, and Kernig and Brudzinski signs admitted in June 2004 had a WBC of 10,680/microL and CRP of 10.5mg/dL. Streptococcus pneumoniae was detected in blood and spinal fluid culture, but brain CT was normal. Meningitis was diagnosed and antibiotics and corticosteroids begun. After four days of treatment, his blood test and spinal fluid data had improved, but his consciousness had deteriorated. ADEM was diagnosed by the clinical course and brain MRI (T2-weighted image) that showed high-intensity lesions in the white cerebral matter, and steroid pulse treatment was begun on day 4 after admission. His consciousness disturbance rapidly disappeared and brain MRI showed that the multiple high-intensity lesions had ameliorated. The patient was released after 40 days of treatment.

Successful therapy with steroid and cyclophosphamide pulse for CNS lupus and lupus myelitis

The patient was a 33 year female. In 2001, she was diagnosed with systemic lupus erythematosus (SLE) and treated with prednisolone and ciclosporin. In May 2006, she noticed slight muscle weakness in the bilateral lower limbs. In July of the same year, she experienced gait difficulty and was admitted to our hospital because of fatigue, appetite loss, fever and disorientation. Soon afterwards, she had a fit of general convulsion and suffered from urinary retention and fecal incontinence. A brain magnetic resonance image revealed atrophy of the thoracic cord in T2 weighted images, and cerebrospinal fluid examination showed high total protein and interleukin-6 concentration, indicating complication of lupus myelitis as well as cerebral involvement. Steroid pulse and oral prednisolone treatment resulted in ameriolation of cerebral complications such as disorientation and convulsion, but muscle weakness and paresthesia in the lower limbs and urinary retention persisted. Cyclophosphamide pulse therapy was started and resulted in a marked recovery from muscle weakness, paresthesia and urinary retention, and she could discharge. We conclude that steroid and cyclophosphamide pulse therapy for a SLE patient with CNS lupus and lupus myelitisis is effective for ameriolation of symptoms such as disorientation, convulsion, urinary retension, fecal incontinence, muscle weakness and paresthesia in the lower limbs as well as elevated level of serum anti-ribosomal P antibody.

Effective immunosuppresive therapies including steroid pulse treatment for intramuscular hematoma in iliopsoas in acquired hemophilia

Effective immunosuppresive therapies including steroid pulse treatment for intramuscular hematoma in iliopsoas in acquired hemophilia

Effectiveness of CT for Clinical Stratification of Occupational Lung Edema

We treated two occupational lung diseases in different situations during military training. The purpose of this study is to investigate the availability of CT scanning for the evaluation of inhalation pulmonary edema. Two soldiers suffered severe lung edema after using a spray for the daily maintenance of their firearms. Four soldiers suffered severe dyspnea after undertaking drills in a narrow zone where numerous smoke bombs had been used. We evaluated these patients from several aspects. CT scans of the chest of spray-induced patients revealed bilateral infiltration predominantly in the upper lung fields. The patients received steroid pulse treatment and gradually recovered. CT scans of the chest of smoke-induced patients revealed bilateral ground-glass attenuation with peripheral lung sparing. The patients gradually recovered with steroid therapy. In accordance with previous studies, CT scans of the chest in our patients demonstrated that the periphery of the lungs remained normal, except in cases of serious injury. When differential diagnosis is required, we consider that CT scans of the chest are particularly useful; CT findings are useful in determining the severity of lung injury as well as the diagnosis of inhalation pulmonary edema.

Avascular necrosis of the bones: an overlooked complication of pulse steroid treatment of multiple sclerosis

The most common cause avascular osteonecrosis (AVN) is corticosteroid medication. However, the impact of pulse steroid therapy on AVN development is not known properly. We intended to investigate the effect of this therapy on AVN development in multiple sclerosis (MS) patients. MS patients without AVN, chronic alcoholism, thrombophilia, sickle cell anemia, inflammatory bowel disease, cytotoxic drug usage history participated in the study. All of the patients were ambulatory (EDSS < 4). Thirty-three MS patients who received pulse steroid therapy at least 10 g or at least 15 g during the course of the whole disease, were the treatment group and 27 MS patients who did not receive any steroid therapy were the control group. There was no statistical difference between groups for age, sex and disease duration. Bilateral femoral magnetic resonance imaging (MRI) was performed to the both groups. In the treatment group, five patients (15.5%) had AVN whilst no AVN was found in the control group. In conclusion, it is interesting to find AVN at such a high rate in the MS patients who received pulse steroid therapy without any AVN history or complaint. The neurologists have to be more careful about AVN which early diagnosis is important to prevent the complications.

Utility of CT findings on diagnosis of occupational lung disease

Introduction We experienced two occupational lung diseases on a different situation in military training. The purpose of this study was to investigate the use of CT images for inhaled pulmonary edema from the standpoint of early diagnosis and assessment of severity. Materials and methods Case 1: Two soldiers who used the rust proofing sprays and dust removal sprays for the daily maintenance of their arms suffered from severe lung edema. Case 2: Four soldiers suffered from severe dyspnea because they drilled in the field where many smoke canisters were used. We evaluated these patients by clinical symptoms, laboratory data, pulmonary function test, chest X-P and CT images. Results Case 1: Their chest CT scan showed bilateral infiltration mainly in the upper lung field. Steroid pulse treatment was used for recovery. Seven days later, their chest X-P showed almost normal. Case 2: Their chest radiographs showed mild infiltrative shadows. Their chest CT scan showed bilateral ground grass attenuation with the peripheral lung spared. They recovered gradually by steroid therapy. Seven days later, mild abnormal shadow was observed in their chest CT images. Discussion In the literature, chest CT scan showed diffused ground glass opacities mainly in the upper lung field of inhaled pulmonary edema patients. The peripheral part of the lungs was kept normal excluding the seriously injured cases. These findings are the same as those in our cases. When differential diagnosis was needed, we considered chest CT scan especially useful. The findings of CT scan are useful not only for the diagnosis of inhaled pulmonary edema but also for determining the severity of lung injury.

自発性上眼瞼向き眼振を認めた多発性硬化症の１例

Primary position upbeat nystagmus (PPUN) is an important indication of central nervous system disorder. We herein report a case of PPUN accompanied with multiple sclerosis.The case was a 32-year-old female. Nausea, vomiting, and numbness of the upper limbs developed on January 14, 2004. Upbeat nystagmus was observed in the primary and supine positions. She was hospitalized on January 27, because abnormal signals in the medulla oblongata and tegmentum mesencephali were noted on brain MRI imaging. After steroid pulse treatment was started, her neurological symptoms and PPUN disappeared. She was discharged from hospital on February 13.

Glucocorticosteroid therapy decreases CD14-expression and CD14-mediated LPS-binding and activation of monocytes in patients suffering from systemic lupus erythematosus

In order to study the possible action of glucocorticosteroids (GCS) on the CD14/Toll like receptor mediated activation of monocytes the CD14-expression, CD14-mediated LPS binding and activation of these cells of patients suffering from Systemic Lupus Erythematosus receiving no, low dose or pulse steroid treatment was studied. The CD14-expression was determined on whole blood monocytes by flow cytometry, while the LPS-binding of an FITC-LPS preparate and the LPS-induced TNFα secretion were tested on isolated monocytes. The CD14-dependent and -independent LPS-binding and activation were evaluated with the help of a blocking anti-CD14 mAb. Our results showed that the CD14-expression, CD14-dependent LPS-binding and activation were significantly inhibited by the in vivo applied pulse steroid therapy. In contrast, the CD14-independent LPS-binding and activation were not altered by the GCS treatment. Our data provide further in vivo evidence for a possible new way of GCS therapy is able to initiate its anti-inflammatory action.

Seronegative systemic lupus erythematosus: etiology of nephrotic syndrome and acute renal failure in early postpartum period

Systemic lupus erythematosus (SLE) is an autoimmune syndrome that occurs most commonly in women during their reproductive years. Nephritis is known to be one of the most serious complications of SLE. Lupus nephropathy is frequently associated with ANA and anti-dsDNA antibodies. Rarely, serological markers may be initially absent, and in many cases, they become positive after sometime. We present a 28-year old, otherwise healthy female who admitted to our clinic with edema, hypertension, proteinuria and acute renal failure following her fourth delivery. Serum immunological markers were negative and renal biopsy showed histopathological changes consistent with systemic lupus erythematosus as the etiology of nephrotic syndrome. A dramatic therapeutic response was achieved by pulse steroid and cyclophosphamide treatment following oral steroid therapy. In women with new onset nephrotic syndrome or renal function deterioration in postpartum period, even if the patient is asymptomatic or seronegative, it is crucial to exclude SLE for a rapid diagnosis and prompt treatment in the case of lupus nephritis. Renal biopsy is of diagnostic importance in such cases in which there is no other clinical, biochemical and serological evidence of the disease.

Successful steroid pulse treatment in childhood acquired haemophilia with nephrotic syndrome

We encountered a 2-year-old boy with acquired haemophilia, which rarely occurs in children, who was complicated with nephrotic syndrome. In mid-August 2001, he was diagnosed to have nephrotic syndrome based on the presence of massive proteinuria and hypoalbuminaemia. Activated partial thromboplastin time (APTT) was normal at 42.4 s at that time. After starting prednisone administration of 2 mg kg(-1) day(-1), the proteinuria disappeared immediately. However, in early October the same year, subcutaneous ecchymosis and intramuscular bleeding occurred for no apparent reason, and from the examination results his APTT was 106.4 s, factor VIII (FVIII) activity was <1%, and the anti-FVIII inhibitor titre was 6.9 BU ml(-1). As a result, he was diagnosed to have acquired haemophilia. The anti-nuclear antibody and anti-phospholipid antibody were negative. With recombinant activated FVII, haemostasis was obtained, and after administering three courses of steroid pulse therapy (methyl prednisolone: 20 mg kg(-1) day(-1) x 3 days), the anti-FVIII inhibitory activity disappeared. An analysis of the immunological and coagulation properties of his FVIII autoantibodies showed the anti-FVIII inhibitory activity to be mediated by IgG(1) antibody. In other words, his FVIII inhibitor was a Th1 dominant and it provided a good response to treatment. These findings correlate with those of previous reports. The patient thereafter frequently demonstrated a recurrence of nephrotic syndrome. As a result, he is presently being managed with cyclosporine. However, no recurrence of the anti-FVIII titre has been observed.

The role of anti–IL-2 receptor in high-risk kidney transplant patients

Anti–IL-2 receptor has been proved to be effective in reducing the rate of acute rejection in kidney transplantation and also improving both the rate of graft and patient survival. In this study, we retrospectively review the role of anti–IL-2 receptor as induction immunosuppression in immunologically high-risk kidney transplant patient compared with normally low-risk patients. Methods From January 1999 to December 2002, we performed 246 kidney transplantations in two transplant centers in Bangkok. These were divided into two groups: group 1, high-risk group containing 50 patients who had one of the following criteria: (1) high panel reactive antibody (>50%); (2) retransplantation; (3) marginal donor (with expectancy of delayed graft function); (4) spouse donor; (5) >4 HLA mismatch. All group 1 patients receive anti–IL-2 receptor as induction immunosuppression (either Basiliximab ( n = 27) or Daclizumab ( n = 23).) Group 2 consisted of the control group of 196 patients with normal immunological risk. The following data of both groups were collected and analyzed: patient demography, type of donor, acute rejection incidence, severity, and time. Results In this study, the anti–IL-2 receptors are 27 cases of Basiliximab and 23 cases of Daclizumab. The rates of acute rejection are not significantly different in both groups, namely, 46 of 194 (23.7%) in group 2 compared with 10 of 50 (20%) episodes in group 1 ( P = .602). All rejections in both groups responded to pulse steroid treatment. The mortality rate and rate of graft failure were also not significantly different, i.e., 6 of 196 (3.1%) vs 2 of 50 (4.0%) ( P = .666) and 7 of 196 (3.6%) vs 3 of 50 (6.0%) ( P = .429) in low risk group versus high risk group, respectively. Kaplan-Meier estimates of the probabilities of acute rejection free, patient survival rate, and graft survival rate also showed no difference between groups. Conclusions The use of anti–IL-2 receptor antibodies as induction immunosuppression in immunologically high-risk patients results in the same rate of acute rejection, severity of acute rejection, graft survival, and patient survival as recipients with normal immunological risk.