Steroid Therapy In Patients Research Articles

Recurrence of Inflammatory Pseudotumor of the Lung after Eleven Years of Remission

The standard treatment of inflammatory pseudotumor of the lung is surgical excision. However, little data is available on steroid therapy in patients with the unresectable disease. Here, we report a patient with recurrent inflammatory pseudotumor of the lung with pleural involvement who had been successfully treated with corticosteroid eleven years previously. Like the previous treatment, retreatment with corticosteroid proved to be effective for the recurred lesion. In addition, the patient had developed extramammary Paget's disease and bladder cancer after the initial onset of inflammatory pseudotumor. Steroid therapy could be an optional modality in treating unresectable inflammatory pseudotumor, although long-term follow-up is definitely necessary.

Cochlear blood flow during occlusion and reperfusion of the anterior inferior cerebellar artery – effect of topical application of dexamethasone to the round window

Conclusion. Topical application of dexamethasone may support autoregulation of cochlear blood flow (CBF), although it had no direct effect on CBF. Objectives. Although intratympanic steroid therapy for patients with inner ear disorders is common, the mechanism by which steroids exert their effect is unclear. We investigated the response of CBF to topical application of dexamethasone onto the round window. Materials and methods. Two concentrations of dexamethasone (3.3 mg/ml and 33 mg/ml dexamethasone in 0.5 µl saline) were applied to the round windows of rats, and CBF responses were measured using a laser Doppler flowmeter. The effects on CBF of a 2 h occlusion of the anterior inferior cerebellar artery (AICA) and subsequent release of the clamp with or without previous dexamethasone application were investigated. Results. No significant change in CBF was observed after topical application of dexamethasone, and it did not affect the decrease in CBF caused by AICA occlusion. However, recovery of CBF after release of the AICA clamp was better in animals treated with dexamethasone than in those that did not receive dexamethasone.

Resolution of Portal Hypertension Following Steroid Therapy for Hepatic Sarcoidosis

Purpose: Granulomatous diseases including sarcoidosis are an unusual cause of pre-sinusoidal portal hypertension. We present a case of a patient with both Hepatitis C and sarcoidosis who had significant improvement in portal hypertension following steroid therapy. Methods: A 51 year old Caucasian woman was diagnosed in 2001 with chronic Hepatitis C during a workup for elevated liver enzymes. She was offered treatment with interferon and Ribavirin but she refused. In 2002, she developed peripheral edema, severe anemia, grade 2 esophageal varices and significant splenomegaly. A liver biopsy showed grade 2 hepatitis with stage 3 fibrosis. In 2004, she developed jaundice for the first time and had worsened splenomegaly with secondary pancytopenia. She had gastric varices and grade 3 esophageal varices which required banding. She had never developed encephalopathy. She was offered a TIPS procedure and splenectomy but she came to our institution in 2005 for a second opinion. Laboratory studies showed pancytopenia, mildly elevated liver enzymes, normal albumin and INR. Contrasted CT scan showed extensive abnormal lymphadenopathy in the abdomen, massive splenomegaly, extensive portosystemic varices, mild ascites, normal appearing liver and a spontaneous spleno-renal shunt. Her previous biopsy was reviewed at our institution and showed multiple large granulomas consistent with sarcoidosis along with stage 3 fibrosis. Transjugular liver biopsy was repeated and confirmed the findings. Hepatic venogram showed a hepatic venous pressure gradient (HVPG) of 19 mmHg. Serum angiotensin converting enzyme level was normal. Bone marrow biopsy showed hypercellularity with no evidence of lymphoma. A diagnosis of portal hypertension secondary to sarcoidosis of the liver was made because the fibrosis in the liver was not considered advanced enough to explain the severity of portal hypertension. Results: Prednisone 60 mg a day was started to treat the sarcoidosis and was slowly tapered. Six months later, a repeat hepatic hemodynamic study showed HVPG of 7 mmHg, while still on Prednisone 20 mg a day. CT showed resolution of ascites and repeat endoscopy showed no esophageal or gastric varices. In one year, HVPG decreased further to 5 mmHg. Currently, she is on Prednisone 2.5 mg a day without any further worsening of her portal hypertension. Conclusion: This is a patient with non cirrhotic stage Hepatitis C who had portal hypertension out of proportion to her chronic viral liver disease. She was found to have extensive sarcoidosis in the liver which ultimately was treated with steroids and the portal hypertension resolved. This report supports the use of steroid therapy in patients with sarcoidosis who have portal hypertension.

Substitution of Aspartic Acid at Position 57 of the DQβ1 Affects Relapse of Autoimmune Pancreatitis

Although autoimmune pancreatitis (AIP) responds well to corticosteroid therapy, relapse during maintenance corticosteroid therapy or after the withdrawal of corticosteroid treatment is not uncommon. To date, the factors related to relapse of AIP have not been fully explored. To determine the clinical and genetic predictors relating to the relapse of AIP, we evaluated clinical factors, HLA polymorphisms, and the amino acid sequences in 40 patients with AIP. At a median follow-up period of 40 months (range, 12-67 months), relapse developed in 13 of 40 patients with AIP (33%), in whom complete remission was achieved with oral corticosteroid therapy. Among demographics, clinical characteristics in the initial diagnosis of AIP, we could not find any clinical predictor for relapse of AIP; however, in amino acid sequence analysis for relapse of AIP, the substitution of aspartic acid to nonaspartic acid at residue 57 of DQbeta1 showed a significant association with relapse of AIP (nonrelapse group, 29.6%; relapse group, 100%; P = .00003; odds ratio, 3.38; 95% confidence interval, 1.9-6.0). There was a significant difference in the timing of relapse of AIP, according to density of the nonaspartic acid residue at DQbeta1 57 (nonaspartic acid homozygosity: mean +/- SD, 6.7 +/- 4.2 months; nonaspartic acid heterozygosity: mean +/- SD, 33 +/- 11 months; P < .001). Substitution of aspartic acid to nonaspartic acid at DQbeta1 57 appears to represent a key genetic factor for relapse of AIP (ClinicalTrials.gov number, NCT00444444).

Oxidative Stress, Histone Deacetylase and Corticosteroid Resistance in Severe Asthma and COPD

Cigarette smoke-mediated oxidative stress enhances inflammation through the activation of stress kinases (JNK, ERK, p38) and redox-sensitive transcription factors such as NF-κ B and AP-1 resulting in increased expression of distinct pro-inflammatory mediators. Cigarette smoke and oxidants alter chromatin remodelling by targeted acetylation of histones and inhibition of histone deacetylase activity and in so doing further enhances inflammatory gene expression. Resistance to steroid therapy in patients with chronic obstructive pulmonary disease (COPD) and asthma has been attributed to the altered balance between oxidative stress and the acetylation-deacetylation states of histones. Corticosteroids/ glucocorticoids are potent anti-inflammatory hormones that mediate a vast array of tissue and cell specific pathways via different tissue specific co-activators or co-repressors. Glucocorticoids exert their effect via specific glucocorticoid receptors and involve modulation of the acetylation status of histones. Histone deacetylases (HDACs) are recruited by glucocorticoids which lead to deacetylation of histones and a subsequent switching off various inflammatory genes. Cigarette smoke-mediated oxidative stress alters HDAC levels by post-translational modifications with reactive aldehydes and NO present in cigarette smoke. Pharmacological agents and polyphenolic antioxidants, especially theophylline and curcumin are now known to assist glucocorticoid recruitment of HDACs, in particular HDAC2. Various therapeutic strategies are being employed either to control the activity of NF-κ B or to increase the activity of HDACs. Co-administration of theophylline, curcumin or its derivatives along with glucocorticoids could greatly overcome the resistance and enhance the therapeutic efficacy of the steroids in COPD and steroid resistant asthma. Keywords: Cigarette smoke, polyphenols, NF-κ B, glucocorticoids, histone acetylation, histone deacetylase, COPD

Hyperglycemia after Repeated Periocular Dexamethasone Injections in Patients with Diabetes

To assess the hyperglycemic effect of 3 consecutive daily periocular steroid injections in patients with diabetes. Retrospective observational study in a national eye center. Twenty-five hospitalized patients with type 2 diabetes who received a subconjunctival (n = 11) or a peribulbar injection (n = 14) with 4 mg dexamethasone disodium phosphate once a day for 3 consecutive days for ocular conditions. Baseline patient characteristics were recorded as well as serial blood glucose measurements and hypoglycemic interventions, both performed according to a written protocol. Serial blood glucose measurements and hypoglycemic interventions. Each ocular injection with dexamethasone was followed around 6 hours later by an increase of blood glucose up to a median doubling from baseline (+100% increase) followed by falls until the next injection, toward a median 13% increase from baseline before the next ocular injection. Older age (P<0.05), duration of diabetes (P = 0.01), and microangiopathy or macroangiopathy (P = 0.01) were associated with higher blood glucose rises. Using a 14-mmol/l threshold for intervention, the probability of requiring additional hypoglycemic treatment during ocular steroid therapy in patients with HbA1c >7.5% and up to 7.5% was 100% and 60%, respectively. Periocular injections with dexamethasone in patients with type 2 diabetes induce a marked hyperglycemic effect, similar to that observed during intravenous pulse methylprednisolone.

Cheilitis Granomatosa

To increase awareness of the unusual manifestations of extraintestinal Crohn's disease by reporting a solitary granulomatous lesion of the lower lip in a patient with Crohn's colitis. A biopsy of the lower lip was taken from a patient with active Crohn's disease who complained of swelling of the lips. A 53 year old female with long standing Crohn's disease (CD) described several months of discomfort and swelling of the lower lip. Physical examination revealed a fissure of the median line of the lower lip with swelling, that, on palpation, had a superficial granular texture and rubbery non pitting edema. Her serum chemistries, liver enzymes, CBC and CRP, were normal. Biopsy of the lip revealed small noncaseating granulomas, subepidermal lymphaedema, and inflammatory infiltrates. Cheilitis granulomatosa (CG) or Miescher's cheilitis, swelling of the lips, was first described in 1945, but its causes remain unknown. Allergic reactions to cobalt or food additives have been implicated. CG may be considered a subtype of oroafacial granulomatosis (OFG), which is the chronic swelling of the lips and the lower half of the face with oral ulcerations and hyperplastic gingivitis. Alone it can be viewed as a monosymptomatic variant of the Melkersson- Rosenthal syndrome (facial paralysis, swelling of the face and lips and furrows in the tongue). It has also been noted in systemic diseases sarcoidosis and CD. Only.5% of patients with CD ever develop CG. Pathological specimens may reveal non-necrotizing granulomas, edema, lymphangiectasia, and/or perivascular lymphocytic infiltration. Nevertheless, the diagnosis is made clinically based on a patient's history, symptoms and physical examination. Some authors believe a topical anesthetic with systemic corticosteroids is the most effective, while others recommend intralesional steroids. Jenss recommended local steroid therapy in patients on low dose systemic steroids if OFG occurs without gastrointestinal signs. Surgical intervention may only be considered if the disease in the quiescent stage and there is pronounced disfigurement. Although pathologically, the two entities appear grossly similar, it is unclear what the relationship is between cheilitis granulomatosa and Crohn's disease. Further immunological, microbiological and histological testing must be performed to delineate the similarities and differences between these two entities and further define their relationship.

Preoperative Steroid-Related Complications in Japanese Pediatric Patients With Ulcerative Colitis

This study was designed to clarify a limit for steroid therapy in patients with ulcerative colitis through analyzing the preoperative major steroid-related complications and to define when alternative therapies, including surgery, should be performed in pediatric ulcerative colitis patients. The medical records of 28 pediatric and 57 adult patients with ulcerative colitis who underwent total proctocolectomy and ileal J-pouch-anal anastomosis were reviewed. The relationship between the preoperative dose of glucocorticoids and major steroid-related complications, as well as the surgery variables, was evaluated. Significantly higher incidences of growth retardation, osteoporosis, glaucoma, and cataracts were noted in pediatric patients than in adult patients. In pediatric patients, major steroid-related complications occurred at a significantly lower preoperative total dosage of glucocorticoids/body weight (mg/kg) or preoperative total dosage of glucocorticoids/body surface area (mg/m2) than in adult patients. A similar surgical procedure was performed in both pediatric and adult patients. The presence of major steroid-related complications can lower a patient's long-term quality of life. Evidence-based guidelines for the recommended dose of glucocorticoids according to body weight or body surface area are needed. To allow patients to feel well and maintain a good quality of life, early introduction of alternative treatments, including surgery, should be considered.

Exhaled nitric oxide identifies the persistent eosinophilic phenotype in severe refractory asthma

The fractional concentration of exhaled nitric oxide (FENO) is increased in asthma, correlates with eosinophilic inflammation, and decreases after steroid therapy. We sought to examine whether persistent eosinophilia would be accompanied by an increased FENO level despite steroid therapy in patients with severe refractory asthma (SRA) as manifestations of steroid resistance. Subjects with SRA, subjects with mild-moderate asthma, and healthy control subjects had FENO measured, followed by endobronchial biopsy and bronchoalveolar lavage. Tissue and bronchoalveolar lavage inflammatory cells were assessed for all subjects, and eosinophil status (EOS+/EOS-) was determined for subjects with SRA. Twenty-four subjects with SRA, 15 subjects with moderate-mild asthma, and 17 healthy control subjects were studied. Subjects with EOS+ SRA had significantly higher median FENO levels compared with levels in subjects with EOS- SRA (P = .0084) and all other groups. In subjects with SRA, FENO levels correlated with tissue eosinophils (r(s) = 0.54, P = .007), lymphocytes (r(s) = 0.40, P = .003), and mast cells (r(s) = 0.44, P = .05). FENO levels of greater than 72.9 ppb were associated with a sensitivity of 0.56 and a specificity of 1.0 for EOS+ status in subjects with SRA. FENO measurement identified the subgroup of subjects with SRA with persistent eosinophilia despite steroid therapy. Further studies are needed on the use of FENO to monitor response to therapy over time in subjects with SRA.

Is Steroid Therapy Needed in the Treatment of Destructive Thyrotoxicosis Induced by α-Interferon in Chronic Hepatitis C?

Objective: Treatment with interferon (IFN) of patients affected by chronic hepatitis C (CH-C) may produce alterations in thyroid function, such as hypothyroidism, Graves’-like hyperthyroidism and destructive thyrotoxicosis (DT). IFN-induced DT is characterized by suppressed serum TSH levels, normal or elevated FT₄ and FT₃ concentrations, with the presence or absence of thyroid peroxidase antibodies and antithyroglobulin antibodies, the absence of thyroid receptor antibodies and radioactive iodine uptake suppressed or <5%. Design: IFN-induced DT is a mild clinical disease, because thyroid-destructive processes last for a short time and involve a small portion of the gland. At present, the therapeutic approach in DT suggests IFN withdrawal and 1–2 months of methylprednisolone treatment. Methods: In consideration of possible untoward side effects of steroid treatment in patients with CH-C, we studied two groups of patients with CH-C who developed DT after treatments with various preparations of recombinant IFN (with or without ribavirin). Patients sequentially entered the study during a 4-year period, at the time of DT diagnosis, when IFN therapy was discontinued. The first 12 subjects (group A) were treated with 8–16 mg/day methylprednisolone for 30–40 days after IFN withdrawal; in the following 15 patients (group B), IFN withdrawal was not followed by any additional treatment. All patients underwent clinical and laboratory controls of thyroid function at 1, 2, 3 and 6 months after DT diagnosis. Results: The results showed restoration of euthyroidism in both group A and group B patients at 6 months after DT diagnosis, regardless of steroid treatment. Conclusions: The simple withdrawal of IFN therapy in patients with CH-C, who had developed DT, appears to be effective in the treatment of the thyroid disease. This therapeutic approach should be preferred in order to avoid possible undesired side effects of steroid therapy in patients with CH-C.

The combination of ACTH-cortisone-hydrocortisone with antibiotics in the management of overwhelmingly severe infections

Jahn et al describe the salutary effects of “hormone therapy” in 82 patients with severe infection. Their clinical experience from 1950 to 1953 is summarized and includes presenting patient signs and symptoms, hospital treatment, and eventual outcome. Though not specifically stated, the mortality rate for their patients was 33%, quite similar to mortality rates observed today. More than 50% of their patients were children, many of whom had either Neisseria meningitidis or Haemophilus influenza infections. They write, “There is no question but that the administration of ACTH or cortisone in sufficient amounts to patients with severe infections will result in rapid and striking clinical improvement.” The authors suggest possible mechanisms for these beneficial effects and provide a guideline for steroid use. They specifically mention that all patients with meningococcal meningitis should receive hormonal therapy. Furthermore, they recommend the use of steroid treatment with concomitant antibiotic therapy and adjuvant measures including diet, potassium, testosterone, and estrogen. They make specific recommendations that the duration of antibiotic therapy always be greater than that of hormone therapy, lest cortisone induced “alteration of the inflammatory response permit greater proliferation and dissemination of bacteria.” In sum, strong recommendations are made for the adjuvant use of steroids in all patients with severe infections, especially meningococcal disease. They might be surprised to find that 50 years later the debate continues. Over the past 50 years, recommendations for steroid therapy in patients with severe infection have varied widely. In the 1960s and 1970s, high-dose solumedrol (30 mg/kg/dose) was accepted treatment for patients with septic shock.1 Then in 1987, two randomized trials conducted in adults and two subsequent meta-analyses suggested that steroid treatment for septic shock should be “abandoned.”2-5 More recently, Annane et al demonstrated that steroid therapy for adult patients with relative adrenal insufficiency and septic shock can significantly reduce mortality.6 In 2002, a College of Critical Care Medicine published guideline indicated that children with “refractory septic shock be treated with steroids.”7 Despite this recommendation, no efficacy data regarding steroid treatment for children with septic shock exists. As a result, efforts are underway to conduct a randomized, placebo-controlled trial to examine the value of steroid therapy in children with septic shock. Indeed, the pendulum continues to swing in the saga of steroids and infection. Will we have the definitive answer in the next citation for “50 Years Ago in The Journal”? Download .pdf (.02 MB) Help with pdf files Application 1References can be found online at www.us.elsevierhealth.com/jpeds.

Experience with programmed steroid treatment with thymectomy in nonthymomatous myasthenia gravis

Background The benefit of thymectomy in myasthenia gravis management is recognized but the perioperative course can fluctuate. The goal of this study was to assess the feasibility and clinical benefit of dose-escalated steroid therapy with thymectomy for nonthymomatous myasthenia gravis. Methods We reviewed the records of 69 myasthenia gravis patients who were followed up after undergoing transsternal thymectomy with extended anterior mediastinal dissection in our hospital between 1976–2000. Forty-eight patients in the programmed treatment group who had dose-escalated and de-escalated steroid therapy during the perioperative period comprised 17 patients with ocular myasthenia gravis and 31 patients with generalized myasthenia gravis. Clinical benefits and clinical remission, which was diagnosed when the patients were symptom-free without medications for at least 1 year, were compared with those of 21 patients in the occasional treatment group who received medications occasionally over the perioperative period. Results Postoperative respiratory failure and myasthenic crisis did not occur in the programmed treatment group but did occur in 6 patients in the occasional treatment group. Remission rates in the programmed treatment group (mean follow-up, 6.4 years) were 30% at 3 years, 38% at 5 years, and 46% at 10 years; rates in the occasional treatment group (mean follow-up, 9.6 years) were 25% at 3 years, 25% at 5 years, and 45% at 10 years. Conclusions Programmed steroid therapy in patients with nonthymomatous myasthenia gravis is feasible and it provides clinical benefit when fluctuating symptoms occur during the perioperative period.

Cohort Study of Advanced IgA Nephropathy: Efficacy and Limitations of Corticosteroids with Tonsillectomy

Background: Elevated serum creatinine is associated with poor outcome in IgA nephropathy (IgAN). The efficacy and limitations of corticosteroids in advanced IgAN (Cr ≧1.5 mg/dl), however, remains controversial. Methods: We retrospectively investigated 70 patients with advanced IgAN (Cr ≧1.5 mg/dl) classified into three groups according to their treatment regimens, that is, steroid pulse with tonsillectomy, conventional steroid, and supportive therapy. We evaluated the three groups to elucidate predictors for the endpoints ESRF and doubled serum creatinine from baseline. Results: Steroid pulse with tonsillectomy, conventional steroid and supportive therapy were performed in 30, 25 and 15 patients, respectively. During the mean follow-up period of 70.3 (12–137) months, 41.4% of patients reached ESRF (13.3 vs. 56.0 vs. 73.3%, p < 0.001) and 45.7% doubled serum creatinine from baseline (16.7 vs. 64.0 vs. 73.3%, p < 0.001). The incidence of ESRF in the patients treated by steroid pulse with tonsillectomy was significantly lower than the incidences in the patients treated by conventional steroid and supportive therapy at a baseline creatinine level of 1.5–2 mg/dl, but no statistical difference was observed at a level of >2 mg/dl. The Kaplan-Meier estimated probability of renal survival without ESRF was 89.2, 74.1 and 72.2% at 5 years and 82.8, 51.0 and 45.1% at 8 years, respectively (p = 0.017). The predictors for ESRF, identified in a Cox proportional hazards model, were baseline serum creatinine (p < 0.001) and interstitial infiltrate (p = 0.003). Steroid pulse with tonsillectomy also had a protective effect on the risk of reaching ESRF (p = 0.013). By target cross-stratification, the patients with baseline creatinine of 1.5–2 mg/dl who underwent steroid pulse with tonsillectomy showed a better renal survival rate than the others (p < 0.001). Conclusion: Steroid pulse therapy combined with tonsillectomy may be more effective than conventional steroid therapy in patients with a baseline creatinine level of ≤2 mg/dl.

Fibrillary glomerulonephritis: Early diagnosis associated with steroid responsiveness

Patients with fibrillary glomerulonephritis usually present with nephrotic proteinuria, microscopic hematuria, impaired renal function, and hypertension; 50% develop end-stage renal disease within a few years. There is no known effective therapy for fibrillary glomerulonephritis. We describe three patients with biopsy-proven fibrillary glomerulonephritis, in whom pathognomonic fibrillar deposits of 20-nm diameter were seen by electron microscopy. All patients had nephrotic syndrome and normal renal function at the time of diagnosis. They were treated initially with prednisone, 1 mg/kg body weight, then tapered individually according to the clinical course. Additional therapy consisted of an angiotensin-converting enzyme inhibitor and diuretics. Proteinuria disappeared in two of three patients after 9 and 12 months of therapy. In one patient, proteinuria was reduced from 7.49 mg/mg creatinine to 0.63 mg/mg creatinine after 6 months of treatment. The kidney function remained normal in all three cases. Two patients are now free of steroid therapy for 9 and 6 months. They show no signs of recurrence of kidney disease and have normal renal function without significant proteinuria. Steroid therapy in patients with biopsy-proven fibrillary glomerulonephritis, starting with prednisone, 1 mg/kg body weight, and tapered individually according to the clinical course, is a promising strategy. Early start of treatment in patients with preserved renal function seems to be crucial for a favorable outcome. © 2002 by the National Kidney Foundation, Inc.

The Effect of Steroid Therapy in Patients with Late ARDS

The Effect of Steroid Therapy in Patients with Late ARDS

Steroid therapy and urinary transforming growth factor-β1 in IgA nephropathy

Transforming growth factor-β1 (TGF-β1) has an important role in the pathogenesis of glomerular damage by influencing matrix metabolism. An association of TGF-β1 with glomerulosclerosis and interstitial fibrosis has been shown in various renal diseases, suggesting that TGF-β1 may serve as a diagnostic marker of glomerular diseases. The aim of this study is to determine the usefulness of urinary TGF-β1 values to monitor therapeutic effects of steroids in patients with immunoglobulin A (IgA) nephropathy. Concentrations and activation rates of TGF-β1 (mature/total) were determined in urine of patients with renal diseases by means of a double-antibody enzyme immunoassay. The urinary TGF-β1 level before steroid therapy was compared with renal histological characteristics, creatinine clearance, and proteinuria in patients with a variety of renal diseases. Urinary excretion of total and mature TGF-β1 was significantly greater in patients with crescentic glomerulonephritis and IgA nephropathy than in healthy controls, whereas the activation rate of urinary TGF-β1 was similar among patients with other renal diseases. Urinary TGF-β1 excretion at the time of renal biopsy significantly correlated with the degree of crescent formation in patients with IgA nephropathy, but not in those with glomerular sclerosis or tubulointerstitial fibrosis. Urinary excretion of total and mature TGF-β1 was reduced in patients with IgA nephropathy after treatment with prednisolone (0.8 mg/kg/d) for 1 month. The activation rate of urinary TGF-β1 also decreased significantly after steroid therapy. Urinary TGF-β1 values therefore may be useful to assess disease activity or the effects of steroid therapy in patients with IgA nephropathy. © 2001 by the National Kidney Foundation, Inc.

Monitoring of serum hepatitis C virus RNA level during steroid therapy for hepatitis C virus-positive antineutrophil cytoplasmic antibody-related glomerulonephritis: report of two cases

We report on two adult patients with hepatitis C virus-positive antineutrophil cytoplasmic antibody-related glomerulonephritis. These patients had a renal-limited form of crescentic glomerulonephritis with high levels of serum hepatitis C virus RNA. We investigated the efficiency and transition of the serum levels of creatinine, hepatitis C virus RNA, and hepatic enzymes during corticosteroid therapy in the two patients. Corticosteroid therapy consisted of intravenous high-dose methylprednisolone for 3 days, followed by oral prednisolone without cyclophosphamide. During this course of treatment, renal function in both patients recovered remarkably. A marked increase in the level of hepatitis C virus RNA, however, was observed with or without an increase in serum levels of hepatic enzymes. It seems that steroid therapy for patients who have antineutrophil cytoplasmic antibody-related pauci-immune crescentic glomerulonephritis and high levels of hepatitis C virus RNA is efficient and relatively safe, although serum levels of hepatitis C virus RNA and liver function need to be carefully monitored.

Rapid steroid withdrawal versus standard steroid therapy in patients treated with basiliximab, cyclosporine, and mycophenolate mofetil for the prevention of acute rejection in renal transplantation

Rapid steroid withdrawal versus standard steroid therapy in patients treated with basiliximab, cyclosporine, and mycophenolate mofetil for the prevention of acute rejection in renal transplantation

Immunosuppression as adjuvant therapy for biliary atresia

Despite improvements in the surgical management of biliary atresia, the long-term incidence of progressive liver failure remains high. Because chronic inflammation involving both bile ducts and liver parenchyma contributes to the pathology, the authors have hypothesized that the liver damage may be altered using immunosuppressive therapy. The aim of this study was to examine the safety and efficacy of long-term steroid therapy in patients with biliary atresia. A retrospective analysis of all patients with biliary atresia treated with an hepatoportoenterostomy and postoperative steroid therapy at our 3 institutions was undertaken. Patients were treated uniformly with immunosuppressive doses of oral steroids for a minimum of 6 weeks after surgery. Twenty-five infants with biliary atresia were treated with steroid therapy. Overall survival rate was 22 patients (88%) with a mean follow-up period of 50 months. Nineteen patients (76%) became jaundice free with native liver function. Four patients (16%) did not respond to treatment and required transplantation. Age less than 12 weeks was a crucial predictor of success of adjuvant steroid therapy. Cholangitis developed in 8 patients (32%). There were no complications caused by steroid therapy. Steroid administration at immunosuppressive doses markedly improves the clinical outcome within the first 5 years after surgery as measured by jaundice-free status and survival without liver transplantation when compared with concurrent reports. These results suggest that immunosuppressive therapy is safe and has a positive impact on the clinical course of this disease. However, a randomized study is needed to ultimately prove such an hypothesis.

Does Adjuvant Nutritional Support Diminish Steroid Dependency in Crohn Disease?

Background: Nutritional therapy plays an important role in the management of Crohn disease, particularly during the acute phase. Nutritional supplementation may also prevent relapses during the quiescent phase of Crohn disease, though this aspect has not been widely explored. Methods: Thirty-three patients with Crohn disease in remission were studied. All had steroid-dependent disease. Patients were randomized to receive either elemental diet (n = 19, EO28 Extra) or polymeric diet (Forticips, n = 14). The supplement was given orally in addition to normal food in an amount to provide 35%-50% of pre-trial total calorie intake. Prednisolone was withdrawn gradually. Patients were followed up for 12 months. Failure was defined as increase in CDAI by 100 points from baseline to >200, inability to withdraw chronic steroid therapy completely, need for surgery or steroid therapy. Results: The nutritional supplement was successful in 14 (43%) patients who remained in remission for 12 months with complete withdrawal of steroids. The response to elemental diet (42%) was similar to that of polymeric diet (43%). Nutrition supplement failed in 13 (39%). Six (18%) patients were intolerant to enteral feeding because of smell and taste problems. Per-protocol analysis of data indicated that the success rate of nutrition supplement in steroid-dependent patients was 52% (14 out of 27 patients). No disease or patient-related factors helped predict the response to nutrition supplement. Conclusion: Nutritional supplementation with either an elemental or polymeric diet may provide a safe and effective alternative to chronic steroid therapy in patients with steroid-dependent Crohn disease.