Maintenance Dose Of Prednisone Research Articles

Abstract #1401368: Does A Male Sheehan’s Like Syndrome Exist?

Panhypopituitarism is defined as a condition in which secretion of most or all hormones from the pituitary gland is inadequate or absent. Deficiency of pituitary hormones such as Adrenocorticotropic Hormone (ACTH) and Thyroid Stimulating Hormone (TSH) impairs the ability of the adrenal glands and thyroid gland to secrete hormones that are vital for survival. Hypopituitarism is a rare condition, and its incidence is approximately 4.2 per 100,000 per year. Sheehan syndrome is a form of pituitary necrosis secondary to infarction in setting of severe postpartum hemorrhage. Hemorrhagic pituitary apoplexy of pre-existing pituitary adenomas in setting of hypotension has been reported. We describe a unique case of hypopituitarism in a man without known pituitary adenoma after liver transplantation. A 48-year-old male underwent liver transplantation for cirrhosis. A few years after the transplant the patient developed dizziness, lightheadedness, polyuria, polydipsia, decreased libido, loss of muscle mass and fatigue. Post transplant he was on maintenance dose of Prednisone which was discontinued several years after the surgery. Testosterone replacement therapy with testosterone gel for fatigue was initiated by primary care provider, and he was referred to endocrinology for further evaluation. Hormonal profile revealed TSH 0.724 mcIU/mL (0.450-4.500), free T4 1.13 ng/dL (0.82-1.77), AM cortisol 5.2 mcg/dL (6.2-19.4), ACTH stimulated cortisol 11.4 mcg/dL after 30 min, 14.8 mcg/dL after 90 min, morning total testosterone 104.8 ng/dL (264-916), free testosterone 4.1 pg/mL (6.8-21.5), prolactin 2.1 ng/mL (4.0-15.2), LH 1.2 mIU/mL (1.7-8.6), IGF-1 26 ng/mL (75-216), FSH 1.6 mIU/mL (1.5-12.4), ACTH 19.6 pg/mL (7.2-63.3). Sodium was 143 mmol/L with urine osmolality 1135 mOsm/kg after overnight water deprivation so DI was successfully ruled out. An MRI of the brain demonstrated a very small pituitary gland within a small pituitary fossa. This was a significant change compared to the MRI performed soon after transplantation - it was unremarkable with normal sella and parasellar structures. The findings of empty sella on MRI and blood work consistent with hypogonadism, central adrenal insufficiency, hypoprolactinemia and growth hormone deficiency indicated a diagnosis of panhypopituitarism. Since ACTH stimulation test demonstrated partial adrenal insufficiency Hydrocortisone 20 mg daily was initiated. Patient was switched from gel to injectable testosterone with significant improvement in testosterone concentration. During a follow up visit patient reported feeling better but still endorsed fatigue and tiredness. Trial of growth hormone therapy resulted in resolution of his symptoms and improved wellbeing. This case describes a unique presentation of hypopituitarism after major surgery in a patient with intact pituitary gland possibly secondary to hypotension during surgery and the pathophysiology may be analogous to Sheehan syndrome. Early diagnosis of hypopituitarism is crucial as deficiency in ACTH and TSH can cause adrenal insufficiency and hypothyroidism, respectively, which could be life-threatening.

Cyclosporine treatment for steroid-resistant nephrosis complicated with acquired hemophilia A: a case report.

A 12-year-old girl with steroid-resistant nephrosis (SRN) was admitted complaining of pain in her right leg and an ultrasound showed a hematoma in her right calf. The coagulation profile revealed partial thromboplastin time prolongation and high titers (156 BU) of anti-factor VIII inhibitors were observed. Where half of the patients with antifactor VIII inhibitors were associated with underlying disorders, additional tests were performed that rule out secondary causes. This patient with long-standing SRN, who was on a maintenance dose of prednisone for six years, was complicated with acquired hemophilia A (AHA). In different to the last recommendations of AHA treatment, we preferred using cyclosporine; which is considered the initial second-line therapy for children with SRN. Complete remission was achieved of both disorders after a month with no recurrence of nephrosis or bleeding events. To our knowledge, nephrotic syndrome with AHA was reported in only three patients, two cases after remission and one during a relapse but no one were treated with cyclosporine. The authors encountered the first case of cyclosporine treatment for AHA in a patient with SRN. This study supports the use of cyclosporine to treat AHA, especially with nephrosis.

Real-word adrenocorticotropic hormone treatment for childhood-onset nephrotic syndrome.

Current first-line anti-proteinuric treatments do not produce a satisfactory therapeutic effect in a considerable number of patients with nephrotic syndrome (NS). Interest in adrenocorticotropic hormone (ACTH) for the treatment of NS has recently been revived. The present study investigated the efficacy and safety of ACTH treatment in children with frequent relapsing NS (FRNS), steroid-dependent NS (SDNS), and steroid-resistant NS (SRNS). The ACTH treatment group was comprised of NS patients receiving ACTH treatment. Patients with serum cortisol concentrations <85.3 nmol/L and who had not received ACTH treatment previously were enrolled in the control group from January 2018 to January 2021. The maintenance dose of prednisone, the number of disease recurrences, the time of first disease relapse, immunosuppressant use, serum cortisol levels, and adverse events were recorded in both groups. Fifty-one patients were included in the ACTH group, and twenty-one patients were enrolled in the control group. Concurrent treatment with one or more immunosuppressive and/or cytotoxic treatments occurred in 92.2% and 85.7% of patients in the ACTH and control groups, respectively, throughout the study period. A greater reduction in the prednisone maintenance dose was observed in the ACTH group compared with the control group after 1 year of follow up (0.603 ± 0.445 mg/kg vs. 0.267 ± 0.500 mg/kg, p = 0.006). During the one-year study period, fewer participants experienced one or more disease relapses in the ACTH group (45.1%) compared to the control group (76.2%, odds ratio = 3.896, p = 0.016). The number of disease recurrences per patient in the ACTH group was less than that in the control group (median difference = -1, p = 0.006). The mean length of remission was 8.902 m and 7.905 m in the ACTH group and control group, respectively. A log-rank test showed a longer relapse free survival for patients in the ACTH group (p = 0.046), but the Breslow test showed no significant difference between groups (p = 0.104). Ten patients in the ACTH group successfully discontinued all drug therapies. No patients in the control group were able to discontinue drug therapy as of February 2022. ACTH, combined with multiple drugs, is effective at reducing the prednisone maintenance dose and may effectively prevent disease relapses in childhood NS.

Short Stature in Patients with Diamond-Blackfan Anemia: A Cross-Sectional Study

To systematically describe the short stature of patients with Diamond-Blackfan anemia and to explore factors affecting the height development of patients with Diamond-Blackfan anemia. This cross-sectional study was conducted at the Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, and the height, weight, and clinical data of 129 patients with Diamond-Blackfan anemia were collected from June 2020 to September2020. The median height-age-z score (HAZ) of children affected by Diamond-Blackfan anemia was -1.54 (-6.36-1.96). Short stature was found in 37.98% of the patients. Specific Diamond-Blackfan anemia growth curves were developed for weight, height, and body mass index, separately for male and female patients. Multivariable logistic regression models showed that female sex (aOR 4.92; 95% CI 1.29-18.71; P=.0195), underweight (aOR 10.41, 95% CI 1.41-76.98, P=.0217), cardiovascular malformations (aOR 216.65; 95% CI 3.29-14279.79; P=.0118), and RPL11(aOR 29.14; 95% CI 1.18-719.10; P=.0392) or RPS26 (aOR 53.49; 95% CI 1.40-2044.30; P=.0323) mutations were independent risk factors for short stature. In the subgroup of patients who were steroid-dependent, patients with a duration of steroid therapy over 2years (OR 2.95; 95% CI 1.00-8.66; P=.0494) or maintenance dose of prednisone >0.1mg/kg per day (OR 3.30; 95% CI 1.02-10.72; P=.0470) had a higher incidence of short stature. Patients with Diamond-Blackfan anemia had a high prevalence of short stature. The risk of short stature increased with age and was associated with sex, underweight, congenital malformations, and RPL11 or RPS26 mutations. The duration of steroid therapy and maintenance dose of steroid was significantly associated with the incidence of short stature in steroid-dependent patients with Diamond-Blackfan anemia.

Predicting lupus low disease activity state and remission in SLE: novel insights

ABSTRACT Introduction:Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with extreme heterogeneity, which sometimes may be life-threatening. Principles of treat to target (T2T) in SLE were put forward more recently, leading to better long-term survival and reduced damage accrual. Areas covered: Lupus low disease activity state (LLDAS) and remission are currently the most widely accepted principal goals of SLE-T2T recommendations. In this article, we will deliver the novel insights into the definitions of LLDAS/remission, attainability, and, most importantly, clinical predictors of LLDAS and remission in SLE. Expert opinion: Since the release of the LLDAS and the framework on definitions of remission in SLE, there has been much evidence of a correlation between target attainment or maintenance and better prognosis. In the meantime, researchers are searching for predictors of target attainment. Noteworthy, prospective randomized trials are lacking worldwide to verify the benefits of T2T in various aspects of SLE. The most essential issue is that the optimal definition of the therapeutic target for SLE remains controversial, particularly regarding the maintenance dose of prednisone, the need for immunosuppressive withdrawal, and the requirement for serologic conversion. How to implement T2T principles in clinical practice also needs further investigation.

IDIOPATHIC PULMONARY HEMOSIDEROSIS IN A DOWN SYNDROME PATIENT

SESSION TITLE: Fellows Diffuse Lung Disease Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Idiopathic Pulmonary Hemosiderosis (IPH) is a rare disease found primarily in children characterized by recurrent episodes of diffuse alveolar hemorrhage. The exact etiology and pathogenesis of IPH is unknown. Patients with Down Syndrome have a higher risk of developing pulmonary hemosiderosis. Additionally, in this population IPH manifests more severely. CASE PRESENTATION: Patient is 25 months old ex 36-week female with history of Trisomy 21, Atrial septal defect (ASDs) who has had a history of recurrent hemoptysis, anemia, and pulmonary infiltrates. Patient underwent extensive work-up including bronchoscopy and lung biopsy with the final diagnosis of Idiopathic Pulmonary Hemosiderosis (IPH). The patient presented to our PICU with acute alveolar hemorrhage and severe anemia secondary to RSV pneumonia and IPH recurrence despite being on maintenance therapy of prednisone and hydroxychloroquine. Patient required advanced respiratory support and was treated with a short course of pulse steroids. Patient eventually was placed on Azathioprine instead of Hydroxychloroquine with an increased Prednisone maintenance dose. DISCUSSION: IPH generally occurs in children below the age of 10 years, most commonly from 1 to 7 years. The classical triad of anemia, hemoptysis, and pulmonary infiltrates is a distinguishing characteristic of this disease; however, any one of them could be the initial manifestation. The estimated prevalence of IPH reaches 1.85 per 1,000,000 children, compared to 138.5 per 1,000,000 children with Down Syndrome. Patients with Down Syndrome tend to present earlier and more severely; they experience more dyspnea at diagnosis, more frequent secondary pulmonary hypertension (PAH), and an increased risk of fatal evolution. Systemic glucocorticoids are the mainstay of therapy for acute alveolar hemorrhage due to IPH. In a prior series of 23 children with IPH, low-dose oral prednisone was associated with prolonged survival compared with historical controls. In another series of 17 patients, long-term glucocorticoid therapy was associated with a five-year survival of 86 percent. The duration of corticosteroid treatment was variable among the patients based on clinical, radiological (alveolar infiltrates), and biological (hemoglobin, reticulocytes) evolution. Currently, there are no definitive guidelines directing use or choice of steroid-sparing medications in IPH; however, a few case reports describe the use of an immunosuppressant (cyclophosphamide, hydroxychloroquine, mycophenolate mofetil, azathioprine) in conjunction with oral glucocorticoids in the setting of respiratory failure. CONCLUSIONS: Additional studies investigating different therapeutic options are critical in order to reduce overall morbidity & mortality, particularly in children with Down Syndrome who are more predisposed to IPH relapse. Reference #1: Dr. Inderpal Randhawa Reference #2: Dr. Bugsu Ovunc Reference #3: Dr. Cyrus Shahriary DISCLOSURES: No relevant relationships by Yaron Fireizen, source=Web Response No relevant relationships by Nastasia Nianiaris, source=Web Response No relevant relationships by Inderpal Randhawa, source=Web Response No relevant relationships by Cyrus Shahriary, source=Web Response No relevant relationships by Dibanni Vasquez, source=Web Response

Tolosa Hunt Syndrome: Current Diagnostic Challenges and Treatment

Tolosa-Hunt syndrome (THS) is an uncommon diagnosis with an incidence of nearly 1 to 2 cases per million hallmarked by the presence of painful ophthalmoplegia (PO) due to a granulomatous inflammation (GI). Diagnostically, the major THS challenges encountered are owing to the exclusion of other GI presenting conditions necessitating multi-specialization consultations. This article presents uniquely advances in diagnosis and challenges encountered attempting to exclude THS mimics, details on physical examination and laboratory investigations have been incorporated. Tolosa Hunt MRI protocol (contrast-enhanced MRI), restricted diffusion and CISS MRI have lately proved to be precise investigations for THS diagnosis and follow up, on the contrary, number of false-negative/positive MRI diagnoses appears to be rising, hence proposed that MRI or biopsy shouldn’t be mandatory criteria for diagnosis as opposed to IHS 2018 guidelines. Despite corticosteroids being the cornerstone therapy for THS, there are controversies concerning the better administration route, optimal dosage, and therapy longevity, through case reports, high dose IV mPSL of 500 mg BID for 3 days, followed with maintenance dose of prednisone 60 mg resulted in the earliest recovery, hereafter standardized guidelines are required. Alternatively, infliximab (300 mg infusion), azathioprine, methotrexate, and acupuncture can be used, farther studies are required to appraise the effectiveness and their safety. On the other side, microsurgery can be utilized for GI evacuation however associated risks of permanent CN VI palsy have been stated, nonetheless, GKRS can be employed when contraindication or corticosteroid intolerability exists though the hazard of late malignancy is a drawback.

Delirium as presentation of late-onset and relapsing Susac syndrome

A 67-year-old patient, only known for bilateral presbycusis, presented with subacute onset of delirium. Clinical examination showed multifocal neurological deficits, all together suggesting subcortical frontal dysfunction together with cerebellar and...

Role of local eosinophilopoietic processes in the development of airway eosinophilia in prednisone-dependent severe asthma.

In severe asthmatics with persistent airway eosinophilia, blockade of interleukin-5 has significant steroid-sparing effects and attenuates blood and sputum eosinophilia. The contribution of local maturational processes of progenitors within the airways relative to the recruitment of mature cells from the peripheral circulation to the development of airway eosinophilia is not known. We hypothesize that local eosinophilopoiesis may be the predominant process that drives persistent airway eosinophilia and corticosteroid requirement in severe asthmatics. In a cross-sectional study, the number and growth potential of eosinophil-lineage-committed progenitors (EoP) were assayed in 21 severe eosinophilic asthmatics, 19 mild asthmatics, eight COPD patients and eight normal subjects. The effect of anti-IL-5 treatment on mature eosinophils and EoP numbers was made in severe eosinophilic asthmatics who participated in a randomized clinical trial of mepolizumab (substudy of a larger GSK sponsored global phase III trial, MEA115575) where subjects received mepolizumab (100mg, n=9) or placebo (n=8), as six monthly subcutaneous injections. Mature eosinophil and EoP numbers were significantly greater in the sputum of severe asthmatics compared with all other subject groups. In colony-forming assays, EoP from blood of severe asthmatics demonstrated a greater response to IL-5 than mild asthmatics. Treatment of severe asthmatics with mepolizumab significantly attenuated blood eosinophils and increased EoP numbers consistent with blockade of systemic eosinophilopoiesis. There was however no significant treatment effect on mature eosinophils, sputum EoP numbers or the prednisone maintenance dose. Patients with severe eosinophilic asthma have an exaggerated eosinophilopoeitic process in their airways. Treatment with 100mg subcutaneous mepolizumab significantly attenuated systemic differentiation of eosinophils, but did not suppress local airway eosinophil differentiation to mature cells. Targeting IL-5-driven eosinophil differentiation locally within the lung maybe of relevance for optimal control of airway eosinophilia and asthma.

Relapses in patients with giant cell arteritis: prevalence, characteristics, and associated clinical findings in a longitudinally followed cohort of 106 patients.

Giant cell arteritis (GCA) is a relapsing disease. However, the nature, chronology, therapeutic impact, and clinical consequences of relapses have been scarcely addressed. We conducted the present study to investigate the prevalence, timing, and characteristics of relapses in patients with GCA and to analyze whether a relapsing course is associated with disease-related complications, increased glucocorticoid (GC) doses, and GC-related adverse effects. The study cohort included 106 patients, longitudinally followed by the authors for 7.8 ± 3.3 years. Relapses were defined as reappearance of disease-related symptoms requiring treatment adjustment. Relapses were classified into 4 categories: polymyalgia rheumatica (PMR), cranial symptoms (including ischemic complications), systemic disease, or symptomatic large vessel involvement. Cumulated GC dose during the first year of treatment, time required to achieve a maintenance prednisone dose <10 mg/d (T10), <5 mg/d (T5), or complete prednisone discontinuation (T0), and GC-related side effects were recorded. Sixty-eight patients (64%) experienced at least 1 relapse, and 38 (36%) experienced 2 or more. First relapse consisted of PMR in 51%, cranial symptoms in 31%, and systemic complaints in 18%. Relapses appeared predominantly, but not exclusively, within the first 2 years of treatment, and only 1 patient developed visual loss. T10, T5, and T0 were significantly longer in patients with relapses than in patients without relapse (median, 40 vs 27 wk, p < 0.0001; 163 vs 89.5 wk, p = 0.004; and 340 vs 190 wk, p = 0.001, respectively). Cumulated prednisone dose during the first year was significantly higher in relapsing patients (6.2 ± 1.7 g vs 5.4 ± 0.78 g, p = 0.015). Osteoporosis was more common in patients with relapses compared to those without (65% vs 32%, p = 0.001). In conclusion, the results of the present study provide evidence that a relapsing course is associated with higher and prolonged GC requirements and a higher frequency of osteoporosis in GCA.

Treatment with angiotensin II receptor blockers is associated with prolonged relapse-free survival, lower relapse rate, and corticosteroid-sparing effect in patients with giant cell arteritis

ObjectiveTo determine whether concomitant treatment with angiotensin-converting enzyme inhibitors (ACEI) or angiotensin receptor blockers (ARB) is associated with changes in the outcome of patients with giant cell arteritis (GCA). MethodsA study cohort of 106 patients with biopsy-proven GCA was longitudinally followed up for 7.8 ± 3.3 years. Patients were stratified according to their treatment with ACEI, ARB, or no ACEI/ARB. Time to first relapse, number of flares, time to achieve a stable prednisone dose <10mg/day and <5mg/day with no relapses, time required to completely discontinue prednisone, cumulative dose of prednisone received during the first year, and concentrations of acute-phase reactants at pre-defined time points (baseline, 6, 12, 18, and 24 months) were compared among the 3 groups. Cox proportional hazards models were performed to adjust for potential confounders. ResultsPatients receiving ARB presented a significantly longer relapse-free survival than patients treated with ACEI or patients not receiving ACEI/ARB (p = 0.02). The adjusted hazard ratio for relapses in patients treated with ARB was 0.32 (95% CI: 0.12–0.81, p = 0.017). In addition, patients who received ARB achieved a prednisone maintenance dose <10mg/day faster than all other patients (p = 0.0002). No significant differences were observed among groups in acute-phase reactant levels during follow-up. However, patients not receiving ACEI/ARB had significantly higher C-reactive protein and haptoglobin concentrations than those receiving ACEI or ARB at various time points. ConclusionsAddition of ARB to glucocorticoids is associated with lower relapse rate and more prolonged disease-free survival in patients with GCA.

Methotrexate in the treatment of pemphigus vulgaris: experience in 23 patients

The standard treatment for patients with pemphigus vulgaris has long consisted of high-dose glucocorticoids. Studies regarding the use of methotrexate in pemphigus vulgaris date back to 1968, but few have quantitatively described a steroid-sparing effect conferred by methotrexate. We sought to evaluate the efficacy of methotrexate in 23 patients with pemphigus vulgaris, using the drug's steroid-sparing effect as the primary indicator of clinical improvement. We investigated whether methotrexate could be used as monotherapy in some patients. Retrospective chart review was used to analyse the records of patients with pemphigus vulgaris treated with methotrexate at the New York University Langone Medical Center for at least three consecutive months between 2000 and 2012. Diagnosis was made by tissue biopsy and either direct or indirect immunofluorescence tests and enzyme-linked immunosorbent assay. Improvement in clinical symptoms was observed in 91% of patients. Sixteen patients (70%) were eventually weaned completely off prednisone, with a mean time to discontinuation of 18months. In total 23% of patients enjoyed a partial steroid-sparing effect, requiring a mean maintenance dose of prednisone of 6·75mg daily. Two patients (9%) developed possible adverse events requiring cessation of the drug, and one patient received no therapeutic benefit from the drug. Methotrexate is a useful and well-tolerated therapy with considerable steroid-sparing effect in patients with pemphigus vulgaris. It may be considered a first-line adjuvant therapy in the treatment of this difficult disease.

Pulse therapy in pemphigus: report of 11 cases

In this study, five cases of pemphigus vulgaris and two cases of pemphigus foliaceus were treated with cyclophosphamide pulse therapy associated with prednisone, resulting in the need for a smaller maintenance dose of prednisone. In three cases of pemphigus vulgaris and one case of pemphigus foliaceus, dexamethasone and cyclophosphamide pulse therapy associated with prednisone helped the lesions to heal more rapidly. Neither treatment however prevented the recurrence of the disease. Amenorrhea, myelotoxicity and Stevens-Johnson syndrome were among the cyclophosphamide side effects. All the patients treated with prednisone experienced known side effects.

FRI0336 Medium doses of glucocorticoids are as effective as and safer than high doses of glucocorticoids in patients with giant cell arteritis

BackgroundAfter the advent of methotrexate, there are not comparative studies between different regimes of prednisone in giant cell arteritis (GCA). Due the complications of an excessive prednisone cumulative dose1, a...

Increased IL-17A expression in temporal artery lesions is a predictor of sustained response to glucocorticoid treatment in patients with giant-cell arteritis

BackgroundInterleukin 17A (IL-17A) exerts pivotal proinflammatory functions in chronic inflammatory and autoimmune diseases.ObjectiveTo investigate IL-17A expression in temporal artery lesions from patients with giant-cell arteritis (GCA), and its relationship with...

A Unique Case of Autoimmune Hepatitis Developing in a Patient with Kikuchi-Fujimotoʼs Disease

Purpose: We describe a 35 year old Hispanic woman with Kikuchi-Fujimoto's disease (KFD) who developed jaundice; abnormal liver function tests (LFTs) and was found to have developed autoimmune hepatitis (AIH). KFD (histiocytic necrotizing lymphadenitis) is a rare condition of unknown etiology first described in 1972 independently by Kikuchi and Fujimoto. It is a nonmalignant disease characterized by fever and cervical adenopathy and has a self-limiting course. This is the first reported case of a patient with histologically confirmed KFD developing AIH. One prior case report has described KFD occurring after AIH. A 35 year old Hispanic female was referred to our hepatology clinic due to complaints of worsening fatigue and jaundice for two weeks. Her past medical history was significant for KFD diagnosed 14 months ago via cervical lymph node biopsy. She was not on any treatment. On examination, the patient's vital signs were stable and she was afebrile. Abdominal examination was significant for mild tenderness in the right upper quadrant without any guarding or rigidity. Pertinent abnormal laboratory data included Aspartate aminotransferase/Alanine aminotransferase (AST/ALT) 1122/1247 U/L, Alakaline phosphatase 213 U/L, Gammaglutamyl transferase 216 U/L, total bilirubin 5.7 mg/dl, conjugated bilirubin 3.6 mg/dl, total protein 8.5 g/dl, albumin 3.3 g/dl, elevated Immunoglobulin G (IgG) level 2450 mg/dl and anti-smooth muscle antibody positive 1:640. The complete blood count, serum chemistry, viral hepatitis serologies, iron panel, ferritin, ceruloplasmin, alpha one antitrypsin, anti-nuclear antibody, anti-mitochondrial antibody and tissue-transglutaminase antibody were normal. Liver ultrasound was suggestive of mild diffuse fatty infiltration. A liver biopsy was performed which showed lymphoplasmacytic infiltrates, interface hepatitis, grade 4/4 inflammation, stage 2/4 fibrosis. The patient was diagnosed with AIH based on the clinical presentation, laboratory data and the biopsy results. The pre-treatment AIH score was 18. The patient was started on prednisone 60 mg orally daily for one week and there was significant improvement in LFTs. The steroid dose was tapered in the following weeks and the patient was noted to have complete normalization of LFTs and IgG by the fifth week of therapy. Azathioprine 75 mg daily was added with the tapering of the prednisone dose. Six months post-diagnosis, the patient is asymptomatic on a maintenance dose of prednisone 5 mg and azathioprine 75 mg daily. The coexistence of KFD and AIH is extremely rare in the reported literature. Our case is unique as it is the first reported case of histologically confirmed KFD who developed jaundice and was subsequently found to have AIH.

Mycophenolate mofetil for steroid-dependent nephrotic syndrome: a phase II Bayesian trial

Mycophenolate mofetil (MMF) has emerged as a new therapeutic option in steroid-dependent nephrotic syndrome (SDNS). We conducted a phase II Bayesian trial of MMF in children with SDNS. Phase II trials, usually single-arm studies, investigate the effect of new treatments. Standard Fleming's procedure relies on observed results (relapse rate during the trial), whereas Bayesian approach combines observed results with prior information (expected relapse rate according to prior studies and clinical experience). All patients were required to have received prior alkylating-agent treatment. Sixty-seven percent of them had also received levamisole. Patients received MMF (1,200 mg/m(2)/day) and prednisone according to a defined schedule [reduction of alternate-day (e.o.d) dose to 50% of pre-MMF dose at 3 months, 25% at 6 months]. Twenty-four children (median age 6.0 years, 2.8-14.4) entered the study and 23 completed it. Bayesian analysis showed that adding four patients would not change significance of results, allowing stopping inclusions. Four patients relapsed during the first 6 months (estimated probability 17.6%, 95% credibility interval: 5.4-35.0%) and two at months 8 and 11.5. In the 19 patients free of relapse during the first 6 months, median (Q1-Q3) prednisone maintenance dose decreased from 25 (10-44) to 9 (7.5-11.2) mg/m(2) e.o.d (p < 0.001) and cumulative dose from 459 (382-689) to 264 (196-306) mg/m(2)/month (p < 0.001) before and on MMF respectively. Pre-MMF patient characteristics and MMF pharmacokinetics did not differ between patients with or without relapse. MMF reduces relapse rate and steroid dose in children with SDNS and should be proposed before cyclosporine and cyclophosphamide.

Necrotizing sarcoid granulomatosis: a case report and review of progresses in this disease

We present the case of a young male who visited the emergency room with progressive dys pnea and left sided chest pain. A chest X-ray was performed which showed a pattern of diffuse nodular and reticular opacities. The diagnosis of Necrotizing Sarcoid Granulomatosis (NSG) was made by histopathological examination of a lung biopsy specimen and by exclud ing other causes of granulomatous disease. He was treated with corticosteroids. Because of persistent extrapulmonary (ocular) involvement a maintenance dose of prednisone was needed. Necrotizing Sarcoid Granulomatosis is a granulomatous disease with necrosis and vasculitis of unknown cause. The resemblance between NSG and (nodular) sarcoidosis is strong, but subtle differences exist. The prognosis is good and therapy is usually not necessary. However in some cases there is a need for therapy with corticosteroids, depending on the involved organs and/or the degree of impairment.

17: Renal Allograft Survival Among Patients with Lupus Nephritis

RENAL ALLOGRAFT SURVIVAL AMONG PATIENTS WITH LUPUS NEPHRITIS Muhammad Ashraf, Nagaraju Sarabu, St. Luke’s Hospital, Chesterfield, MO. Daniel C. Brennan, Washington University, St. Louis, MO, USA. The incidence of Systemic Lupus Erythematosus (SLE) in the US is 1 in 2000. Half will develop lupus nephritis and a quarter of these will develop end-stage renal disease (ESRD) requiring dialysis or transplantation. We wished to determine the rate of transplantation of patients with SLE and prognostic factors affecting rejection or graft failure/return to dialysis. We reviewed the electronic and paper medical records of all patients with SLE transplanted at our institution between January 1970 and June 2007 and performed survival analysis on the data available. There were 2891 kidney transplants performed, 82 (2.8%) had SLE. 68 had sufficient data to determine the time to rejection. 43 (63.8%) showed signs of rejection and 25 (36.2%) were censored (either lost to follow-up or never rejected). Mean time to first rejection after transplantation was 2920 days (95% confidence intervals; 1969, 3871). 71 recipients had sufficient data to assess return to dialysis. 19 (26.8%) returned to dialysis and 52 (73.2%) were censored (lost to follow-up, never resumed dialysis or died). Mean time to graft failure and return to dialysis was 6812 days (95% confidence intervals; 5273, 8350). Significances of log rank tests for the Kaplan Meier curves of variables of interest are: Race (Caucasian vs. African American; p = 0.004), sex (p = 0.34), blood group (O vs. others; p = 0.654), HLA A mismatch (none vs. A1 or A2 mismatch; p = 0.02), HLA B mismatch (none vs. B1 or B2; p = 0.01), HLA DR mismatch (none vs. either DR1 or DR2; p = 0.11), maintenance prednisone dose ( 5mg; p = 0.97) In conclusion, patients with SLE can receive kidney transplants and have reasonable rates of rejection and excellent survival. Race and type of HLA mismatch affected the over all survival in this study.

Bilateral occlusive retinal vasculitis in Kikuchi–Fujimoto disease

A 22-year-old Chinese woman noted a sudden decrease in vision in both eyes 3 days after being histologically proven Kikuchi-Fujimoto disease. Fundus fluorescein angiography of both eyes showed multiple focal areas of retinal arteriolae occlusion with adjacent areas of capillary non-perfusion and leakage from the dilated retinal vessels in the posterior pole and midperiphery. During the follow-up period of 7 months, the patient was treated with intravenous methotrexate and a low maintenance dose of prednisone. Occlusive vasculitis resulted in proliferative retinopathy in both eyes and there were no other systemic abnormal findings. Autoimmune event was considered as the possible pathogenesis of occlusive retinal vasculitis.