High-dose Corticotherapy Research Articles

A Case of Unremitting Cellulitis in a Lung Transplant Recipient: What is the Infectious Etiology?

<h3>Introduction</h3> <i>Mycobacterium chelonae</i> is a rare, non-tuberculous mycobacterium that is highly pathogenic in immunosuppressed patients. Herein, we describe a chronic presentation of cellulitis which was later biopsy-proven as <i>M. chelonae</i> in a lung transplant recipient. <h3>Case Report</h3> A 61-year-old female bilateral lung transplant recipient presented 15-months post-transplant with multiple erythematous cutaneous lesions of the right arm. She had a 3-month history of waxing and waning cutaneous lesions that had not fully cleared despite antibiotic therapy. The first transplant year was eventful with two episodes of acute cellular rejection necessitating high-dose corticotherapy. Dermatological biopsy performed in a prior visit demonstrated features consistent with dermatitis. Autoimmune etiologies were ruled out, and the patient reported no history of trauma or other nidus of entry for infection. At the time of the current presentation, we observed nodular changes to the cutaneous lesions, which necessitated repeat biopsy, revealing granulomatous infiltrate (Figure 1). Acid-fast staining was positive, and <i>M. chelonae</i> was properly identified. Given her immunosuppressed state, the patient was started on multidrug antibiotic therapy, guided by antibiogram. Skin showed some improvement on observation, however, one large, nodular cutaneous lesion persisted. Decision was made to perform excision in effort to reduce bacterial growth burden. Subsequent biopsy findings were negative, likely a result of immunological phenomenon. She ultimately finished multidrug treatment with complete resolution of skin nodules. <h3>Summary</h3> This case underlines the role of corticotherapy and immunosuppression as a major risk factor for atypical infections, such as <i>M. chelonae</i>, in lung transplant recipients. Eradication can be difficult as demonstrated, and thus early recognition and multidrug antibiotic therapy are the cornerstone for management in this population.

Henoch-Shonlein purpura in adults in South Tunisia: a series of 14 cases

Le purpura rhumatoïde (PR) est une vascularite des petits vaisseaux secondaire à des dépôts d'immunoglobulines de type IgA. À travers une étude rétrospective colligeant 14 cas de PR de l'adulte en se basant sur les critères de classification du PR de EULAR/ PRINTO/PRES durant une période de 18 ans (1996 à 2014) dans le service de médecine interne du CHU Hédi Chaker de Sfax dans le sud tunisien, les caractéristiques épidémiologiques, cliniques, thérapeutiques et évolutives du PR chez nos patients adultes seront précisées. L'âge moyen des patients était de 33 ans (extrêmes: 17-64 ans) avec une prédominance masculine (sex-ratio de 1.8). Le purpura vasculaire pétéchial était constant. Les manifestations articulaires (arthralgies et/ou arthrites) étaient notées chez 9 patients (64,2%). L'atteinte digestive était présente chez 13 patients (92.8%). L'atteinte rénale était notée chez 11 patients (78.5%) révélée par une protéinurie de rang néphrotique dans 7 cas, une hématurie microscopique dans 9 cas, une hypertension artérielle dans 4 cas et une insuffisance rénale dans un cas. Le type histologique le plus fréquent était la glomérulonéphrite proliférative endocapillaire diffuse (36.3%). La corticothérapie à forte dose était instaurée chez 7 patients ayant une atteinte rénale proliférative initiée par des bolus de solumédrol dans 4 cas et associée au cyclophosphamide dans un cas. Deux patients avec une atteinte digestive sévère ont reçu une corticothérapie. Après un suivi moyen de 18,5 mois (4-36 mois), l'évolution était favorable dans tous les cas sans rechutes et l'insuffisance rénale chronique était notée dans un seul cas.

Hypereosinophilic Syndrome of Undetermined Significance – Difficulties in Clinical and Therapeutic Approach

Abstract We report a case of a 69-year-old woman who is followed since seven years for persistent blood hypereosinophilia up to 5100/mmc. She has been extensively investigated for other diseases known to induce hypereosinophilia, including allergies, parasitic infections and neoplasia. No end-organ dysfunction could be confirmed. We considered a possible primary hypereosinophilic syndrome (HES) and determined the genetic mutation FIP1L1-PDGFRA characteristic for HES, which was negative. Bone marrow showed reactive eosinophilia with no malignant cells and rare mast cells, less than 15 in aggregates, which is the major criterion for diagnosing mastocytosis. Knowing the association between HES and mastocytosis, we measured and found high serum tryptase levels and positive c-kit D816V genetic mutation, characteristic for systemic mastocytosis. The patient was closely monitored, with regular hematologic and clinical evaluation, mainly for cardiac and neurologic manifestations. A short trial of high dose corticotherapy induced remission of hypereosinophilia, but this could not be maintained with lower doses. The clinical outcome during follow-up period was rather good, except mild cognitive decline and atrial fibrillation.The reported case is illustrative for versatile presentation and difficulties in management of hypereosinophilia in clinical practice.

THE PREVALENCE OF HEPATITIS B REACTIVATION (HBV) IN CANCER PATIENTS TREATING WITH CHEMOTHERAPY

ackground and Objectives: Nowadays, the incidence of cancer is constantly increasing in the world as well as in Vietnam. The treatment of cancer is based on multimodality principle. Among those principal modalities, chemotherapy is widely used for different purposes such as neoadjuvant, andjuvant and palliation. However, chemotherapy can induce activation of latent infections, including hepatitis B. Vietnam is in the endemic region of hepatitis B so the reactivation of hepatitis B on cancer patients with chemotherapy has emerged a concerned problem. However, few interests were gained on this problem in the aspect of clinical setting or researching. Aims: to determine the prevalence of hepatitis B reactivation (HBV) in cancer patients treating with chemotherapy and to detect some risks factors of this situation. Subjects and methods: descriptive prospective. The study included 33 cancer patients with inactive HBV infection who are treating with chemotherapy. We define HBV reactivation by ALT &gt; 3 ULN and HBV DNA copies &gt; 10 positive control limit. Results: We found 6 patients with reactivated HBV, accounting for 18.18 %. Among reactivated HBV patients, age less than 60 accounts 83,33%. Rate of reactivated HBV in males was 25,00% while this rate in females was 14,28%. Rate of reactivated HBV in lymphoma, lung cancer and breast cancer was 33,33%, 25% và 22,22% respectively. Clinial manifestation of reactivated HBV includes jaundice (33,33%), fulminant hepatic failure (6%) and death (5%). The reactivated rate was higher in patients got high dose of corticoid (28,57%) vs low dose (15,38%). This rate was 29,41% in patients treated with anthracyclines which was higher than in group without anthracyclines. The reactivated rate of HBV was dramatically higher in patients treated with rituximab (75%). Conclusion: the reactivation of hepatitis B on cancer patients with chemotherapy is common. We found 6 patients with reactivated HBV of 33 subjects of the study which accounts 18.18 %. We recognized that reactivated HBV rate was higher subgroups of age &lt; 60 years old, males, patients with lymphoma, lung cancer, breast cancer. Reactivated HBV may be more prevalent in patients with high-dose corticotherapy, anthracyclines and Rituximab. Key words: HBV reactivation, chemotherapy, cancer, hepatitis B

Severe Axonal Peripheral Polyneuropathy Revealing a Systemic Lupus Erythematosus: About One Case

Aim: We report a case of acute and severe sensorimotor peripheral polyneuropathy (with a severe motor damage) revealing a lupus. Case Presentation: A 48-year-old female patient was interned in rheumatology for a chronic polyarthritis. Four days after her hospitalisation, she was presenting a flask distal and proximal tetraparesia, with rapidly progressive installation. Electromyogram showed severe acute axonal sensorimotor polyneuropathy. The antinuclear antibody was positive as the anti-ds-DNA antibodies. The evolution has been unsatisfactory despite the high-dose corticotherapy and the immunosuppressor. Conclusion: Even if it is rare, peripheral neuropathy can be a lupus discovery circumstance.

THU0116 Efficacy of Glucocorticoids for Early Rheumatoid Arthritis (RA): A Meta-Analysis of Randomised Controlled Trials

BackgroundGlucocorticoids are among the most used and most discussed treatment option for early RA.ObjectivesThe aim of this study is to evaluate the structural and clinical efficacy of corticotherapy (CT) for...

THU0142 Safety of Glucocorticoids for Early Rheumatoid Arthritis: A Meta-Analysis of Randomised Controlled Trials

BackgroundSeveral studies have shown structural and clinical benefit of corticotherapy (CT) for 1 to 2 years treatment of recent-onset rheumatoid arthritis (RA). However, because of its numerous adverse effects (AE),...

Multisystem sarcoidosis in a patient on interferon-α therapy for chronic hepatitis C

Sarcoidosis is a chronic multisystemic granulomatous disease that is triggered by an autoimmune process. Nowadays, this pathology represents a well-recognized but uncommon complication for antiviral treatment in hepatitis C virus (HCV) infection. Herein, we report a remarkable case of 47-year-old woman treated for chronic HCV infection; the patient has developed interferon alfa-induced sarcoidosis involving the central nervous system. The evolution was fatal despite disrupting the antiviral therapy and initiating a high-dose corticotherapy. This complication of interferon alfa treatment was reported in the literature in only one case. Through this case and a review of the literature, we aim to underline the importance of screening for sarcoidosis before and during the follow-up of HCV patients undergoing antiviral therapy.

New treatment options for lupus &amp;ndash; a focus on belimumab

Belimumab is the first biologic approved for patients with systemic lupus erythematosus (SLE). Belimumab is the first of a new class of drug targeting B cell-stimulating factors or their receptors to reach the market. Its target, BLyS, also known as BAFF (B cell-activating factor from the tumor necrosis factor family), is a type II transmembrane protein that exists in both membrane-bound and soluble forms. Additionally to a robust rational from murine experiments conducted in lupus prone mice, BLyS circulating levels are increased in SLE patients. After the negative results of a Phase II trial, two Phase III trials met their primary endpoints. Some SLE patients are still refractory to the standard options of care or necessitate prolonged high-dose corticotherapy and/or long-term immunosuppressive regimens. However, some experts still feel that the effect of this biologic might not be clinically relevant and blame the use of the new systemic lupus response index as well as the discrepancies between both trials and the noninclusion of the severe form of the disease as nephritis. In this review, we aim to discuss the characteristics of belimumab, critically evaluate the different steps of its development, and consider its future place in the arsenal against SLE, taking into account the patients’ perspectives.

On the mean residual lifetime of consecutive k-out-of-n systems

Acquired haemophilia A is a very rare (1-2 cases per million people) but often life-threatening haemorrhagic disorder characterized by antibodies directed against coagulation factor VIII. We report the case of a 55-year old woman under treatment with Pegylated alpha 2a interferon (IFN) and Ribavirin for chronic viral C hepatitis, who developed a progressive severe haemorrhagic syndrome diagnosed as acquired haemophilia based on supplementary laboratory data (prolonged activated partial thromboplastin time, extremely low factor VIII level - 1%, high titre of factor VIII inhibitor - 30 Bethesda U/ml).The onset was insidious, about three months before presenting to our unit. Antiviral therapy had been stopped three weeks before current admission. Emergency intensive treatment included: haemostatic agents - rFVII (Novoseven), FEIBA (Factor VIII Inhibitor Bypassing Activity), vitamin K, adrenostazin, cryoprecipitate, fresh frozen plasma, as well as immunosuppressive therapy (high dose corticotherapy and cyclophoshamide), immunoglobulins (Humaglobin), prophylactic PPI and antibiotics. The evolution was slowly favourable with the remission of the haemorrhagic syndrome and regression of the iliopsoas muscle haematoma. Clinicians should be aware that acquired forms of haemophilia do exist, representing a rare diagnosis and a therapeutic challenge. To our knowledge, this is the first reported case of acquired haemophilia in Romania, in a patient with chronic viral C hepatitis under antiviral treatment.

Nonlethal, attenuated, transfusion‐associated graft‐versus‐host disease in an immunocompromised child: case report and review of the literature

Transfusion-associated graft-versus-host disease (TA-GVHD) is a rare complication of transfusion of nonirradiated blood components. It usually affects children in high-risk groups, including those who have primary immunodeficiencies (PIDs). It usually presents with skin, hepatic, digestive, and hematologic involvement and is normally fatal. We report the case of a nonlethal, attenuated, TA-GVHD in a 7-month-old boy. The disease was marked by the presence of a severe rash but lacked all the other usual manifestations. We speculate that the unusually benign course of this disease, which has normally a fulminant course, was due to the fact that this child was under high-dose corticotherapy at the time of the engraftment. This fortunate coincidence led to the survival of this child and allowed the diagnosis of a combined immunodeficiency. A high index of suspicion is required for the diagnosis and proper management of PID. The administration of nonirradiated blood components in the first year of life, sometimes before the clinical suspicion of a PID, is of great concern. TA-GVHD may be more prevalent than reported in the literature and it is possibly a nonidentified cause of death in recipients with unexplained death and nondiagnosed PID.

Une luxation bilatérale postérieure des épaules sur ostéonécrose des têtes humérales

We report a case of simultaneous bilateral posterior shoulders dislocations in a 46-year-old male with antecedent of high-dose corticotherapy. The mechanism was non-traumatic after a contraction of the sub scapularis muscle in internal rotation. The interscapular pain was not initially diagnosed although a tomodensitometry was realized to eliminate a cardiovascular emergency. After reduction, the shoulders were unstable and the MRI showed an osteonecrosis of the humeral heads. The patient underwent surgery with an iliac spongy bone graft in the humeral nick. At 3 months, there was no recurrence and mobilities were good. Bilateral posterior shoulders dislocations are unusual and it is the first case of non-traumatic dislocation. Aetiology are often epilepsy, electrocution, and extreme traumatism. Diagnosis is often misrecognized and the treatment is not well codified.

S234 – The Immune System in Immunomediated Inner Ear Disease

Objectives 1) To assess the different clinical varieties and the evolution of the patients. 2) To assess the clinical impact of the use of high dose corticotherapy. 3) To analyze the phenotype of the lymphocyte populations in peripheral blood. 4) To study the proliferative answer of mononuclear cells in peripheral blood under polyclonal mitogen stimulus. Methods A prospective study in patients with immunomediated inner ear disease was carried out, dividing them in 4 different clinical presentations: Sudden deafness, fluctuant hearing loss, Meniere's disease, and rapidly progressive hearing loss. We analysed 22 patients, controlling their clinical evolution and response to corticotherapy. We also made an immunophenotypical and functional characterization of the T lymphocytes in patients before and after treatment and comparing them with healthy controls. Mann-Whitney U and Wilcoxon range tests were used as statistical resources. Results 59.09% of the patients treated with corticotherapy improved, with a 95% of treatment fulfillment. The T lymphocyte compartment's proliferative response was significantly lower in these patients (p value less than 0.05) and there was a higher CD56+ (p value less than 0,01) and CD8+ CD45RO+(p value less than 0.01) lymphocyte subpopulations expression in absolute and percentage numbers. Conclusions Immunomediated inner ear disease is related to T, B, and especially NK lymphocytosis associated to a redistribution of the T cell subpopulations.

Budd–Chiari syndrome associated with Behçet's disease

Budd-Chiari syndrome is a rare and serious complication of Behçet's disease, and is the result of occlusion of the major hepatic veins, the adjacent inferior vena cava, or both. The aim of this study was to determine the prevalence, clinical and laboratory findings, and treatment and clinical course of Budd-Chiari syndrome associated with Behçet's disease. We analyzed retrospectively the charts of 220 patients fulfilling the international diagnostic criteria of Behçet's disease. From them, we selected those with Budd-Chiari syndrome, and analyzed their epidemiological and clinical imaging features and outcomes. Seven male patients, mean age 29 years and already diagnosed with Behçet's disease, had Budd-Chiari syndrome. The clinical course was from subacute to chronic in all cases. Thrombosis of hepatic veins was associated with inferior vena cava thrombosis in six cases. Four patients had other venous thromboses (superior vena cava and lower limbs) and one also had pulmonary emboli. One patient was positive for anticardiolipin antibodies. All patients had anticoagulation therapy, and six had high-dose corticotherapy associated, in two cases, with monthly cyclophosphamid intravenous pulses. Clinical outcome was favourable in six cases, and one patient died of hepatic failure. The prevalence of Budd-Chiari syndrome in patients with Behçet's disease is 3.2%, confirming that this syndrome is not uncommon in Behçet's patients. The inferior vena cava is frequently involved in combination with hepatic veins and often associated with other venous thrombosis. The prognosis may be favorable with medical interventions, including anticoagulation, treatment of the vasculitis and the use of diuretics when required.

An Update on the Diagnosis of Adrenal Insufficiency and the Use of Corticotherapy in Critical Illness

To examine recent literature regarding corticotherapy in critically ill patients suffering from sepsis, acute respiratory distress syndrome (ARDS), and severe community-acquired pneumonia (SCAP). Literature was identified through MEDLINE (1966-April 2007) using combinations of the key words hydrocortisone, adrenal insufficiency, acute respiratory distress syndrome, pneumonia, sepsis, and cortisol. Bibliographies of relevant articles were reviewed for additional citations. Presentations at recent critical care meetings were also incorporated. Articles were chosen based upon their relevance to the topics covered. Earlier studies using high-dose corticotherapy in the intensive care unit have shown treatment to be ineffective. Recent studies using extended courses of low-to-moderate doses of steroids have found favorable results; however, these results must be interpreted with caution due to limitations in the data. One trial of steroids in septic shock found a survival benefit in patients who failed to increase their baseline cortisol by greater than 9 microg/dL in response to adrenocorticotropic hormone, but these results were not reproduced in a subsequent Phase 3 trial. Recently, inaccuracies in measuring cortisol have been identified, making interpretation of cortisol concentrations difficult. A large-scale study failed to confirm a previously reported mortality benefit of corticotherapy in late ARDS, but preliminary data suggest a role for steroid treatment in early ARDS. Finally, a pilot study has found that hydrocortisone lowers morbidity and mortality in SCAP. Corticotherapy may be beneficial to some patients with sepsis. The decision to administer steroids in sepsis cannot be based on biochemical markers of adrenal function; rather, treatment should be considered in septic patients with vasopressor refractory hypotension. Although preliminary evidence suggests a role for steroids in early ARDS and SCAP, there are not enough data to suggest routine administration of steroids in these conditions. Additional studies are needed to assess corticotherapy in the critically ill.

Découverte d’une infestation à Anguillule, 25 ans après retour de zone d’endémie, à l’occasion d’une corticothérapie pour traumatisme oculaire

In the field of ophthalmology, indications for high-dose corticotherapy are various. This paper reports the case of a Caledonian man who presented with intestinal strongyloidiasis, discovered 25 years after he had left the endemic area. A checkup before corticotherapy for traumatic retina edema provided the diagnosis of the infection. The authors emphasize the importance of searching for Strongyloidiasis stercoralis larva before initiating corticotherapy, as it is the main treatment responsible for parasitic dissemination. The most severe form of strongyloidiasis, the disseminated form, has a high mortality rate: 70%-90%. The definitive diagnostic test is enhanced larva recovery, which should be proposed to every patient returning from endemic area, people with precarious hygiene or with high eosinophilia or intestinal symptoms of chronic infection. Delay in diagnosing strongyloidiasis frequently results in death, despite vigorous treatment.

Symptomatic Radionecrosis after AVM Stereotactic Radiosurgery. Study of 16 Consecutive Patients.

The purpose of our study was to analyze the outcome of symptomatic radionecrosis following stereotactic radiosurgery for brain arteriovenous malformations. Of 225 patients treated by linear accelerator radiosurgery for brain AVM, 16 (7,1%) presented post-radiosurgery symptomatic radionecrosis on a mean follow-up period of 50 months (range 1-123 months). Once diagnosed with radionecrosis, 14 of 16 patients were subjected to high dose corticotherapy consisting of escalating doses of dexamethasone for several weeks. The mean interval of occurrence of new symptoms was 11.6 months post-radiosurgery (range 6-20 months). The mean time of follow-up was 2.9 years post radiotherapy ranging from seven months to eight years. Of the 16 patients with symptomatic radionecrosis, 11 (68,75%) showed complete resolution of symptoms while five (31,25%) showed improvement but still presented a neurological deficit at the closing date of the study. At the closing date, 11 patients (68.75%) had angiographically completely obliterated arteriovenous malformations while another two patients had an obliteration of 95% to 98% and one patient had a 98% obliteration with development of a new contralateral AVM. In our series, symptomatic radionecrosis occurred in 7.1% of patients treated with stereotactic radiosurgery for brain AVM. These patients where subjected to a prompt, high dose corticosteroid treatment and most presented symptom resolution or improvement with a fair obliteration rate, offering protection from bleeding. Permanent neurologic deficits attributable to radionecrosis occurred in 2.2% of our patient population treated with stereotactic radiosurgery for brain AVM.

La sarcoïdose chez l’enfant, difficultés diagnostiques : à propos d’une observation

Sarcoidosis is rare in children and may present misleading appearances. Authors report the case of 13-years-old child explored for prolonged fever, inflammatory syndroma and skin lesions. Abdominal ultrasound showed hepatomegaly, mesenteric lymphadenopathy and splenic nodule. Chest CT scan demonstrated bilateral hilar adenopathy. Histology of inguinal adenopathy concluded to a caseo-follicular tuberculosis. Sarcoidosis was suspected after tuberculous treatment failure, persistent high fever, negative tuberculin test and skin biopsy results. This diagnosis was reinforced by bronchoalveolar lavage results, high level of angiotensin converting enzyme and histological control. Favourable evolution was noted with a high dose corticotherapy. Sarcoidosis, though rare in children, should be suspected when prolonged fever is associated with important inflammatory syndroma.

Les surdités brusques idiopathiques

Purpose. – Sudden idiopathic deafness is a sensorineural hearing loss with no recognized causes at the time of onset. The impairment site is usually localized in the cochlea, but some cases of retrocochlear lesions (e.g., cerebellopontine angle tumors, degenerative neural diseases, neuraxial ischemic lesions) can induce sensorineural deafness. The medical management of patients presenting with sudden deafness aims at detecting a causal mechanism, and at administering emergency therapeutic drugs. The diagnosis of idiopathic sudden deafness can be definitely made when no causes are found. Usually, the impairing mechanism involves the cochlea. The pathophysiology of this sensorineural alteration is still unknown. It is most likely that several mechanisms are associated together, their common point being an impairment to the feedback loop of the organ of Corti. Current knowledge and key points. – It is very likely that reactivation of neurotropic viruses and/or cochlear ischemia are frequent etiologies. Whatever the cause, the treatment is to be administered urgently, and consists of a high-dose corticotherapy at the least. Other treatments have never really proven to be effective. It is secondarily checked that no retrocochlear pathological processes, such as a cerebellopontine angle tumor, is present, in particular in young people. Future prospects and projects. – One of the current objectives is to determine when cochlear ischemia is involved, in a mini-invasive manner, such as with laser Doppler flowmetry, so that the treatment can be optimized. From a therapeutic point of view, early acoustic protection has been proven to be effective in cases of cochlear ischemia in small laboratory animals. Its efficacy in case of sudden deafness, non-exclusive of other causes than ischemia, is being assessed in a multicentric project.

Relapse of Wegener’s granulomatosis. Concerning a case after 20 years of remission

Immunosuppressive drugs have transformed the prognosis of systemic Wegener’s granulomatosis. Nowadays, the main residual problem is illness relapses, for which management is largely undefined. We describe the case of a patient, aged 47 in 1977. The diagnosis of Wegener’s granulomatosis was made when faced with polyarthralgias, cutaneous vasculitis, rhinitis, dyspnea, hemoptysis and global decline of her physical condition. The treatment associated high-dose corticotherapy and intramuscular cyclophosphamide for 1 year. This treatment led to a complete remission. Twenty years later, the patient was hospitalized for reoccurrence of rhinitis, dyspnea and right knee effusion associated with biological inflammatory syndrome, renal insufficiency and antibodies against polymorphonuclear neutrophil cytoplasm, type c-ANCA. Chest CT-scan disclosed parenchymal infiltrates. Wegener relapse was diagnosed and the combination of three methylprednisolone perfusions followed by oral prednisone (1 mg/kg/d) and a monthly bolus of cyclophosphamide led to a new remission. Nevertheless, 4 months after beginning the treatment the patient died from an infectious complication ( Pneumocystis carinii and aspergillosis). Relapses of Wegener’s granulomatosis are frequent and difficult to predict. Moreover, some cases occur very early. The remarkable efficiency of cyclophosphamide to induce remission is however shaded by the high rate of relapse. Other drugs are studied to identify more efficient therapy, able to both induce remission and prevent relapses, but reliable data are still missing to determine the best therapeutic regimen.