High-dose Oral Steroids Research Articles

Rituximab in refractory Vogt–Koyanagi–Harada disease

IntroductionVogt–Koyanagi–Harada (VKH) prognosis depends on early recognition and treatment; chronic disease may be developed when either delayed or inadequate treatment is performed, whereas other cases despite correct treatment are refractory to different drugs and also become chronic. We report a case of refractory VKH controlled with rituximab treatment.Case reportA 41-year-old female with painful visual loss and headache was examined. (VA 0.4 in RE and hand movements (HM) in LE). Retinal examination demonstrated multiple serous retinal detachments in both eyes. High-dose oral steroids were started, followed by progressive tapering of prednisone. New acute anterior and posterior relapses were achieved, and other immunommodulators were progressively added—new high-dose steroid treatment, adalimumab, cyclosporine, and methotrexate—but patient had new anterior and posterior recurrences associated with tinnitus and headache. Thus, an infusion of 1 g of rituximab was administered after 15 months follow-up; the VA was 0.2 in RE and counting fingers in LE. Three additional doses of 1 g each were administered 1, 6, and 16 months later. We have achieved a final VA after 34 months follow-up of 0.2 in RE and HM in LE, with definitive control of inflammation, without acute relapses since rituximab was administered.ConclusionAfter searching PubMed/Medline, this is the first report of VKH disease treated with rituximab. Additional studies are warranted to confirm the efficacy of this new approach for inflammatory control in refractory cases of VKH disease.

Occult Temporal Arteritis in a 54-Year-Old Man

A 54-year-old white man with a remote history of pars planitis reported transient monocular visual loss (TMVL) in the left eye on standing. The following week he experienced multiple similar episodes. He denied associated systemic symptoms. Initial examination showed old peripheral retinal vascular sheathing and delayed retinal arterial filling time. Complete blood count, erythrocyte sedimentation rate, and MRI studies of the head and neck were normal. One week later, there were multiple cotton wool spots in the posterior pole, a relative afferent pupillary defect, and subtle visual field loss in the left eye. Evaluation for infectious, inflammatory, or embolic etiologies was nonrevealing. Biopsy of the prominent but nontender temporal arteries showed granulomatous inflammation, fragmentation, and duplication of the internal elastic lamina consistent with the temporal arteritis (TA). Radiography and MRI of the chest revealed dilation of the ascending aorta. The patient began treatment with high-dose oral steroids with resolution of his TMVL and retinal cotton wool spots and decrease in the size of the temporal arteries. Our case demonstrates the importance of considering TA in the setting of TMVL, visual loss, cotton wool spots, or dilated nontender temporal arteries in an otherwise asymptomatic patient even with normal inflammatory markers. Long-term follow-up is essential in unusual cases such as this one, given the high risk of ocular and systemic morbidity with TA.

Headache in a 27-year-old man: Bilateral serous retinal detachment

A 27-year-old man with a complaint of headache, tinnitus, and visual obscuration presented to a neurologist. Neurologic evaluations, including MRI and CT scan, were within normal limit. The prescribed medicines were propranolol, sumatriptin, valproate sodium, and dexamethasone. Ophthalmic examination was associated with reduction of visual acuity of eyes, bilateral uveitis, and serous retinal detachment. The most probable diagnosis was Vogt-Koyanagi-Harada (VKH) disease. Prescribing high-dose oral steroid and acetazolamide-improved systemic and ocular symptoms. Although HLAB5 is positive in Behηet disease, it also may be seen in VKH.

An Unusual Presentation Of Sarcoidosis With Progressive Hearing Loss

ObjectiveWe present a case report of a 52 year old female in whom the first and only presenting symptom of sarcoidosis was hearing loss. Neurosarcoidosis is a rare condition and published data are limited to case reports and small case series. A small number of cases with hearing loss as an initial symptom have been described, however all have also had other neurological disturbances. Case ReportThe patient presented to emergency outpatient clinic with sudden deterioration in hearing but was otherwise asymptomatic. Initial investigations suggested a diagnosis of ossicular fixation, however, her hearing rapidly and progressively deteriorated over the following months. Further investigation revealed raised serum angiotensin converting enzyme and anti-nuclear factor and suggested a diagnosis of sarcoidosis, however, there was no discrete lesion to make a tissue diagnosis. Treatment with high dose oral steroids failed to improve her symptoms, the efficacy of which has been debated in the literature. ConclusionThe Otolaryngologist should consider an autoimmune cause in any patient with unexplained hearing loss.

025 Guest Lecture

025 Guest Lecture

Central nervous system histoplasmosis in an immunocompetent patient

Histoplasma capsulatum is endemic in parts of Africa, Asia, the United States, and Latin America. Most infections are benign and self-limiting, but disseminated disease can occur in immunocompromised patients, particularly in the setting of HIV infections [3]. Central nervous system (CNS) involvement is found in 5–10% of cases of disseminated histoplasmosis [6], but isolated CNS histoplasmosis is rare. Here we present an unusual case of recurrent granulomatous disease of the CNS and epiglottis in an immunocompetent adult due to histoplasmosis. A 46-year-old Ugandan woman, who came to England in her teens, presented in 2001 with progressive headaches, vomiting, meningism and confusion. Recent travel history was limited to North American and European cities. Chest X-ray and MRI of the brain were normal. Cerebrospinal fluid (CSF) examination revealed 350 lymphocytes, 1.19 g/l protein and 2.3 mmol/l glucose (6 mmol/l plasma), and negative culture. She was treated for probable tuberculous meningitis with dexamethasone and anti-tuberculous therapy for 12 months. Her condition improved significantly although repeat MRI of the brain in 2002 showed abnormal leptomeningeal enhancement around the pons and occipital sulci. She developed hydrocephalus in 2003, necessitating insertion of a ventriculo-peritoneal shunt. She remained well until 2004 when she developed dysphonia. Laryngoscopy detected an epiglottic mass with non-caseating granulomata on biopsy. No mycobacteria were seen. Two subsequent biopsies confirmed the findings. Whole body fluorodeoxyglucose PET in 2005 revealed increased laryngeal uptake. She received courses of high dose oral steroids for probable epiglottic sarcoidosis with partial clinical response. She presented to us in December 2006 with worsening dysphonia, spastic paraparesis, sensory deficit to the T8 level and urinary retention. Blood tests including inflammatory markers, autoantibodies, angiotensin converting enzyme and HIV serology were normal or negative. MRI showed diffuse meningeal enhancement around the brainstem and entire spinal cord but no intramedullary lesions. CSF examination showed normal opening pressure, 44 mononuclear cells, 2.5 mmol/l glucose (8.1 mmol/l plasma), 3.44 g/l protein, and intrathecal synthesis of oligoclonal bands. She was treated empirically with high dose steroids and anti-tuberculous therapy (rifampicin, isoniazid, pyrazinamide and ofloxacin). Her condition continued to decline with ascending weakness and sensory deficit (T1 level). MRI in January 2007 showed more extensive meningeal enhancement and intramedullary nodules of enhancement in the cervical cord and medulla (Fig. 1). CSF examination showed 180 polymorphs, 0.2 mmol/l glucose (6.6 mmol/l plasma) and 12.3 g/l protein. CSF culture and PCR for mycobacteria were negative. Serum brucella and histoplasma antibodies and aspergillus precipitins were negative. Laryngeal biopsy showed non-caseating granulomata with no organisms. Protionamide was added for possible resistant tuberculosis. Her condition did not improve and intravenous liposomal amphotericin B was initiated to cover granulomatous fungal infection. Y. F. Tai (&) D. M. Kullmann R. S. Howard N. P. Hirsch T. Revesz S. M. Leary National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK e-mail: yen.tai@imperial.ac.uk

41-jährige Patientin mit ST-Hebungsinfarkt und multiplen arteriellen Embolien

A 41-year-old woman presented with acute angina in the emergency unit. Additionally, she reported pain in both legs and a weight loss of 5 kilograms within the last 10 days. ECG revealed an acute anterior myocardial infarction. However, immediate coronary angiography showed open arteries with minimal arteriosclerosis. A characteristic rise of cardiac enzymes together with an akinesis of the anterior wall and an adjacent mural thrombus was highly suggestive of a transient coronary thrombosis. Further investigations showed occlusion of multiple arteries in both legs and a splenic infarct. Although there was a typical risk profile including smoking, hyperlipidemia and regular estrogen medication, a further work-up was started. Urin analysis was decisive for the presence of proteinuria and a severe nephrotic syndrome. The definite diagnosis was made by direct biopsy of the kidney that revealed the characteristic findings of a minimal change glomerulopathy. Rapid remission could be induced by high-dose oral steroids. During routine work-up of coronary syndromes, especially in those with normal coronaries, rare but treatable causes of myocardial infarction and coagulopathy have to be thought of and should carefully be excluded.

A 41-Year-Old Woman With Severe Dyspnea and Painful Oral Mucosal Ulcerations

A 41-year-old woman with a 19 pack-year smoking history presented to her physician with a 2-month history of progressively worsening dyspnea. Pulmonary function testing revealed a severe obstructive ventilatory defect and air trapping with an FEV1 of 0.53 L (18% of predicted) and a residual volume of 4.09 L (254% of predicted). CT chest scan showed diffuse bilateral bronchiectasis and air trapping (Fig 1). She initially received a diagnosis of severe asthma and was started on high-dose oral steroids.

Spectrums of encephalopathy associated with antithyroid antibodies

Aim Illustrate encephalopthies associated with antithyroid antibodies through two cases. Case 1 A child with known hypothyroidism who presented acutely encephalopathic with deranged thyroid function who responded well to steroids and made a full recovery; previously described in adult cases. Case 2 13-year-old boy with acute, severe short-term and long-term memory loss, stroke like episodes and generalised seizures over the course of three different admissions in a 1-month period. He subsequently developed focal status epilepticus, unilateral hippocampus changes which progressed to bilateral on MRI. Antibody testing revealed a diagnosis of Hashimoto9s encephalitis. He had four cycles of plasmapheresis after immunoglobulin infusions, remains on high-dose steroids. The initial response to this treatment was excellent but after 6 months he continued to have episodes of deterioration in memory function and has started on retuximab for immunomodulation in addition to high-dose oral steroid. This combination of treatment has stabilised his memory function and he has now returned to school on a part-time basis. Conclusion The presentation of case 1 is well recognised in the literature in adult patients with established thyroid disease. In case 2 our patient developed focal status epilepticus which has not been recognised as a feature of Hashimoto9s encephalitis in the literature. He is also responding to a new form of immunomodulation medication which has previously never been described; most cases in the literature have used azathioprine as a second-line agent. Both patients had an encephalopathy related to antithyroid antibodies but their history, signs and symptoms highlight the spectrum of presentations that these patients can have as well as the varied clinical response to treatment. The authors feel presenting the above cases with an overview of current literature will aid clinical practice and would be grateful for the opportunity to highlight the spectrum if disease associated with antithyroid antibodies.

Orbital inflammatory disease secondary to a single-dose administration of zoledronic acid for treatment of postmenopausal osteoporosis

A 60-year-old woman received a single 5-mg dose of zoledronic acid (Aclasta) for treatment of postmenopausal osteoporosis. One day after receiving the drug, she acutely developed a painful periorbital swelling, chemosis, and hyperemia in the right eye. The condition worsened despite initial treatment with topical steroids. An orbital CT scan showed right eye proptosis, eyelid edema, and intraorbital fat stranding. A diagnosis of orbital inflammatory disease was made, and the patient was treated with high-dose oral steroids (prednisone 80 mg/day) tapered along 6 weeks. The symptoms and the swelling reduced progressively after initiating oral prednisone, and after 12 days, there was complete resolution of the condition. The patient remained symptom free and had no remission after the treatment interruption.

Successful treatment of hypertrophic pachymeningitis in refractory Wegener’s granulomatosis with rituximab

Central nervous involvement (CNS) is uncommon manifestation of Wegener's granulomatosis (WG). We are describing a patient with refractory WG with CNS involvement which responded to rituximab. This lady presented with nodular scleritis. Three months later, she developed headache, vision loss with complete opthalmoplegia, and relative afferent papillary defect. The brain MRI showed thickened dura along ant temporal region and enlargement of right cavernous sinus. C ANCA and PR 3 ELISA were positive. Non-contrast CT scan of orbit and paranasal sinuses showed soft tissue swelling of the right orbit along with mucosal thickening of right maxillary sinus. There were bilateral upper lobe nodules on HRCT of the chest. She responded to 3 days of methylprednisolone pulses and 1 g pulse cyclophosphamide but had multiple relapses while receiving high dose oral steroids and pulse cyclophosphamide. Then she was given four infusions of rituximab (375 mg/m(2)) at one-weekly interval. She had complete remission following rituximab. She relapsed after 6 months but again responded to repeat rituximab infusion.

Chorée aiguë post-streptococcique : une étrange réaction après chirurgie d’une insuffisance mitrale rhumatismale

We report on a 12-year-old patient from Congo who presented acute chorea following cardiac surgery for poststreptococcal mitral valvulopathy. She showed severe and asymmetrical chorea, associated with motor impersistence and agitation. Biological investigations disclosed inflammatory signs and brain MRI was normal. Due to the negative results of the biological and morphological investigations, the diagnosis of Sydenham chorea was suspected. High doses of oral steroids resulted in a dramatic improvement of the chorea as well as the behavior disturbance within 1 month. Sydenham chorea is not an unusual complication of rheumatic fever. Usually, patients develop chorea a few weeks after beta-hemolytic streptococcal pharyngitis. Details on its pathophysiology remain to be determined. Our case highlights its possible onset in the postoperative period if alternative etiologies of infantile chorea have been excluded.

Ménière's Disease: Medical Therapy, Why and When

Diagnosis of Ménière's disease (MD) is mainly clinical and based on symptoms of episodic vertigo, fluctuating hearing loss, tinnitus, and aural fullness. An autoimmune etiology should be considered for middle-age female patients with bilateral involvement. A positive response to a short course of high-dose oral steroids or intratympanic steroid perfusion may be helpful in diagnosing an autoimmune origin.

High Dose Oral Steroids Commonly Used to Treat Relapses in Canadian MS Clinics

Glucocorticoid treatment improves the speed of functional recovery of acute multiple sclerosis (MS) relapses but has not been shown to provide any long-term functional benefit. There is currently no convincing evidence that the clinical benefit is influenced by the route of administration or the dosage of glucocorticoid, or the particular glucocorticoid prescribed. Recent studies support similarities in the bioequivalence and in the clinical effect of high dose oral corticosteroids for MS relapses. This survey aimed to determine the relapse treatment preferences of clinicians in Canadian MS clinics. Members of the Canadian Network of MS Clinics are linked by an email server. A one page survey was distributed to the group to determine and report use of corticosteroids to manage MS relapses amongst Canadian MS specialists. Fifty-one clinicians from 17 MS clinics were surveyed. 32 (63%) surveys were returned representing 16 clinics. Five doses are most commonly prescribed, usually without a taper. Three or four doses and the use of a corticosteroid taper, however, are not uncommon. Gastric cytoprotection and sedatives are often prescribed for use as needed. This survey illustrates that when Canadian clinicians with expertise in managing MS treat MS relapses they choose high dose corticosteroids, either oral or i.v. The results therefore represent Canadian practice as these clinicians provide direct patient care and influence care by community neurologists. Until evidence clearly identifies a superior practice all options should be available to clinicians and their patients.

Posterior Ischaemic Optic Neuropathy after Intranasal Anesthetic Injection: Case Report with OCT Findings

A 42 year-old man underwent a nasal septoplasty and awoke with vision of light perception in his right eye. An afferent pupillary defect was noted with a normal fundus examination, normal fluorescein angiography and intact optic canal on imaging studies. A diagnosis of posterior ischaemic optic neuropathy was made. No visual improvement was noted in our case despite treatment with high dose oral steroids. Follow-up showed optic atrophy on fundoscopy and retinal nerve fiber layer thinning on OCT. Visual loss after nasal septoplasty surgery is extremely rare, with few cases reported in the literature. It can occur if the optic canal is fractured, but has been most commonly reported as a complication of intranasal anesthetic injections. Vigorous pressure during injection can overcome arterial pressure causing the injected material to circulate in a retrograde manner via arterial branches and cause ischaemia of the optic nerve. A further possibility is that intranasal injection could cause emboli to enter the optic nerve circulation. Physicians should be aware of this potentially blinding complication after intranasal anesthetic injections.

Susac's syndrome: Intratympanic therapy for hearing loss and a review of the literature

Two cases of Susac's Syndrome are presented with a discussion of treatment modalities for the associated severe sensorineural deafness. Patients were managed with high dose oral steroids, anticoagulants and immunosuppressive drugs. The addition of intratympanic methylprednisolone injections were added to one patient's regimen with initial improvement but subsequent relapse and progression to profound deafness. The management of Susac's syndrome is difficult and still in need of innovative methods as the standard treatment continues to be ineffective in the long term.

Conjunctival and episcleral Splendore–Hoeppli phenomenon

The Splendore-Hoeppli phenomenon originally described in 1908 is a rare pathological state with an as yet unknown cause. Reported is the Splendore-Hoeppli phenomenon present in both eyes of a 36-year-old woman. The pathology then proceeded to resolve itself completely within 10 weeks. Of note was the fact that the patient actually developed these granulomata despite being on high doses of oral steroids with the lesions disappearing despite her steroids being withdrawn during the resolution phase. An indication is that the phenomenon is unlikely to be because of an autoimmune response.

Recurrent posterior scleritis and orbital myositis as extra-intestinal manifestations of Crohn's disease: Case report and systematic literature review

Background Ocular episcleritis and uveitis are well-recognised extra-intestinal manifestations of Crohn's disease. Orbital myositis is rare: to our knowledge it has been associated with Crohn's disease in thirteen cases. Posterior scleritis, orbital myositis and Crohn's disease have been reported as coexisting in only two cases. Methods and results We describe a third case, that of a 31-year old female with Crohn's colitis for 8 years, complicated by enteropathic arthritis and pyoderma gangrenosum. She presented with intense and intractable periorbital pain, particularly at night and worse on eye movements. B-scan ultrasonography confirmed posterior scleritis and treatment with high dose oral steroids (up to 60 mg prednisolone) was initially effective, but subsequently failed to control the inflammation. There was only a partial response to infliximab. Five months after presentation, diplopia developed, with failure of abduction of the left eye. MRI scan of the orbits confirmed orbital myositis involving the left lateral and medial rectus muscles. Pulsed intravenous methylprednisolone and six cycles of intravenous cyclophosphamide over a three month period resulted in complete resolution of inflammatory symptoms. Conclusions This case highlights a rare combination of ocular abnormality secondary to Crohn's disease and reports successful resolution with aggressive immunosuppressive therapy.

A paraneoplastic retroperitoneal fibrosis resistant to corticosteroids treated with tamoxifen

Retroperitoneal fibrosis (RPF) is a rare disease characterized by an inflammatory proliferative fibrosing process occurring in the retroperitoneum, often causing urinary tract obstruction. Medical therapy is not well-defined, but glucocorticoids have been the mainstay of therapy. Recently, positive response to tamoxifen, an antiestrogen drug, has been reported among patients with RPF. We report the case of a 65-year-old male with a renal cell carcinoma in the upper pole of the right kidney showing acute renal failure due to a biopsy-confirmed RPF determining bilateral hydronephrosis. After polar resection of the right kidney, a high-dose oral steroid therapy did not modify the hydronephrosis. At 6 months, therapy with tamoxifen determined the retroperitoneal fibrotic mass regression and resolved the ureteral obstruction, that persists at the 13th month of follow-up. Tamoxifen can be considered as an effective alternative to corticosteroids and immunosuppressors in treating RPF.

Developing and auditing multiple sclerosis relapse management guidelines

This article describes the development and audit of local guidelines on how to manage multiple sclerosis (MS) relapses based on the National Institute for Health and Clinical Excellence (NICE) guidelines (2003). This was a collaboration between MS specialist nurses and neurologists with a specialist interest in MS working in southeast London, Kent and Medway. The aim was to promote a consistent approach to the management of relapses that reflected best practice and prescribing guidelines as outlined in the NICE guidelines (2003). The audit revealed that patients benefited from the use of high-dose oral methylprednisolone steroids by offering them swift access to treatment that was generally well tolerated and cost effective. In the process of the evaluation other methods of relapse management were reviewed. Further evaluation of the use of high-dose oral methylprednisolone for MS relapses is required, with particular reference to its efficacy when compared to the more established treatment with intravenous methylprednisolone.