Pulsed Intravenous Corticosteroids Research Articles

Clinical, demographic characteristics, and treatment protocols of optic neuropathies: Three-year follow-up experiences from a tertiary hospital in Turkey.

Background: This study was conducted to review the demographic and clinical characteristics, treatment protocols, and visual outcomes of patients with optic neuropathy. Methods: This historical cohort study analyzed the clinical features of 91 patients with optic neuropathy followed up for three years at a university hospital in Turkey. Results: Non-arteritic anterior ischemic optic neuropathy (NA-AION) was the most common group among the optic neuropathy subgroups (47.2%), and optic neuritis (ON) was the second most common group (38.5%). The mean age of symptom onset for NA-AION was 64.97 ± 12.15 years, significantly higher than the mean age of onset for ON (40.28 ± 15.52 years). Most of the patients with NA-AION had at least one systemic disease causing microangiopathy [51.1% had diabetes mellitus (DM), 33.3% had hypertension (HTN)]. Among the patients with ON, 51.4% were idiopathic, and 25.7% were multiple sclerosis (MS)-related ON cases. Patients with ischemic optic neuropathy (ION), ON, and traumatic optic neuropathy received pulse intravenous (IV) corticosteroids, and eleven patients with NA-AION received acetylsalicylic acid (ASA) therapy in addition to corticosteroids. There was a statistically significant increase in visual acuity in NA-AION and ON groups (P = 0.019). It was observed that the cases of ON peaked in the winter months in Turkey. Conclusion: In the differential diagnosis between NA-AION and idiopathic ON, the presence of one or more vascular systemic diseases and mean age may be the main factors. IV steroid treatment given to patients with NA-AION in the acute phase may significantly improve visual acuity.

Multifocal Mycobacterium kansasii osteomyelitis in a patient on ruxolitinib

• The presence of non-caseating granulomas on biopsy does not preclude mycobacterial infection, and should trigger careful investigation. • JAK inhibitors such as ruxolitinib increase the risk of mycobacterial infections, making specific investigations critical to diagnostic certainty. • M. Kansasii infection in bones and joints is rare, and is mainly found in immunosuppressed patients. • Biopsy and culture including specifically for mycobacteria should be initiated if there is any clinical suspicion. Mycobacterium kansasii spinal infection is rare. This is the first reported case in a patient taking ruxolitinib. A 69-year-old woman attended the emergency department with severe debilitating lumbar back pain and right sided flank pain. She had experienced persistent right lower chest wall pain following 4 weeks of coryzal symptoms but denied fever or weight loss. She was being treated with ruxolitinib for myelofibrosis. C reactive protein was 9 mg/L. CT and MRI showed bilateral hilar and mediastinal lymphadenopathy, and lytic lesions in the T10 vertebral body and left iliac bone. Noncaseating granuloma were found on endobronchial ultrasound guided lymph node biopsy and on biopsy of the iliac crest, with sarcoid considered a possible diagnosis. On two occasions the patient had pain so severe that she required hospitalisation. Pulsed intravenous corticosteroids was given with symptomatic improvement. Iliac bone biopsy grew Mycobacterium Kansasii at 6-week culture. The patient completed 12 months of treatment (12 months rifampicin and isoniazid, 6 months ethambutol), with resulotion of pain. We present a challenging case of mycobacterial osteomyelitis where there was initial diagnostic uncertainty. Sarcoidosis can involve multiple bony lesions but would not typically cause a spondylodiscitis. A recent pharmacovigilance report highlights that JAK inhibitors such as ruxolitinib increase the risk of mycobacterial infections, making specific investigations critical to diagnostic certainty.

Pediatric sarcoidosis with diagnostic and therapeutical insights.

Sarcoidosis is a chronic granulomatous disorder involving multiple systems and organs of undefined etiology. Although most of the morbidity relies upon lung disease, the function of several systems and organs can be affected. The natural history of lung disease consists of pulmonary involvement. An exaggerated and abnormal inflammatory response accompanies this aspect. There are noncaseating confluent epithelioid granulomas and, potentially, a progressive airway obstruction ab externo. As the disease is multisystemic, there is an increased likelihood of complications that may be serious and even fatal. The American Thoracic Society (ATS) Core Curriculum updates clinicians annually in adult and pediatric lung disease, critical medical care, and sleep medicine. In late 2020, the ATS targeted sarcoidosis. Also, in 2019, the French Sarcoidosis Group thoroughly revised the literature on pediatric sarcoidosis. Currently, staging is based on chest radiograph findings, and the most commonly used system is the Scadding classification, which has been applied to both children and adults alike. Treatment may consist of oral or pulsed intravenous corticosteroids, but it should be implemented in union with a rheumatologist, as there are no randomized controlled studies in children. Sarcoidosis is rare in childhood. Diagnosis is complex and relies on multiple diagnostic modalities with both staging and therapy progressively mirroring the sarcoidosis, which affects adults. In the majority of patients, spontaneous resolution will occur and observation is justified above treatment. Nevertheless, in case treatment is needed corticosteroids remain the mainstay of the treatment in some pediatric patients. Relapses are not uncommon and a long-term follow-up is essential.

Pulsed intravenous corticosteroids in chronic inflammatory demyelinating polyneuropathy: why not?

Pulsed intravenous corticosteroids in chronic inflammatory demyelinating polyneuropathy: why not?

Clinical features and visual outcomes of 111 patients with new-onset acute Vogt-Koyanagi-Harada disease treated with pulse intravenous corticosteroids

PurposeTo describe the clinical features, treatment and visual outcomes of Japanese patients with new-onset acute Vogt-Koyanagi-Harada (VKH) disease.MethodsClinical records of 111 patients who presented between 1999 and 2015 to the...

Acute coronary syndrome in Behcet's disease caused by a coronary artery aneurysm and thrombosis

Behcet's disease (BD) is a multisystemic vasculitis that can involve vessels of all sizes and is characterized by recurrent oral and genital ulcers with variable manifestations affecting the skin, eyes, and central nervous and musculoskeletal systems. Vascular involvement in BD is reported to be up to 40% in different series. The abdominal and thoracic aorta and pulmonary and femoral arteries are the most commonly involved arteries. However coronary arteries are rarely affected. Herein, we present a 29-year-old man who was consulted with progressive severe chest pain of 3 days in duration to our clinic. The patient was diagnosed with BD with mucocutaneous symptoms and a positive pathergy test 1 year ago and was in clinical remission for the last 6 months. At the first evaluation in the emergency department, the patient's vital signs were stable, whereas he had elevated troponin T levels with a normal electrocardiogram and hypokinetic areas in the apex of the heart in the echocardiography. Conventional and computed tomography coronary angiography revealed aneurysms and intramural thrombosis in the left anterior descending and right coronary arteries. Although ischemic symptoms and signs improved with anticoagulant and antiaggregant therapies, coronary aneurysms were observed to increase in size. Immunosuppressive (IS) treatment was started with pulse intravenous corticosteroids and cyclophosphamide. Because of the high re-stenosis risk, stents were not applied to the affected vessels during the acute thrombosis period. During routine investigations, an in situ pulmonary thrombosis was also detected bilaterally in the peripheral pulmonary arteries. In conclusion, coronary artery aneurysm is a rare and poor prognostic manifestation of BD. The treatment protocol for these aneurysms is not well clarified. IS therapies are definitely indicated, but the role of anticoagulants and invasive vascular interventions is controversial.

Carbamazepine-induced DRESS syndrome in a child: Rapid response to pulsed corticosteroids

DRESS syndrome is an idiosyncratic reaction to drugs, which can occur in both adults and children. To date there is no agreed upon criteria for its diagnosis; there is even less consensus on its management. We report the case of a 14- year-old boy with carbamazepine induced DRESS syndrome, predominantly involving the liver. He responded rapidly to high dose pulsed intravenous corticosteroids.

Treatment of Optic Neuritis by Plasma Exchange (Add-On) in Neuromyelitis Optica

To assess the contribution of plasma exchange (PE) in association (add-on) with pulsed intravenous corticosteroids in acute optic neuritis of neuromyelitis optica (NMO) and limited forms of NMO. Thirty-six patients with optic neuritis were treated from January 1, 1995, through December 31, 2010, with pulsed intravenous corticosteroids and 16 with pulsed intravenous corticosteroids plus PE. The ophthalmologic examination was performed at least 6 months after optic neuritis treatment. Visual acuity and visual field assessed with the Snellen scale and the logarithmic scale of the Early Treatment Diabetic Retinopathy Study were measured using standard automated perimetry and frequency doubling technology perimetry. Retinal peripapillary fiber thickness was measured using optical coherence tomography. Final visual acuity was 20/400 in the corticosteroid group and 20/50 in the PE group (P=.04). The gain in visual acuity was 20/200 in the corticosteroid group and 20/30 in the PE group (P=.01). A poor final visual acuity outcome (≤20/200) was found in 19 of 36 patients (53%) in the corticosteroid group and 2 of 16 patients (13%) in the PE group (P=.008). Mean (SD) thickness of peripapillary retinal nervous fibers was 63.1 (20.4) μm in the corticosteroid group and 70.3 (20.3) μm in the PE group (P=.16). The mean (SD) thickness in the temporal quadrant was 38.5 (14.1) μm in the corticosteroid group and 44.5 (12.7) μm in the PE group (P=.02). In multivariate analysis, PE treatment was the only independent factor associated with a visual acuity greater than 20/200. In optic neuritis associated with NMO, sequential treatment with pulsed intravenous corticosteroids and PE is more effective than standard monotherapy with corticosteroids on visual acuity outcome.

The Importance of Keeping the Chin Up in Graves Orbitopathy

A 42-year-old white woman suffering from mild euthyroid Graves orbitopathy for 6 years experienced recent-onset upper eyelid swelling, double vision, diminished vision, and reduced visual field of the right eye. Examination of the right eye showed decreased visual acuity, relative afferent pupillary defect, hypotropia with severely restricted elevation, upper visual field defect, impaired color contrast discrimination, and abnormal pattern visual evoked potential, but the Ishihara color plate reading and the optic disc appearance were normal. The left eye was normal, except for marked elevation restriction. On CT scan, the inferior rectus muscles were asymmetrically enlarged. These findings were suggestive of unilateral dysthyroid optic neuropathy, and a 2-week course of pulsed high-dose intravenous corticosteroids was subsequently administered. Since there was no improvement, the clinical examinations and tests were repeated, with the patient positioned in chin lift so that the right hypotropic eye was looking straight ahead. All tests normalized instantly and completely.

Treatment of Thyroid Eye Disease

Thyroid eye disease (TED) is an autoimmune disease characterized by varying degrees of proptosis, congestion and inflammation of the extraocular tissues, and eyelid retraction. It is usually seen in the setting of Graves' disease, but the severity of TED does not necessarily correlate with the level of systemic disease in a given patient. It is very important, nonetheless, to try to achieve a euthyroid state to minimize the chances of exacerbation of TED. Treatment of TED is based on the signs and symptoms displayed by the patient; there is no "one size fits all" approach. Generally, it is advisable to start with conservative measures, such as ocular lubrication with artificial tears, to manage symptoms of chronic irritation and redness. It is also imperative that the patient be advised to quit smoking, because there is a clear link between smoking and disease activity. Medical treatment with systemic oral or pulsed intravenous corticosteroids should be reserved for patients with active inflammation resulting in increased orbital pressure, compressive optic neuropathy, severe periorbital edema, or similar presentations. Once there is significant improvement in the acute inflammation, it is useful to treat patients who have residual inflammation with external beam radiation in order to be able to wean the patient off steroids and avoid their well-known complications.If there is significant corneal exposure due to lid retraction, and the lid position has been stable for at least 6months, eyelid surgery can be considered. If exposure is minimal, this may consist of a lateral tarsorrhaphy. For larger amounts of exposure, recession of the levator muscle, Müller's muscle, or both can be performed. Those patients who have stable diplopia for at least 6months are candidates for strabismus surgery. Patients who progress to severe proptosis or compressive optic neuropathy may need orbital decompression surgery. Generally, if more than one type of surgical procedure is necessary, orbital decompression is performed first, followed by strabismus surgery; eyelid surgery is performed last.

Giant cell arteritis complicated by acute pancreatitis: a case report

IntroductionWe describe a case of giant cell arteritis in a woman who was treated with high-dose systemic corticosteroids and subsequently developed acute pancreatitis.Case presentationA 78-year-old Caucasian woman presented with four weeks of progressive headache and scalp tenderness. One day before ophthalmology assessment, she had experienced visual obscurations in both eyes. Her visual acuity was 6/9 in both eyes, with a right afferent pupillary defect and right swollen optic nerve. She was diagnosed as having temporal arteritis and was urgently treated with high-dose pulsed intravenous and oral corticosteroids. Her previous diet-controlled diabetes needed insulin and oral hyperglycaemic therapy to control erratic blood sugars. On day 8 of treatment with steroids, she became unwell with epigastric pain and vomiting. She was diagnosed with acute pancreatitis and was treated conservatively.ConclusionAcute pancreatitis, a potentially life-threatening condition, is a rare but important side effect of systemic corticosteroids.

Therapies for steroid-resistant nephrotic syndrome

Between 10 and 20% of children with primary nephrotic syndrome are steroid-resistant (SRNS). From earlier studies in children with SRNS, we know that cyclosporin (with or without alternate-day prednisone) and cyclophosphamide (with pulse intravenous corticosteroids) result in comparable complete or partial remission rates of about 60%. An evaluation of the relative effectiveness of cyclophosphamide and cyclosporin has not been possible because of the absence of a head-to-head randomised trial. The Arbeitsgemeinschaft für Pädiatrische Nephrologie trial, published in this issue of Pediatric Nephrology, has filled this gap in our evidence base. Although there was no difference in the number of patients achieving complete remission, those patients receiving cyclosporin treatment were significantly more likely to achieve partial remission than those receiving intravenous cyclophosphamide. This result suggests that cyclosporin rather than cyclophosphamide should be used as first line therapy for children with SRNS.

Blindness due to the IgA variant of epidermolysis bullosa acquisita, and treatment with osteo-odonto-keratoprosthesis

Conflicts of interest: none declared. Sir, We describe a woman who initially presented in 1994, at age 47 years, with numerous groups of blisters in an annular arrangement on the trunk, strongly suggestive of linear IgA disease (LAD). Biopsy with immunofluorescence confirmed intense linear dermoepidermal IgA deposition which was felt to support the diagnosis of LAD, although recent re‐evaluation using indirect immunofluorescence and salt‐split skin has shown that the binding of IgA is predominantly dermal, thus suggesting that she actually has IgA‐epidermolysis bullosa acquisita (IgA‐EBA). No cause or associated disease have become apparent over 12 years of follow‐up. Some therapeutic aspects of this patient were included in an earlier report of vitamin E prophylaxis for dapsone‐induced headache1 (her initial treatment was with dapsone) but her treatments and response are further documented here as they are remarkable for the lack of efficacy. Treatments (individually or in combination) have included dapsone (with vitamin E); sulfapyridine; numerous topical (including ocular, buccal and vaginal) and systemic (oral and pulsed intravenous) corticosteroids; emollients/lubricants; topical and oral antibiotics; ciclosporin; mycophenolate mofetil; cyclophosphamide; intravenous immunoglobulin (six courses of infusions, each 2 g kg−1 over 5 days); subconjunctival mitomycin C; and subcutaneous interferon alfa (3 MIU three times weekly). Azathioprine was suggested but refused. Interferon, used on the basis of a single report in a patient who also had hepatitis C,2 was the most effective agent but caused significant anaemia and had to be discontinued.

Pyoderma gangrenosum

SummaryPyoderma gangrenosum (PG) is a non‐infectious reactive neutrophilic dermatosis which typically starts with pustules which rapidly evolve to painful ulcers of variable size and depth with undermined violaceous borders. Since its first description in 1930, the pathogenesis of PG has remained elusive even as an ever‐widening range of systemic diseases has been described in association with it. The diagnosis of PG is based on clinical and pathologic features and requires exclusion of other conditions that produce ulcerations, since misdiagnosis exposes patients to risks associated with treatment. Critical to proper management are correct diagnosis, identification and treatment of any underlying disorder, and the appropriate choice of topical and systemic therapy. PG has four distinctive clinical and histologic variants, and the specific clinical features of the lesion may provide a clue to the associated disease. The most common associated diseases are inflammatory bowel disease, rheumatological or hematological disease or malignancy. Although there is no single successful treatment for PG, certain type of PG lesions are recognized to respond more readily to accepted therapies than others. Local treatment may be sufficient for mild disease, while systemic immunosuppressive therapy is necessary for severe cases. The treatments with the best clinical evidence are oral or pulse intravenous corticosteroids, and cyclosporine. Surgical therapy is useful in selected cases in conjunction with immunosuppression. Wound stabilization is obtained only through control of the systemic and local inflammatory process. Emerging therapies include use of platelet‐derived growth factor and cell culture grafts when re‐epithelialization is slow, and the TNF‐alpha blocking agent infliximab for refractory disease. Despite advances in therapy, the long‐term outcome for patients with PG remains unpredictable, because relapses are common.

Optimal treatment of pyoderma gangrenosum.

The optimal treatment of pyoderma gangrenosum includes a combination of local wound care and systemic medications. Oral and pulse intravenous corticosteroids have traditionally been the most commonly recommended first-line systemic therapies. Cyclosporine, with or without corticosteroids, has more recently emerged as a first-line systemic treatment. A multitude of immunosuppressive and immune-modulating medications, as well as antimicrobial agents with anti-inflammatory properties have also been widely prescribed. Often, it is difficult to achieve control of aggressive cases of pyoderma gangrenosum, necessitating administration of a combination of systemic therapies. Furthermore, patients recalcitrant to one or many medications are frequently reported. Concomitant disease, intolerance to a class of medications, and the patient's response to prior therapies can help guide a practitioner in choosing the optimal treatment of pyoderma gangrenosum.

Multiple sclerosis intra-blood-brain-barrier IgG synthesis: effect of pulse intravenous and intrathecal corticosteroids.

Nine severely disabled clinically definite chronic progressive multiple sclerosis (MS) patients who had at least one determination of intra-blood-brain-barrier (BBB) IgG synthesis rate of greater than 7 mg/day (upper limit of normal = 3.3) participated in this study. Seven patients were given 1 gram of methylprednisolone sodium succinate (MP) by intravenous infusion over 30 minutes once a day for 3 days. Statistically significant (p less than .05) reduction in intra-BBB IgG synthesis (mg/day) was seen in 4/7 patients, but in only 2 were normal levels of synthesis rate (less than 3.3 mg/day) attained. Rebound of IgG synthesis to premedication rates occurred within 30 days in 2/4 patients. There was no change in intensity or pattern of cerebrospinal fluid (CSF) oligoclonal IgG bands by isoelectric focusing, immunofixation, and silver staining. A subsequent course of intrathecal methylprednisolone acetate (MPA) (80 mg twice a week for 5 weeks) was given to 5 of the 7 patients and to 2 additional patients not previously treated. In spite of signs of subarachnoid inflammation, a statistically significant depression of intra-BB synthesis, which far exceeded that from the pulse treatment occurred in all 7, including the 2 patients whose intra-BBB IgG synthesis rates were previously resistant to pulse steroid administration. Normal levels of synthesis were rapidly reached in 4/7 patients; however, an IgG synthesis rebound occurred in 3/7 patients which was just as rapid. One out of 7 patients showed a temporary reduction in the number of cathodic IgG oligoclonal bands in the CSF. Two patients required discontinuation of treatment due to aseptic meningitis in one and progressive weakness in the other. Clinically, these severely afflicted patients with fixed deficits remained unchanged with either treatment protocol. While MPA and ACTH have similar initial effect on the central nervous systems (CNS) inflammatory response in MS, the well documented risk of serious adversities with MPA prohibit its clinical use in MS in its present form.