High-dose Steroid Pulse Therapy Research Articles

Retrobulbar Optic Neuritis after COVID-19 Infection

Purpose: To report a case of retrobulbar optic neuritis after COVID-19 infection.Case summary: A 20-year-old Korean man, without any previous relevant medical history, presented with sudden blurred vision in the left eye that began 6 days after a COVID-19 infection. At his initial visit, the best-corrected visual acuity in the left eye was counting fingers at 30 cm. The pupil showed a grade 2 relative afferent pupillary defect. The fundus exam was normal in both eyes. In the visual evoked potential (VEP) exam, the P100 amplitude of the left eye was decreased and delayed. Orbit magnetic resonance imaging showed asymmetric enlargement and mild enhancement in the left optic nerve. No other lesions were observed around the optic nerve. Steroid pulse therapy was started after a diagnosis of retrobulbar optic neuritis. After 1 month, the patient’s visual acuity had improved to 1.0 in the left eye. The relative afferent pupillary defect and visual field defect disappeared. On VEP, the P100 amplitude recovered, and the delay disappeared.Conclusions: Retrobulbar optic neuritis is a possible neuro-ophthalmological complication of COVID-19 infection. In such cases, high-dose steroid pulse therapy may be helpful, as in the treatment of other forms of optic neuritis.

Bickerstaff’s brainstem encephalitis: a rare case of neurologic complication in Ulcerative Colitis

Bickerstaff’s brainstem encephalitis is a rare autoimmune disorder that presents with ataxia, ophthalmoplegia, disturbance of consciousness and quadriplegia. A 45-year-old man with a history of ulcerative colitis (UC) taking mesalazine (5-aminosalicylic acid) visited the emergency room presenting with ataxia, ophthalmoplegia and a progressively worsening cognitive impairment. Cerebrospinal fluid analysis showed mild elevation in protein and white blood cell count and increased intracranial pressure. Anti-GQ1b autoantibodies were found positive in the patient’s serum and contrast-enhanced brain magnetic resonance imaging showed diffuse leptomeningeal enhancement and pontine lesions. Based on these findings and the patient’s clinical course and history, he was diagnosed with Bickerstaff’s brainstem encephalitis. Mesalazine was discontinued and high-dose steroid pulse therapy was started, followed by intravenous immunoglobulin, which resulted in gradual improvement of the neurologic symptoms. When an ulcerative colitis patient presents with progressive cognitive impairment, quadriplegia and disturbance of consciousness and gait, Bickerstaff brainstem encephalitis should be considered in the differential diagnosis and prompt immunotherapy may lead to favorable prognosis.

Prognostic Factors for Visual Outcomes in Acute Anterior Optic Neuritis Using Optical Coherence Tomography

Purpose: To identify the clinical characteristics and predictors of visual outcomes in acute anterior optic neuritis patients treated with high-dose steroid pulse therapy using spectral domain optical coherence tomography (SD-OCT).Methods: In this study, the medical records of 34 acute anterior optic neuritis patients treated with intravenous high-dose steroid pulse therapy and followed-up for at least 3 months were retrospectively reviewed. The patients were divided into two groups based on the final best-corrected visual acuity (BCVA); group 1 (n = 23) had a final BCVA ≥20/32, while group 2 (n = 11) had a final BCVA <20/32. Clinical characteristics and optical parameters obtained using SD-OCT were evaluated to identify factors influencing the final visual outcome.Results: There were no statistically significant differences in clinical or demographic data between the two groups. Peripapillary retinal nerve fiber layer (pRNFL) swelling at initial presentation was less common (p = 0.015), and the time to pRNFL thickness stabilization was longer (p = 0.016), in group 1. Multivariate logistic regression demonstrated better final visual acuity for patients in whom pRNFL thickness stabilization took longer, as well as in those with a smaller reduction in macular ganglion cell-inner plexiform layer (mGCIPL) thickness, and those without recurrence (p = 0.002, 0.016, and 0.035, respectively).Conclusions: Intravenous high-dose steroid pulse therapy much improved long-term visual outcomes in acute anterior optic neuritis patients with delayed pRNFL thickness stabilization, a smaller mGCIPL thickness reduction, and no recurrence. This suggests that inner retinal layer thickness and recurrence after stabilization may serve as biomarkers for final visual acuity after intravenous steroid treatment in acute anterior optic neuritis.

Pituitary Abscess as Manifestation of IgG4-Related Hypophysitis: A Case Report

IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition with heterogeneous organ-specific manifestations. IgG4-related hypophysitis results from pituitary involvement and represents a rare neuro-ophthalmic complication of IgG4-RD, but the presentation as pituitary abscess is exceptional. We report the case of a 38-year-old otherwise healthy woman with a 3-year history of relapsing pituitary sterile abscess repeatedly treated with neurosurgery and antimicrobials. Histological re-examination of pituitary biopsy specimens revealed a dense inflammatory infiltrate rich in IgG4-positive plasma cells. Serum IgG4 levels were normal and circulating plasmablasts were increased. A diagnosis of IgG4-related hypophysitis was made and the patient treated with high-dose steroid pulse therapy with subsequent reduction of the pituitary lesion and resolution of symptoms. This case highlights how the diagnostic intuition on the basis of histopathology has changed management with prompt dramatic response to steroid therapy.

Optic neuropathy secondary to granulomatosis with polyangiitis in a patient with Graves\u2019 disease: a case report

BackgroundDysthyroid optic neuropathy is the most commonly suspected diagnosis of optic neuropathy in Graves’ patients; however, other causes need to be ruled out. We present a unique case of optic neuropathy secondary to hypertrophic pachymeningitis with antineutrophil cytoplasmic antibody-associated vasculitis, which was suspected to be antithyroid drug related.Case presentationA 79-year-old Japanese male presented with acute visual loss in the left eye. He had a 24-year history of Graves’ disease and was taking methimazole. Best-corrected visual acuity was 0.8 in the right eye and light perception in the left eye, and relative afferent pupillary defect in the left eye was seen. Ocular movement was normal, and there were no findings explaining visual loss in intermediate optic media and fundus in the left eye. Contrast-enhanced magnetic resonance imaging demonstrated thickened dura mater. Tests for myeloperoxidase-antineutrophil cytoplasmic antibody, proteinuria, and hematuria were positive; pulmonary nodule lesions and a blood clot in the left lower leg were also found. After excluding the presence of diseases that could lead to hypertrophic pachymeningitis, we diagnosed optic neuropathy due to hypertrophic pachymeningitis with granulomatosis with polyangiitis—a subtype of antineutrophil cytoplasmic antibody-associated vasculitis. Since he had history of using methimazole, antineutrophil cytoplasmic antibody-associated vasculitis was considered as drug related. We started high-dosage steroid pulse therapy followed by 1 mg/kg body weight daily of oral prednisolone, and subsequently tapered. Methimazole was stopped. Best-corrected visual acuity recovered to 0.9, 2 weeks after starting treatment. Though myeloperoxidase-antineutrophil cytoplasmic antibody remained negative, the symptom relapsed 6 months after treatment initiation. We gave a second high-dose steroid pulse therapy followed by prednisolone tapered together with methotrexate. Remission remained, and using 4 mg/week methotrexate without prednisolone, myeloperoxidase-antineutrophil cytoplasmic antibody was kept within the normal limit until now, 4 years after onset.ConclusionWe present a case of optic neuropathy with hypertrophic pachymeningitis related to antineutrophil cytoplasmic antibody-associated vasculitis, which was suspected to be drug related. The patient had good visual recovery after quitting the drug and receiving immunosuppressive therapy with systemic steroids. Hypertrophic pachymeningitis with antineutrophil cytoplasmic antibody-associated vasculitis related to antithyroid drugs should be considered as a differential diagnosis for optic neuropathy in Graves’ patients in whom optic nerve compression is not obvious.

Clinical Characteristics and Prognoses of Patients With Systemic Lupus Erythematosus Hospitalized for Pulmonary Infections.

Objective: To identify factors associated with mortality in SLE patients who were hospitalized for pulmonary infections (PIs).Methods: This single-center retrospective study analyzed the characteristics and risk factors for mortality in 95 SLE patients hospitalized for PIs.Results: Ninety-five SLE patients had 97 episodes of hospitalization for PIs, and 33 of these episodes (34.02%) led to death. Death from PI was associated with a higher neutrophil count (6.30 vs. 4.201 × 109/L, p < 0.01), immunoglobulin G (6.20 vs. 9.82 g/L, p = 0.01), serum creatinine (126.00 vs. 73.00 μmol/L, p = 0.01), proteinuria (2.99 vs. 0.54 g/day, p < 0.01), cardiopulmonary involvement (57.58 vs. 34.38%, p < 0.05), SLE disease activity index (SLEDAI; 11.00 vs. 6.00, p < 0.05), and opportunistic infections (78.79 vs. 45.31%, p < 0.05). Demographic characteristics, antibody/complements, bacterial infection, and primary treatment before infection (including corticosteroid and immunosuppressants) had no effect. Multivariate analysis indicated cardiopulmonary involvement (HR: 2.077; 95%CI: 1.022–4.220; p = 0.043) and opportunistic infection (HR: 2.572; 95%CI: 1.104–5.993; p = 0.029) were independent risk factors for mortality. High-dose steroid pulse therapy (HR: 0.982; 95%CI: 0.410–2.350; p = 0.982) and first-line immunosuppressant therapy (HR: 1.635; 95%CI: 0.755–3.542, p = 0.212) had no effect on mortality.Conclusion: Cardiopulmonary involvement and opportunistic infection were independent risk factors for mortality for SLE patients hospitalized for PIs. Use of high-dose pulse steroids and or immunosuppressants before hospitalization had no significant effects.

Hemophagocytic lymphohistiocytosis complicating invasive pneumococcal disease: a pediatric case report

BackgroundHemophagocytic lymphohistiocytosis (HLH) is an infrequent but life-threatening disease due to excessive immune activation. Secondary HLH can be triggered by infections, autoimmune diseases, and malignant diseases. Streptococcus pneumoniae is a pathogenic bacterium responsible for invasive pneumococcal disease (IPD) such as meningitis and bacteremia. Although the pneumococcal conjugate vaccine (PCV) has led to reductions in IPD incidence, cases of IPD caused by serotypes not included in PCV are increasing. There are few reports of secondary HLH caused by IPD in previously healthy children. We herein report a rare case of a previously healthy boy with secondary HLH complicating IPD of serotype 23A, which is not included in the pneumococcal 13-valent conjugate vaccine (PCV-13).Case presentationAn 11-month-old boy who had received three doses of PCV-13 was hospitalized with prolonged fever, bilateral otitis media, neutropenia and elevated C-reactive protein (CRP) levels. Blood culture on admission revealed S. pneumoniae, leading to a diagnosis of IPD. HLH was diagnosed based on a prolonged fever, neutropenia, anemia, hepatosplenomegaly, hemophagocytosis in the bone marrow, and elevated serum levels of triglycerides, ferritin, and soluble interleukin-2 receptor. He received broad-spectrum antibiotics and intravenous immunoglobulins for IPD and high-dose steroid pulse therapy and cyclosporine A for HLH; thereafter, his fever resolved, and laboratory findings improved. The serotype of the isolated S. pneumoniae was 23A, which is not included in PCV-13.ConclusionsIt is important to consider secondary HLH as a complication of IPD cases with febrile cytopenia or hepatosplenomegaly, and appropriate treatment for HLH should be started without delay.

Safety and Efficacy of Steroid Pulse Therapy for Acute Loss of FEV1 in Lung Transplant Recipients After Exclusion of Acute Cellular Rejection

BackgroundThe standard treatment of acute cellular rejection after lung transplantation (LTx) is a high-dose steroid pulse therapy. In our center, this therapy is also the standard of care for LTx recipients with acute loss of forced expiratory volume in 1 second (FEV1), after excluding specific causes such as acute rejection on biopsy. The aim of this retrospective study was to evaluate the safety and efficacy of steroid pulse therapy. MethodsFrom 2015 to 2018, 33 consecutive patients (17 male patients, mean age ± SD, 50.5 ± 12.5 years) were included. All patients underwent routine examinations to exclude acute cellular rejection and other specific causes. FEV1 was routinely measured after 5 days, and 1, 3, and 6 months. Positive response to steroid pulse therapy was defined by increase of FEV1 > 10%. ResultsThe mean decrease ± SD from baseline in FEV1 at the start of steroid pulse therapy was 380 ± 630 mL (P = .02). FEV1 changed after 5 days by 170 ± 180 mL (P = .0007), and after 1 month by 140 ± 230 mL (P = .70), 3 months by −60 ± 240 mL (P = .15), and 6 months by −80 ± 290 mL (P = .73). A positive response was observed in 21% of patients after 3 months and 12% after 6 months. High bronchoalveolar lavage (BAL) eosinophil count correlated with a higher FEV1 after steroid pulse therapy. Serious complications were observed in 4 out of 33 patients (12%) with 1 fatal event (pneumonia). ConclusionsOnly a minority of patients after LTx with loss of FEV1 after exclusion of acute cellular rejection benefit from steroid pulse therapy. Patients with BAL eosinophilia are more likely to respond. However, severe complications were observed.

Cryptococcus endocarditis: A case report and review of the literature

IntroductionCryptococcus neoformans is known to be a cause of meningitis. However, as cryptococcal endocarditis is rare, it is not well understood. Here, we describe a case with Implantable Cardioverter Defibrillator associated endocarditis and meningitis caused by Cryptococcus neoformans and we review the literature associated cryptococcal endocarditis. Case presentationA 72 years old Japanese male presented in emergency department with non-productive cough and respiratory discomfort. His past medical history was ischemic heart disease four years ago and ICD was implanted. Physical examination was unremarkable. Chest computer tomography revealed ground glass opacity in the right lung. He received a diagnosis of amiodarone-induced interstitial pneumonitis and high dose steroid pulse therapy. Septic shock and acute respiratory failure occurred after steroid therapy. Cryptococcus neoformans was identified by blood culture and cerebral spinal fluid. Intravenous liposomal Amphotericin B and oral flucytosine were initiated. Transesophageal echocardiography revealed vegetation on the lead of the ICD. Diagnosis of cryptococcal endocarditis was made. The patient died despite antifungal therapy was continued. DiscussionWe analyzed our case and 8 cases of cryptococcal endocarditis in the literature for 40 years. Almost all of the patients had previous valve replacement surgery or immunocompromised state. Three cases had meningitis. Surgery performed in 3 cases. The overall mortality rate were 44.4%. ConclusionsCryptococcal endocarditis is rare and carries a high mortality. Almost all of the patients had underlying diseases. Diagnosis needs repeating blood culture and echocardiogram, sometimes. Cryptococcal endocarditis needs lumber puncture for rule out meningitis.

Extragonadal germ cell tumor of the posterior mediastinum in a child complicated with spinal cord compression: a case report

BackgroundGerm cell tumors (GCTs) in children are rare neoplasms with diverse pathological findings according to the site and age of presentation. The most common symptoms in children with mediastinal GCTs, which are nonspecific, are dyspnea, chest pain, cough, hemoptysis, vena cava occlusion syndrome, and fatigue/weakness. Because of these nonspecific symptoms, it is difficult to suspect a mediastinal mass. A posterior mediastinal tumor causing spinal cord compression is an important example of an oncologic emergency arising from a neurogenic tumor.Case presentationChildren with posterior mediastinum GCTs can be easily mistaken as having a neurogenic tumor because of site of tumor origin. We treated our 7-year-old patient with emergency decompression surgery and high-dose steroid pulse therapy to prevent secondary injury to the spinal cord. Primary injury was a result of spinal cord compression due to the initial manifestation of GCT in the posterior mediastinum. Cisplatin-based chemotherapy was also administered. The patient was followed up regularly for 3 years and is undergoing rehabilitation without any signs of recurrence.ConclusionsWe present an extremely rare case of a child with paraparesis caused by extradural spinal cord compression as the initial manifestation of GCT in the posterior mediastinum. The child was treated with emergency decompression surgery and high-dose pulse steroid therapy to prevent secondary injury to the spinal cord.

High-dose intravenous steroid pulse therapy in ocular involvement of Behcet's disease: a pilot double-blind controlled study.

To evaluate the efficacy of intravenous high-dose pulses of methylprednisolone (IVPM) for treatment of ocular involvement in Behcet's disease (BD). In a double-blind control study, we randomized BD patients with posterior uveitis (PU) and/or retinal vasculitis (RV) into two groups. They received either IVPM (1000 mg methylprednisolone) or placebo for 3 consecutive days. Both groups received combination therapy with IV cyclophosphamide, azathioprine and prednisolone for 6 months. Visual acuity (VA), Disease Activity Index (DAI) based on the inflammatory state of each section of each eye, total inflammatory (TIAI) and adjusted DAI (TADAI) for each patient were calculated. The comparisons were done by paired t- and Mann-Whitney U-test. Seventeen patients in each group completed the treatment. The mean VA improved from 0.5 to 0.8 (P < 0.000001) for the study and from 0.6 to 0.7 (P < 0.02) for the placebo group. The difference was significant (P = 0.01). The comparison showed no significant difference regarding DAI improvement in other items (P > 0.2): PU, 1.9 to 0.5 (P < 0.0006) versus 2.3 to 0.8 (P < 0.0002); RV: 4.0 to 1.1 (P < 0.0004) versus 3.1 to 1.1 (P < 0.0005); TIAI: 23 to 5.7 (P < 0.0002) versus 24.8 to 8.4 (P < 0.003); TADAI: 24.1 to 7.3 (P < 0.0002) versus 25.9 to 7.9 (P < 0.004). We had one flare in the study versus seven in the placebo group (P < 0.005). Adding high-dose intravenous steroid pulse therapy to conventional combination therapy for severe ocular lesions of BD may cause better improvement on VA and fewer flares during the first 6 months of treatment.

Uremic Optic Neuropathy in Chronic Renal Failure

Purpose: To report a case of uremic optic neuropathy occurring in a patient with chronic renal failure. Case summary: A 40-year-old male who was diagnosed with chronic renal failure and treated with peritoneal dialysis and hemodialysis for 17 years presented with blurred vision and a moving pain in his left eye for 2 days. The best corrected visual acuity (BCVA) was 0.2 in his left eye, and an inferior altitudinal visual field defect was noted on Humphrey perimetry. Fundus examination and optical coherence tomography showed optic disc swelling in his left eye; the right eye was unremarkable. These findings were compatible with a diagnosis of uremic optic neuropathy or anterior ischemic optic neuropathy of his left eye. After treatment of hemodialysis and intravenous high dose steroid pulse therapy, the BCVA in his left eye was 0.8. However, since he refused oral steroid maintenance therapy, his BCVA later decreased to 0.4. After treatment with subtenon triamcinolone injection, the BCVA in his left eye was 1.0 and showed a stable disease course. Conclusions: When patient with chronic renal failure presents with acute decrease in visual acuity and visual field defect, optic neuropathies including uremic optic neuropathy should be considered and prompt hemodialysis and systemic steroid treatment should be done. J Korean Ophthalmol Soc 2016;57(7):1187-1191

SAT0312 Efficacy and Safety of High Dose Steroid Pulse Therapy in Early Progressive Systemic Sclerosis: the Risk of Renal Crisis is with Older Age

BackgroundTherapeutic options for progressive systemic sclerosis (SSc) are extremely restricted. Up to date, high doses of glucocorticoids have a limited role in SSc skin and lung disease severity treatment because...

Efficacy of Azithromycin for Treatment of Acute Exacerbation of Chronic Fibrosing Interstitial Pneumonia: A Prospective, Open-Label Study with Historical Controls

Background: Acute exacerbation of chronic fibrosing interstitial pneumonia (AE-CFIP) is an often fatal condition with no established treatment. Recently, macrolides were found to be beneficial in cases of acute lung injury. Objectives: To examine the clinical effectiveness and safety of intravenous azithromycin in patients hospitalized for AE-CFIP. Methods: A prospective, open-label study with historical controls was conducted. Twenty consecutive patients with AE-CFIP received azithromycin. They were compared with a historical cohort treated with fluoroquinolone (n = 56). All patients received high-dose steroid pulse therapy. The primary end point was mortality at 60 days. The secondary end point was safety of intravenous azithromycin in patients with AE-CFIP. Inverse probability of treatment weighting (IPTW) using the propensity score was performed to investigate the relationship between azithromycin use and survival time. Results: Mortality was significantly lower in the patients treated with azithromycin than in those treated with fluoroquinolone (mortality rate at 60 days: 20 vs. 69.6%, p < 0.001; median survival time: not reached vs. 29.5 days, p < 0.001). The IPTW adjusted hazard of mortality at 60 days in patients receiving azithromycin was 0.17 (95% CI 0.05-0.61). No serious adverse events were observed. Conclusions: Azithromycin was associated with improved outcomes in patients with AE-CFIP. Further studies are needed to verify this finding (Clinical trial JMA-IIA00095).

A Case of Systemic Lupus Erythematosus Initially Presented with Acute Acalculous Cholecystitis

SLE is an autoimmune disease with multiorgan involvement and a wide range of clinical manifestations, and inflammation of gallbladder also can be represented. There were a few cases of acute acalculous cholecystitis (AAC) in previous reports. Most of them tended to already know about underlying SLE when detected AAC at that time. It may be difficult to detect AAC caused by SLE not due to biliary stone if physician is not conscious of undiagnosed lupus. We introduce a 70-year old female patient, who is diagnosed with AAC. Her symptoms were satisfied the ACR classification criteria for SLE, and was diagnosed with SLE, simultaneously. After a high dose steroid pulse therapy, followed by cyclophosphamide, her symptoms have improved rapidly. In order to better diagnose and treat the disease, we need to be aware of AAC as a potential manifestation of SLE.

Association between CYP3A5 Genotypes in Graft Liver and Increase in Tacrolimus Biotransformation from Steroid Treatment in Living-donor Liver Transplant Patients

We retrospectively examined whether cytochrome P450 (CYP) 3A5 genotypes are associated with high-dose steroid pulse treatment-induced functional gain of tacrolimus biotransformation in living-donor liver transplant patients. Concentrations of tacrolimus and its 3 primary metabolites, 13-O-demethyl tacrolimus (M-I), 31-O-demethyl tacrolimus (M-II), and 15-O-demethyl tacrolimus (M-III), were measured in trough blood samples from 18 liver transplant patients, by liquid chromatography-tandem mass spectrometry/mass spectrometry (LC-MS/MS). In patients engrafted with a CYP3A5*1-carrying liver but not with a CYP3A5*3/*3-carrying liver, the concentration/dose ratio of tacrolimus significantly fell after therapy, while ratios of M-I/tacrolimus, M-II/tacrolimus, and M-III/tacrolimus were significantly higher after therapy than before (p = 0.032, p = 0.023, and p = 0.0078, respectively). After steroid pulse therapy, the concentration of tacrolimus measured by immunoassay was significantly higher than that measured by LC-MS/MS in patients engrafted with a CYP3A5*1-carrying liver, but not those engrafted with a CYP3A5*3/*3-carrying liver. This suggests that the increased ratio of tacrolimus metabolites/tacrolimus can be explained by induction of CYP3A5 via high-dose steroid pulse therapy. Further, the concentrations of tacrolimus measured by the immunoassays were overestimated, partly because of cross-reactivity of the monoclonal antibody they incorporated to detect tacrolimus, with the increased metabolites in patients with a CYP3A5*1-carrying graft liver.

Acute Retrobulbar Optic Neuritis with Hematologic Abnormalities

Purpose: We present a case of a patient with optic neuritis who had underlying suspicious idiopathic thrombocytopenic purpura. Case summary: A 35-year-old female with no other systemic disease visited our clinic due to acutely decreased visual acuity in her left eye 10 days in duration. Relative afferent pupillary defect was observed, but without definite papilledema. Based on brain magnetic resonance imaging (MRI), optic neuritis was suspected. Laboratory tests showed increased red blood cells, hemoglobin and, hematocrit levels and decreased platelets. Peripheral blood smear test showed decreased platelets, relative lymphocytosis and atypical lymphocytes. Specific antibodies for autoimmune disease were not present. High-dose steroid pulse therapy (methyl prednisolone 1.0 g/d, 3 days) was started. One month after treatment her visual acuity and platelet count recovered and her visual field defect improved. J Korean Ophthalmol Soc 2014;55(10):1567-1572

Factors associated with outcome after combined steroid and orbital radiotherapy on Graves’ ophthalmopathy

In order to seek possible confounding factors in relation to clinical responsiveness to high-dose intravenous steroid pulse therapy and orbital irradiation followed by oral steroids in the treatment of thyroid-associated ophthalmopathy (TAO), we reviewed the medical records of 53 admitted patients (22 men and 31 women out of a total pool of 167 patients who had diagnosis of TAO). To assess the improvement TAO, we employed proptosis as an objective index and followed up longitudinally. The final ratios of improvement in the left and right eyes respectively were 71.0% and 64.5%. Significant difference in the therapeutic effect on proptosis was found between male and female at the end of observation. The decrease in proptosis was significantly greater in female patients than in male patients (P<0.05). Among patients’ background characteristics, smoking status and body mass index did not relate to the severity of the eye disease but rather to its improvement (right: P=0.18, left: P<0.05). These results suggest that the combined steroid and radiotherapy followed by oral steroid therapy should be optimized to obtain higher effectiveness.

Early Intervention with High-Dose Steroid Pulse Therapy Prolongs Disease-Free Interval of Severe Alopecia Areata: A Retrospective Study

BackgroundSpontaneous recovery of severe alopecia areata is rare and the condition is difficult to treat.ObjectiveThe aim of this study is to investigate and compare the effects and safety of steroid pulse therapy between oral and intravenous administrations between 1999 and 2010 at the Department of Dermatology, National Cheng Kung University Hospital.MethodsData were retrospectively retrieved. A satisfactory response was defined as more than 75% hair regrowth in the balding area.ResultsA total of 85 patients with more than 50% hair loss were identified and treated, with an overall satisfactory response rate of 51.8%. The mean follow-up time was 37.6 months, with a relapse rate of 22.7%. Patients with alopecia areata (hereafter, AA) of recent onset within one year showed higher response rates (p<0.001) and lower relapse rates compared to patients with AA persisting for more than 1 year. Further, even in patients with alopecia totalis, alopecia universalis or ophiasis type, early treatment resulted in a satisfactory response rate of 47% among the treated patients. In general, oral therapy was as effective and well-tolerated as intravenous therapy.ConclusionThe response rate is determined by disease severity and time of intervention, not by the administration form of steroid pulse therapy. Oral steroid pulse therapy can be considered as the first-line treatment for patients with severe AA of recent onset within one year.

A pilot study to monitor Graves' ophthalmopathy with a combination of pattern‐reversal and motion‐onset visual evoked potentials

The aim of this pilot study was to evaluate the efficacy of visual evoked potentials (VEPs) in the monitoring of visual function during a high-dose intravenous steroid pulse therapy and apheresis treatment of severe Graves' ophthalmopathy (GO). PATIENTS AND RESEARCH DESIGN: Nine patients with severe and active GO were treated with high-dose methylprednisolone (1 g day(-1) three times within 1 week, then 0.5 g day(-1) seven times for 2 weeks) combined with plasma filtration (twice a week in weeks 1, 2, 4, 7, and 10). Pattern-reversal and motion-onset VEPs were examined three times, pretreatment, after steroid pulses, and after the last apheresis. After 10 steroid pulses, the visual acuity was significantly better and the pattern-reversal VEP amplitudes (element size of only 20') had a similar trend for improvement. However, this effect disappeared after 7 weeks when only apheresis treatment was performed. No significant changes in the latencies of any of the tested VEP variants were found in relationship to the treatment. Only the observed large intraindividual variability of the VEP parameters between repeated examinations of patients with the shortest duration of GO might be recognized as a marker for functional changes of the visual pathway due to GO. Although this pilot study cannot provide a definite view on the usefulness of the extended set of VEPs in objective monitoring of GO, it seems that the steroid pulse therapy effect is detectable in contrast to the lack of influence by apheresis on the electrophysiological parameters tested.