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Objectives There has been no large-scale study of methylprednisolone pulse therapy in Asian patients with multiple sclerosis (MS) or neuromyelitis optica (NMO), despite it being widely used for acute relapse. We aimed to clarify treatment response of MS and NMO patients to methylprednisolone pulse therapy and post-pulse oral corticosteroids in real clinical practice in a multicenter study in Japan. Methods Investigators at 28 institutions collected changes in neurological symptoms/signs and Expanded Disability Status Scale (EDSS) scores before and within 1 week of completion of methylprednisolone pulse therapy carried out in 2010, and after post-pulse oral corticosteroids therapy, by retrospective review of medical records. Results In 345 patients (95.1% of all registered patients), 457 series of methylprednisolone pulse therapy were carried out for treatment of acute relapse. EDSS scores improved by 0.8 ± 1.1 (mean ± SD) after the first course. The second and third courses also produced sufficient improvements (by 0.7 and 0.6, respectively), but much smaller improvements were observed thereafter. The target neurological symptoms and signs improved in 79.5% of patients. Improvement rates were 5–20% lower after a course of pulse therapy than after a series of pulse therapy. A half dose (500 mg/day) produced less improvement than a standard dose (1000 mg/day; 65.9 vs 79.5%). During post-pulse oral corticosteroid therapy, EDSS scores decreased by 0.6 ± 0.9. No significant adverse effects were observed. Conclusions Methylprednisolone pulse therapy is beneficial in nearly 80% of Japanese MS and NMO patients, and EDSS score improvements after therapy are compatible with those in Western MS patients.

医学部小児科学教室 大澤眞木子教授退任記念特別号

A 42-year-old female with body weight loss, finger tremors and ocular discomfort was diagnosed with Graves' disease complicated with ophthalmopathy. Thiamazole therapy rapidly improved her hyperthyroidism. However, she was admitted to our hospital because her eye symptoms acutely deteriorated over a period of two weeks. She had ocular immotility, exposure keratitis, conjunctival edema, severe proptosis and visual impairment with a high titer of serum thyroid-stimulating antibody (TSAb). Methylprednisolone pulse therapy at a dose of 500 mg/day improved her eye symptoms. Although the mechanism of the progression of Graves' ophthalmopathy has not yet been elucidated, special attention should be paid to the occurrence of ophthalmopathy even after the initiation of thiamazole therapy.

Tomoaki Higashiyama,1 Yasuhiro Nishida,2 Masahito Ohji1 1Department of Ophthalmology, 2Clinical Medical Education Center, Shiga University of Medical Science, Shiga, Japan Purpose: To characterize the relationship between inflammation and swelling of extraocular muscles in thyroid-associated ophthalmopathy before and after methylprednisolone pulse therapy.Methods: The signal intensities and volumes of the superior rectus (SR), inferior rectus (IR), lateral rectus (LR), medial rectus (MR), and superior oblique (SO) muscles were measured with magnetic resonance imaging in 25 eyes of 25 patients with thyroid-associated ophthalmopathy in the acute inflammatory phase before and after methylprednisolone pulse therapy. The signal intensity ratios (SIRs) of muscles and brain white matter were calculated.Result: The mean SIRs before treatment were 2.28±0.74 in SR, 2.66±0.57 in IR, 2.03±0.42 in LR, 2.45±0.49 in MR, and 1.98±0.48 in SO muscles. The mean SIRs after treatment were 1.82±0.62, 1.84±0.52, 1.70±0.35, 1.95±0.46, and 1.60±0.36, respectively. The mean volumes (cm3) before treatment were 1.35±0.67 in SR, 1.21±0.39 in IR, 0.66±0.13 in LR, 0.94±0.31 in MR, and 0.58±0.14 in SO muscles. Those after treatment were 1.12±0.45, 0.91±0.31, 0.61±0.12, 0.85±0.28, and 0.49±0.11, respectively. The SIRs showed significantly positive correlations with volumes both before and after treatment in SR muscles (before, r=0.77; after, r=0.69), IR muscles (before, r=0.65; after, r=0.60), MR muscles (before, r=0.69; after, r=0.73), and SO muscles (before, r=0.52; after, r=0.50) (P

Kawasaki disease (KD) is an acute multi-system vasculitis syndrome of unknown etiology occurring mostly in infants and children younger than 5 years of age. In developed countries, it is the leading cause of acquired heart disease in children. However, KD remains a mysterious disease. Some viruses potentially causing the condition have been isolated, but the results have not been able to be reproduced. This article reviews and summarizes different aspects of KD and provides updated information on diagnosis and treatment. The supplementary criteria for incomplete presentation of KD patients suggested by the American Heart Association, treatment (including tumor necrosis factor-alpha antagonist, methylprednisolone pulse therapy, statins, plasma exchange, and cytotoxic agents) for those with intravenous immunoglobulin treatment failure, and other experiences are also included in this review.

信州大学博士（医学）・学位論文・平成24年3月31日授与（甲第914号）・内藤康介, Background Neuralgic amyotrophy (NA) is a distinct peripheral nervous system disorder characterized by attacks of acute neuropathic pain and rapid multifocal weakness and atrophy unilaterally in the upper limb. The current hypothesis is that the episodes are caused by an immune-mediated response to the brachial plexus, however, therapeutic strategies for NA have not been well established. Methods and Results We retrospectively reviewed 15 case series of NA; 10 of the 15 patients received intravenous immunoglobulin (IVIg) with methylprednisolone pulse therapy (MPPT) and 9 of these10 patients showed clinical improvement of motor impairment. Conclusion Our clinical observations do not contradict the possibility that IVIg with MPPT may be one of the potential therapeutics for NA, however the efficacy remains to be established. Further confirmatory trials are needed in patients with various clinical severities and phases of NA. Further basic research and confirmatory trials should be performed to survey the efficacy of such immunomodulation therapy for NA., Article, INTERNAL MEDICINE. 51(12):1493-1500 (2012)

Background: There is no clear consensus as to which patients with Henoch-Schönlein purpura nephritis (HSPN) at risk of a poor outcome should be treated and what therapeutic regimen should be used. Methods: Nine children with heavy proteinuric HSPN received prompt initiation of methylprednisolone pulse therapy (MPT) combined with tonsillectomy in a prospective study. Results: At presentation, the mean values for the patients’ urine protein excretion (early-morning urinary protein/creatinine ratio), serum IgA, activity index (AI), and chronicity index (CI) were 5.0 ± 5.6 g/g Cr, 135.6 ± 56.5 mg/dl, 4.0 ± 0.7, and 1.7 ± 1.3, respectively. At the second biopsy, conducted approximately 24 months after initiation of therapy, the patients’ serum albumin had significantly increased (4.4 ± 0.2, p < 0.01), and the serum IgA and AI had significantly decreased (88.1 ± 30.8 mg/dl, p < 0.05; 2.0 ± 1.2, p < 0.01, respectively), whereas the CI remained unchanged. Proteinuria disappeared within 24 months in all but 1 patient, and hematuria disappeared within 38 months in all patients. No patient showed renal impairment or experienced a recurrence and/or exacerbation of HSP/HSPN. Conclusions: Early treatment with MPT combined with tonsillectomy is effective in ameliorating the histopathological progression and improving the clinical course of children with heavy proteinuric HSPN.

Many treatments induce remission in patients with alopecia areata. Systemic steroids, for example, are effective in the treatment of severe alopecia areata but have many side-effects. To avoid these side-effects, high-dose bolus infusions of methylprednisolone have been used to treat severe alopecia areata. The purpose of this study was to evaluate the prognostic factors associated with pulse therapy and to establish proper indications for methylprednisolone pulse therapy. Seventy patients with severe alopecia areata were treated i.v. with methylprednisolone on 3 consecutive days. All of the patients had rapid and extensive hair loss with the bald area exceeding 50% of the scalp. Seventy percent of the patients showed terminal hair growth and 41.4% showed complete responses with acceptable cosmetic outcomes. The prognostic factors that influenced successful outcome were the disease duration before treatment and the type of alopecia areata. Based on these two factors, a good response was obtained for all types of alopecia areata with a duration of 3 months or less before treatment and for the plurifocal type of alopecia areata with a duration of 4–6 months. Methylprednisolone pulse therapy is indicated for those alopecia areata patients who fall within our good response group.

Glucocorticoids (steroids) have been widely used for the treatment of patients with rheumatoid arthritis (RA) since Hench had attempted to administer cortisone (Kendall's compound E) to an active RA patient in 1948. Rheumatologists even in the 21st century can learn a lot from the history of steroid. In this feature article on steroid, a brief outline of 11β-hydroxysteroid dehydrogenase type 1, a tissue-specific regulator of steroid response, is presented. The isozyme re-activates inactive cortisone (compound E) to active cortisol (compound F), and seems to play an important role particularly in adipose tissue. In addition, I give an account of non-genomic mechanisms of steroid, which might be relevant to early and rapid effects during methylprednisolone pulse therapy. As for the field of practical rheumatology, rates and dosages of steroid administration for RA in Japan are shown, by looking into 3 large observational cohort researches and post-marketing surveillance programs for several biologics. The definition or an appropriate interpretation of medical/technical terms such as 'effectiveness' in the clinical setting and 'low-dose' steroid is also described.

A meta-analysis of published trials on the treatment of alopecia areata (AA) came to the conclusion that most have been reported poorly and so small that any important clinical benefits are inconclusive, and considering the possibility of spontaneous remission, especially for those in the early stages of the disease, the options of not being treated or depending on individual preference, and of wearing a wig may be alternative ways of dealing with the condition. And yet, from clinical practice, we know that depending on patient age, surface area, disease duration, and comorbidities an empiric treatment algorithm can be designed that is successful in a significant proportion of patients. More recently, it has been suggested that Janus kinase (JAK) inhibitors may represent the drug of choice for AA, based on robust scientific background and preliminary clinical study results. The fact is that a sophisticated treatment of AA cannot be reduced to one drug, while in many patients, depending on disease duration and surface area, either intravenous methylprednisolone pulse therapy, intralesional triamcinolone acetonide or subcutaneous methotrexate will achieve remission rates in the range of the efficacy of the JAK inhibitors. Moreover, at this time point, affordability of the JAK inhibitors for long-term treatment, sustainability of treatment result, and long-term safety are major issues with regard to the treatment of AA with JAK inhibitors.

ObjectiveParaquat is a highly toxic herbicide that binds strongly to tissue and causes high mortality rates due to pesticide intoxication in Taiwan. In this study, we evaluated the usefulness of methylprednisolone pulse therapy and calculation of the severity index of paraquat poisoning (SIPP) to predict the prognosis in patients with oral paraquat intoxicationMaterials and MethodsThirty-two patients with paraquat poisoning from January 2003 to April 2005 were enrolled into this study at a medical center in eastern Taiwan. All 32 patients had history of oral intake of paraquat and urine paraquat was positive at the emergency department. Time of oral intake of paraquat and serum paraquat levels were assayed at the emergency department for calculating SIPP (hour × mg/L) level. Sixteen patients with oral paraquat poisoning were treated with intravenous methylprednisolone 1 g/day and charcoal hemoperfusion for 3 days (MP group), and 16 patients with oral paraquat poisoning were treated with charcoal hemoperfusion only for 3 days (control group).ResultsThe mortality rate of the patients with oral paraquat poisoning was high (87.5%). There were no statistically significant differences in death (p = 1.000), age (p = 0.706), sex (p = 0.069), serum blood urea nitrogen (p= 0.104), creatinine (p= 0.174), aspartate aminotransferase (p= 0.083), alanine aminotransferase (p = 0.365), plasma level of paraquat (p = 0.880) and SIPP level (p = 0.734) between the MP group and control group. Young age (p = 0.030), lower initial plasma paraquat level (p = 0.002), lower serum creatinine (p = 0.009), female sex (p = 0.033), lower elapsed time from ingestion of paraquat to arrival at hospital (p = 0.035) and SIPP level less than 10 (p < 0.001) were associated with survival in patients with oral paraquat poisoning. Multivariate forward stepwise linear regression analysis of deaths showed that SIPP > 10 (hour × mg/L) (p < 0.001) was an independent predictor of death in patients with oral paraquat poisoning and explained 77.1% of the variance (R2 = 0.771).ConclusionTreatment with methylprednisolone pulse therapy did not show better results in patients with acute oral paraquat poisoning. SIPP was an independent predictor of death in patients with oral paraquat poisoning.

In this report, we describe laryngeal findings in a woman with aphonia due to diminished adduction of the vocal cord caused by rheumatoid arthritis (RA) with bilateral cricoarytenoid joint involvements. One month after treatment with methylprednisolone pulse therapy, bilateral cricoarytenoid joint involvements improved and she was able to speak normally. Although laryngeal involvement has been reported to occur frequently in severe RA cases, complaints due to cricoarytenoid arthritis as initial symptoms are comparatively unusual. There are few patients followed by otolaryngologist. However, some patients with cricoarytenoid arthritis demonstrate abduction or adduction disorder that affects the quality of life. In RA patients, careful observations of laryngeal lesions by a laryngoscope and CT scans are important, and serious laryngeal lesions must not be overlooked.

Nuclear factor-kappaB activity was analyzed in multiple sclerosis (MS) patients during the course of a methylprednisolone pulse therapy. Molecular effects were evaluated using lymphocytes derived from 20 MS patients before and after therapy and 24 healthy individuals. All patients responded to treatment clinically. The mean level of DNA-binding p65 in MS was proportionate to that of healthy controls, but was significantly decreased directly after therapy whereas the level of DNA-binding p50 was significantly elevated prior to therapy and remained unchanged. In summary, pulse therapy resulted in a decreased level of activated p65 NF-kappaB subunits leading to decreased levels of transcriptionally active pro-inflammatory NF-kappaB.

Inflammation can cause a local increase in temperature. Digital infrared thermal imaging (DITI) has been used to monitor the temperature distribution of human skin. Graves' ophthalmopathy (GO) is an autoimmune disease, and patients are treated with immunosuppressive agents if the ophthalmopathy is at an inflammatory state. The aim of the present study was to elucidate the feasibility of DITI in determining inflammatory state and follow-up effect of methylprednisolone pulse therapy in patients with GO.Digital infrared thermal imaging was used to measure local temperatures of lateral orbit (reference point), upper eyelid, caruncle, medial conjunctiva, lateral conjunctiva, lower eyelid, and cornea, and to make thermal density plots in 14 patients (28 eyes) with GO including inflammatory signs (mean clinical activity score 2.5), and 16 normal controls (32 eyes). We also performed methylprednisolone pulse therapy in 11 patients (22 eyes) with active GO, and measured clinical activity score and local temperatures before and after treatment. The focal change in temperature after treatment and the correlation between temperature variation and change in clinical activity score were then analyzed. Thermal density plots were also compared.Local temperatures of the caruncle, medial conjunctiva, lateral conjunctiva, and lower eyelid of the patients were significantly higher than those of normal controls. In the 11 GO patients treated with methylprednisolone pulse therapy, the temperatures of the caruncle, medial conjunctiva, and lower eyelid were significantly decreased after treatment. Temperature variation significantly and positively correlated with a change in clinical activity score (correlation coefficient = 0.8, n = 22, p = 0.000). The temperature decreased after treatment in patients who were responsive to methylprednisolone pulse therapy, and the thermal density plot was close to that of normal controls.Digital infrared thermal imaging might be helpful in evaluating the inflammatory state of GO and the follow-up effect of methylprednisolone pulse therapy.

The purpose of this study is to reveal the effect of Erhaungfang on relapsing multiple sclerosis (MS) and investigate the underlying mechanism.In our 2-year, randomized study, a total of 67 patients (diagnosed as MS) with age from 15 to 60 years were enrolled in this study. All patients in the acute phase were treated with methylprednisolone pulse therapy and then were randomly divided into treatment group (n = 50) administrated with erhuangfang, and control group (n = 43) without nothing. Magnetic resonance spectroscopy (MRS) was used to detect N-acetylaspartate (NAA)/creatine (CR) levels. At the end of a 2-year follow-up period, relapse rate, annual relapse rate, expanded disability status scale (EDSS) scores, and NAA/CR levels were compared between groups.Relapse rate and annual relapse rate in the treatment group were significantly reduced after erhuangfang treatment (P 0.05). There was no significant difference in terms of EDSS score between treatment group and control group and between after treatment and before treatment (P 0.05). N-acetylaspartate/CR level was significantly correlated with the disease course (P 0.05). There was no significant difference for NAA/CR levels in the treatment group before and after treatment (P 0.05).Erhuangfang significantly reduced relapse rate and prevented progression of MS, which is an effective therapy for relapsing MS.

Sjogren’s syndrome is primarily a chronic systemic autoimmune disease that involves many systems. However, Sjogren’s syndrome involving the central nervous system lesions, particularly in the central nervous system symptoms as the first performers is uncommon. In this report, we present a 33-year-old woman having PSs with CNS involvement, whose first symptoms presented progressive weakness on the left leg, 2 years later the left upper limb appeared the same symptoms, being diagnosed with Sjogren’s syndrome. To give methylprednisolone pulse therapy, combined with cyclophosphamide and anetholtrithione treatment, her condition improved. After six months’ following-up, no recurrence.

Background: High-dose ( pulse) corticosteroid therapy has been associated with the development of atrial fibrillation. This association, however, is mainly based on case reports. Methods: To test the hypothesis that high-dose corticosteroid exposure increases the risk of new-onset atrial fibrillation, we performed a nested case-control study within the Rotterdam Study, a population-based cohort study among 7983 older adults. Cases were defined as persons with incident atrial fibrillation between July 1, 1991, and January 1, 2000. Their date of diagnosis was defined as the index date. All noncases within the Rotterdam Study who were alive and eligible on this index date were used as controls. Subsequently, we compared the proportion of cases and controls that received a corticosteroid prescription within 1 month preceding the index date. Corticosteroid exposure was categorized into high-dose exposure ( oral or parenteral steroid at a daily dose >= 7.5 mg of prednisone equivalents) and low-intermediate-dose exposure (

A 32-year-old man with a history of adult-onset asthma and a high level of P-ANCA and eosinophilia was referred to our hospital because of renal insufficiency. Renal and skin biopsy specimens confirmed a diagnosis of Churg-Strauss Syndrome (CSS). Treatment with steroid therapy alone including methylprednisolone pulse therapy was unsatisfactory, so additional treatment with cyclophosphamide was started. Three days later, he presented with convulsions and consciousness disturbance. MRI of the brain demonstrated bilateral whitematter abnormality that was characteristic of reversible posterior encephalophathy syndrome (RPES). Since the activity of CSS was still high, immunosuppressive therapy was continued combined with antihypertensive therapy. His consciousness was soon recovered and MRI findings improved later. RPES is an uncommon complication of CSS. In this case RPES might have been associated with CNS vasculitis.

Lymphocytic hypophysitis is a rare inflammatory disorder in the pituitary gland. The lesion is usually confined to the adenohypophysis. Although the involvement of the posterior pituitary gland or the stalk is rare, such patients with diabetes insipidus have been reported. Surgery has been used to make the definitive diagnosis. Recent studies suggest, however, that the pathologic diagnosis may not be necessary always. We reported a case of Lymphocytic hypophysitis managed by methylprednisolone pulse therapy. A 50-year-old premenopausal woman with Lymphocytic hypophysitis and diabetes insipidus was treated with methylprednisolone pulse therapy. Her adenopituitary lesion disappeared and the diabetes insipidus resolved. The optimal management for patients with lymphocytic hypophysitis may be the high index of the suspicion prior to the extensive surgical resection. In addition, methylprednisolone pulse therapy may improve the clinical and MRI findings.

Methylprednisolone pulse therapy (MPT) was introduced to avoid life-threatening complications in systemic lupus erythematosus (SLE) with brain manifestations. However, its efficacy in SLE patients remains uncertain and needs to be objectively evaluated. In this study, technetium-99m ethyl cysteinate dimer (Tc-99m ECD) brain single photon emission computed tomography (SPECT) was used to detect regional cerebral blood flow (rCBF) in SLE patients with normal brain magnetic resonance imaging (MRI) findings. Twelve female SLE patients with neuropsychiatric symptoms were enrolled in this study. All patients had normal brain MRI and abnormal Tc-99m ECD brain SPECT findings. The Tc-99m ECD brain SPECT studies were performed 2 weeks after MPT. Pre- and post-MPT serum levels of anticardiolipin antibodies (ACA) and antiribosomal P antibodies (anti-P) were also measured. Before MPT, four patients had positive ACA and seven had positive anti-P. After MPT, all 12 patients demonstrated negative serologic findings and no neuropsychiatric symptoms. After MPT, ten patients showed complete recovery and two showed partial recovery of rCBF in the follow-up Tc-99m ECD brain SPECT images. This imaging is a logical and objective tool for measuring the effects of MPT in SLE patients with brain involvement by the determination of rCBF changes.

A 43-year-old woman was diagnosed with pulmonary hypertension (PH) associated with systemic sclerosis (SSc). On admission, absence of both thromboembolism and severe interstitial lung disease suggested that vascular damage led to PH. In addition, the patient met the criteria for the classification of SLE. Methylprednisolone pulse therapy was effective not only for the clinical symptoms but also the laboratory findings. The report of overlap syndrome associated with PH is rare. The prognosis of patients with collagen diseases associated PH has been reported to be extremely poor. Since PH in this case responded favorably to methylprednisolone pulse therapy, this therapy may be effective for PH associated with overlap syndrome.

This retrospective study included 54 children with epilepsy. The treatment consisted of four pulses with single doses of 20 mg/kg/d methylprednisolone (MPR), administered every week on 3 consecutive days. After this initial phase, the intervals between the pulses were increased based on individual factors. MPR pulses were administered exclusively orally in 39 patients and 7.8% of all pulses were applied intravenously. After four pulses, 30 of 54 (56%) patients were responders, according to several clinical and electroencephalography criteria. A response was obtained in 12 of 20 (60%) cases with genetic, 7 of 17 (41%) with structural metabolic, and 11 of 17 (65%) with unknown etiology. Responder rates were 11 of 15 (73%) in patients with continuous spike–waves in slow sleep (CSWS) or Landau–Kleffner syndrome, 2 of 6 in patients with myoclonic astatic epilepsy or Lennox–Gastaut syndrome, and 17 of 31 (55%) in patients with unclassified epilepsies. A response was not correlated with any epilepsy-related clinical factor. The patients received a median of eight MPR pulses (range, 1–52), and the median duration of the therapy was 11 weeks. The response was maintained in 19 of 30 (63%) patients, and 3 of 24 (13%) without initial response became seizure-free (total responder rate at the end of the therapy 22/54 [41%]). The majority of patients experienced adverse effects that were typically mild and transient.

To quantitatively evaluate the efficacy of methylprednisolone pulse therapy for extraocular muscle inflammation in thyroid-associated ophthalmopathy (TAO) using the short-tau inversion-recovery (STIR) technique of magnetic resonance imaging (MRI). The signal intensities of the superior rectus (SR), inferior rectus (IR), lateral rectus (LR), medial rectus (MR), and superior oblique (SO) muscles were measured using the STIR images from 34 eyes of 17 patients with TAO before and after methylprednisolone pulse therapy and 19 eyes of 19 controls. The signal intensity ratio (SIR) of the signal intensity in muscles to that in brain white matter was calculated. The mean SIRs of the controls were 1.08 ± 0.26 in the SR, 1.32 ± 0.29 in the IR, 1.34 ± 0.19 in the LR, 1.47 ± 0.25 in the MR, and 1.28 ± 0.22 in the SO muscles. SIRs exceeding 2.0 were out of the normal range. The SIRs of the patients with TAO before treatment were 2.19 ± 0.64, 2.44 ± 0.58, 1.96 ± 0.43, 2.24 ± 0.47, and 1.91 ± 0.42, respectively, which was significantly (P

EDITOR: We read with great interest the article by Hsu and colleagues2013 describing a 28‐year‐old Chinese man with gradual bilateral visual loss treated with methylprednisolone pulse therapy. The ...

We present the cases of two patients (19- and 40-year-old women) with systemic lupus erythematosus (SLE) who showed marked elevation of C-reactive protein (CRP). In both patients, pleural and/or peritoneal effusions were caused by lupus serositis. Methylprednisolone pulse therapy was effective in improving the serositis and normalizing CRP. Although it is generally considered that the CRP response is relatively weak in lupus patients, these cases suggest that a strong CRP response can occur in a subset of SLE.

Abstract: Thrombotic thrombocytopenic purpura (TTP) is rarely associated with Sjögren's syndrome (SS) with only 12 previously reported cases in the literature. All of them were women. A case of a previously healthy 64-year-old Chinese male presenting with microangiopathic hemolytic anemia, otherwise unexplained thrombocytopenia, and central nervous system involvement is reported. At the beginning the patient's family refused plasma exchange (PE) therapy due to cost consideration, later plasma infusion (PI) was initiated along with methylprednisolone pulse therapy. Significant clinical and laboratory response was achieved. However, recurrence happened after discontinuing the treatment for only three weeks without consulting his doctor. Then the patient received another two cycles of therapy of PE with again an excellent clinical and laboratory response. The patient was discharged in a state of remission on methylprednisolone and thymopolypeptides. In order to prevent further complications, prophylactic infusions of plasma was performed per month. The patient remained asymptomatic thereafter. Autoimmune workup revealed positive antinuclear antibodies and Anti-SSA. Lip biopsy revealed findings consistent with SS. TTP with SS tends to be recurrence. PE along with immunosuppressive therapy followed by prophylactic PI can prevent long-term organ complications in these cases. Disclosures No relevant conflicts of interest to declare.

purpose 1) To test the short- and long-term efficacy of Methylprednisolone Pulse Therapy (MPT) for patients with Dysthyroid Optic Neuropathy (DON). 2) To assess possible side-effects of MPT. 3) To assess whether a delay in decompressive surgery would influence the visual outcome. study design Retrospective, uncontrolled. method The records of 62 consecutive patients with DON, who received MPT between 1994 and 2000, were evaluated with special attention for pre- and post treatment ophthalmic and orthoptic parameters, Clinical Activity Score (CAS), visual fields and visual evoked potentials, and treatment complications. DON-patients were hospitalized and received four times 500 mg methylprednisolone intravenously. Immediately afterwards they were treated with oral prednisone (maximal dose 60 mg) and/or orbital irradiation (10 times 2 Gy). Evaluation was done one day after the last bolus (T1) and when the orbitopathy had been stable for at least six months (T2). results Visual acuity, proptosis, elevation and CAS all improved significantly at T1, whereas the lid aperture did not change. At T2, 24 out 62 (39%) DON-patients were stable with normal vision. The other 38 (61%) had undergone orbital decompression because of persistent or recurrent DON within one week to six months after MPT. Final visual acuity in the whole group (121 eyes) was less than 0.1 in 3 eyes, between 0.1 and 0.5 in 17 and more than 0.5 in 101 eyes. Treatment complications were: pylorus ulcer (n = 1), increased osteoporosis (n = 1), abcess formation (n = 1), and increase in insulin dosage (n = 1). conclusions 1) MPT is efficious as emergency treatment. 2) One-third of DON-patients are spared decompressive surgery. 3) MPT causes only rare and no serious side-effects. 4) The final visual outcome after MPT and orbital decompression is comparable to the results of immediate decompression.

A 10 yr old male with hypoxaemia, progressive infiltration on the chest radiograph and biopsy-proven desquamative interstitial pneumonia was treated with daily oral prednisolone for 6 months. Intravenous methylprednisolone pulses were concomitantly administered in doses averaging 10 mg x kg body weight(-1) on three consecutive days every 4-6 weeks. After 6 months improvement could be noted and oral steroids were stopped, while pulse therapy continued. Three months later, when seven pulses had been administered, a relapse occurred and the clinical status deteriorated. Instead of reinstating daily systemic steroids, the dose of methylprednisolone pulses was increased to 20 mg x kg body weight(-1) i.v. given on three consecutive days and repeat pulses every 4 weeks. This was followed by continuous improvement. After 24 months corticosteroid pulses were terminated. Normal lung function, serum lactate dehydrogenase, blood gases upon exertion and regular development was achieved. During the course of treatment, the child has grown 10 cm. It is concluded that the effect of corticosteroid pulse therapy on interstitial lung disease in childhood is dose-dependent and that the dose can be adjusted to the effect observed.

A 70-year-old man was admitted to our hospital because of slowly progressive anemia and thrombocytopenia. He was diagnosed as having myelodysplastic syndrome (MDS) (FAB classification: refractory anemia with excess blasts in transformation, RAEB-T) by bone marrow examination. Because treatment with cytotoxic agents was not effective in improving the pancytopenia, pulse therapy using methylprednisolone was performed. After pulse therapy, marked leukocytosis appeared and was controlled by cytotoxic agents.One month after the pulse therapy,the patient experienced disorientation,headache and muscle weakness.A brain computed tomographic scan revealed multiple mass lesions in the cerebrum.No other malignancy except MDS was detected. Cerebrospinal fluid examination revealed an increase in leukemic blast cells in the fluid.He was therefore diagnosed as having metastasis of granulocytic sarcoma in the cerebrum,which was sensitive to a total cranial radiation therapy of 30Gy. MDS rarely complicates extra-medullary tumors before developing into acute leukemia.We presented here a rare case of MDS with multiple intracranial mass lesions which were sensitive to radiation., Article, 信州医学雑誌 47(5): 381-388(1999)

To clarify the effectiveness of methylprednisolone pulse therapy on crescentic glomerulonephritis (CresGN), data of 46 patients was retrospectively studied. According to the slope of reciprocals of serum creatinine (s-Cr) the patients were divided into acute and insidious types, and each type was further divided into pulse (P) and conventional (C) therapy groups. In group C, s-Cr improved more frequently in the acute type (8/10) than in the insidious type (1/9, p

Progressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disease of the central nervous system caused by an opportunistic infection of the JC virus. PML is known to occur in immunocompromised patients. A patient with systemic lupus erythematosus (SLE) who developed PML during i.v. cyclophosphamide and methylprednisolone pulse therapy is described. The pertinent literature relating to complication of SLE by PML is discussed.