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Background: In 2012, we clarified that the prevalence of food-dependent exercise-induced anaphylaxis (FDEIA) was 0.018% in junior-high students in Yokohama, Japan. Furthermore, although different from FDEIA, one student who had completed oral immunotherapy developed anaphylaxis during exercise after eating causative food. There have been few large-scale epidemiological studies of FDEIA, however, in elementary school children, therefore we conducted an epidemiological study in elementary school children in Yokohama to clarify the frequency and characteristic of FDEIA., Methods: We sent a questionnaire regarding the occurrence of FDEIA to all 348 public elementary school nurses in Yokohama. We also compared the results with those for junior-high school that we previously reported. We excluded those children with a past history of immediate food allergy who had achieved desensitization status after oral immunotherapy, from FDEIA, and instead defined them as having desensitization status and exercise-induced anaphylaxis (DEIA)., Results: Of 348 school nurses, 317 responded (91.1%). Overall, eight of 170 146 children were diagnosed with FDEIA, which was significantly lower than the prevalence in junior-high school students (0.0047% vs 0.018%, P = 0.0009). The causative foods were wheat (n = 4), and soy, fruit, crustaceans, and squid (n = 1 each). Four children had DEIA and the causative foods were wheat and milk (n = 2 each). Multiple episodes occurred in five children with FDEIA and in three children with DEIA., Conclusions: FDEIA was far less common in elementary school than in junior-high school, and wheat was the major causative food. The new appearance of DEIA was notable. Decreasing episode recurrence remains an issue that needs to be resolved., (© 2018 Japan Pediatric Society.)

Purpose: To suggest an objective score for grading the acute ocular severity of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), and to determine predictive factors for severe acute ocular involvement such as ocular surface epithelial defect and/or pseudomembrane formation., Design: Retrospective cohort study., Methods: The medical records of SJS (n = 87) and TEN (n = 48) patients between 2005 and 2007 were reviewed. An acute ocular severity score was determined on a scale from 0 to 3 (none, mild, severe, and very severe) according to the existence of hyperemia, corneal or conjunctival epithelial defect, and pseudomembrane formation. The associations between the severe acute ocular involvement and factors such as patient age, exposed drugs, systemic severity, and the prevalence of ocular sequelae were examined., Results: The number of cases with score grade 0, 1, 2, and 3 was 19 (21.8%), 31 (35.6%), 22 (25.3%), and 15 (17.2%) in 87 SJS cases and 12 (25.0%), 11 (22.9%), 17 (35.4%), and 8 (16.7%) in 48 TEN cases. Multivariate logistic regression analysis revealed that patient age (odds ratio [OR], 0.98; 95% confidence interval [CI], 0.96-0.99; P = .007) and nonsteroidal anti-inflammatory drugs NSAIDs or cold remedies (OR, 2.58; 95% CI, 1.26-5.29; P = .010) were predictive factors for severe acute ocular involvement. The prevalence of visual disturbance and eye dryness increased according to the increase of acute ocular severity (P = .001 and P = .007 in SJS; P = .007 and P = .014 in TEN, respectively)., Conclusions: At the onset of SJS/TEN, strict attention should be paid to ocular involvement in young patients and in patients exposed to NSAIDs or cold remedies., (Copyright © 2015 Elsevier Inc. All rights reserved.)

A food allergy is defined as "a phenomenon in which adverse reactions are caused through antigen-specific immunological mechanisms after exposure to given food." Various symptoms of food allergy occur in many organs. Food allergies are classified roughly into 4 clinical types: (1) neonatal and infantile gastrointestinal allergy, (2) infantile atopic dermatitis associated with food allergy, (3) immediate-type food allergy (urticaria, anaphylaxis, etc.), and (4) food dependent exercise-induced anaphylaxis and oral allergy syndrome (i.e., specific forms of immediate food allergy). The therapy for food allergies includes treatment of and prophylactic measures against hypersensitivity such as anaphylaxis. A fundamental prophylactic measure is the elimination diet. However, elimination diets should be used only if necessary because of the patient-related burden. For this purpose, it is very important that causative foods be accurately identified. There are a number of means available to identify causative foods, including the history taking, a skin prick test, detection of antigen-specific IgE antibodies in the blood, the basophil histamine release test, the elimination diet test, and the oral challenge test, etc. Of these, the oral challenge test is the most reliable. However, it should be conducted under the supervision of experienced physicians because it may cause adverse reactions, such as anaphylaxis.

A new version of the Japanese pediatric guideline for the treatment and management of bronchial asthma was published in Japanese at the end of 2011. The guideline sets the pragmatic goal for clinicians treating childhood asthma as maintaining a "well-controlled level" for an extended period in which the child patient can lead a trouble-free daily life, not forgetting the ultimate goal of obtaining remission and/or cure. Important factors in the attainment of the pragmatic goal are: (i) appropriate use of anti-inflammatory drugs; (ii) elimination of environmental risk factors; and (iii) educational and enlightening activities for the patient and caregivers regarding adequate asthma management in daily life. The well-controlled level refers to a symptom-free state in which no transient coughs, wheezing, dyspnea or other symptoms associated with bronchial asthma are present, even for a short period of time. As was the case in the previous versions of the guideline, asthmatic children younger than 2 years of age are defined as infantile asthma patients. Special attention is paid to these patients in the new guideline: they often have rapid exacerbation and easily present chronic asthmatic conditions after the disease is established., (© 2014 Japan Pediatric Society.)

A food allergy is defined as "a phenomenon in which adverse reactions are caused through antigen-specific immunological mechanisms after exposure to given food." Various symptoms of food allergy occur in many organs. Food allergies are classified roughly into 4 clinical types: (1) neonatal and infantile gastrointestinal allergy, (2) infantile atopic dermatitis associated with food allergy, (3) immediate-type food allergy (urticaria, anaphylaxis, etc.), and (4) food dependent exercise-induced anaphylaxis and oral allergy syndrome (i.e., specific forms of immediate food allergy). The therapy for food allergies includes treatment of and prophylactic measures against hypersensitivity such as anaphylaxis. A fundamental prophylactic measure is the elimination diet. However, elimination diets should be used only if necessary because of the patient-related burden. For this purpose, it is very important that causative foods be accurately identified. There are a number of means available to identify causative foods, including the history taking, a skin prick test, detection of antigen-specific IgE antibodies in the blood, the basophil histamine release test, the elimination diet test, and the oral challenge test, etc. Of these, the oral challenge test is the most reliable. However, it should be conducted under the supervision of experienced physicians because it may cause adverse reactions, such as anaphylaxis., (© 2014 Japanese Society of Allergology.)

Abstract The fourth version of the Japanese Pediatric Guidelines for the Treatment and Management of Bronchial Asthma 2008 (JPGL 2008) was published by the Japanese Society of Pediatric Allergy and Clinical Immunology in December 2008. In JPGL 2008, the recommendations were revised on the basis of the JPGL 2005. The JPGL 2008 is different to the Global Initiative for Asthma guideline in that it contains the following items: a classification system of asthma severity; recommendations for long-term management organized by age; a special mention of infantile asthma; and an emphasis on prevention and early intervention. Here we show a summary of the JPGL 2008 revising our previous report concerning JPGL 2005.

Sarcoidosis is a systemic disease of unknown etiology that is characterized by chronic non-caseating granulomatous inflammation with tissue destruction. It is an uncommon disease in children, and renal sarcoidosis in particular is very rare in adults and children. A 17-year-old boy with renal sarcoidosis was referred to our hospital with an initial diagnosis of pyelonephritis. Prior treatment with various antibiotics had not been effective, but tentative oral daily prednisolone (PSL) had partially ameliorated his symptoms, although the symptoms recurred during steroid tapering. We detected non-caseating granulomatous interstitial nephritis and numerous sclerotic glomeruli in a second biopsy specimen, compatible with the diagnosis of renal sarcoidosis. The patient was treated with pulsed methyl-prednisolone and oral daily doses of PSL and mizoribine (MZR). During the treatment with MZR, the PSL was successfully tapered, and the patient has since presented no signs of recurrence. Our treatment of this patient shows that treatment with MZR can allow steroid sparing and prevent recurrence in a patient with sarcoidosis.

Background: Whole blood interferon-gamma assay QuantiFERON-TB2G (QFT-2G), which is a new specific method for diagnosing tuberculosis (TB), has been developed and used in the clinical field. The aim of the present study was to assess the usefulness of QFT-2G as an indicator, both for diagnosing childhood TB and for assessing therapeutic effectiveness., Methods: The subjects were 61 children introduced to the TB outpatient department for the first time between June 2004 and March 2006. QFT-2G, the tuberculin test and chest computed tomography (CT) were performed for all patients., Results: Ten patients having typical characteristics of primary tuberculosis (PTB) on chest CT, and diagnosed as having tubercle bacillus infections, all had positive reaction on QFT-2G. Of seven patients who had no abnormalities on diagnostic imaging but who reacted positively on QFT-2G, one developed TB later, and no TB was detected over the period of observation in 44 patients with negative QFT-2G at their first consultation. Moreover, four patients with non-tuberculous acid-fast bacilli in which Mycobacterium avium or Mycobacterium gordonae was detected had negative reaction on QFT-2G. In addition, all 10 patients with positive reactions on QFT-2G in whom the subsequent course of the disease was observed had decrease on QFT after treatment., Conclusions: QFT-2G is a powerful tool with a wide application both in diagnosis and in assessment of treatment effectiveness in PTB.

Kawasaki disease is a generalized vasculitis of unknown etiology that occurs predominantly in infants and young children. It is very important to prevent its cardiovascular manifestations, especially coronary artery lesions. Early treatment with intravenous immunoglobulin reduces cardiovascular sequelae, but some patients do not respond to this treatment, and they have a high incidence of coronary artery lesions. On the other hand, acute heart failure is rare in Kawasaki disease. We report on the cases of two patients with persistent fever and shock even after intravenous immunoglobulin therapy. In both cases, plasma exchange may have reduced the risk of coronary artery lesions and proved effective against acute heart failure with catecholamine-refractory shock; yet the mechanism of this improvement remains unclear.

Background: Systemic-onset juvenile idiopathic arthritis does not always respond to available treatments, including antitumour necrosis factor agents. We investigated the efficacy and safety of tocilizumab, an anti-interleukin-6-receptor monoclonal antibody, in children with this disorder., Methods: 56 children (aged 2-19 years) with disease refractory to conventional treatment were given three doses of tocilizumab 8 mg/kg every 2 weeks during a 6-week open-label lead-in phase. Patients achieving an American College of Rheumatology Pediatric (ACR Pedi) 30 response and a C-reactive protein concentration (CRP) of less than 5 mg/L were randomly assigned to receive placebo or to continue tocilizumab treatment for 12 weeks or until withdrawal for rescue medication in a double-blind phase. The primary endpoint of the double-blind phase was an ACR Pedi 30 response and CRP concentration of less than 15 mg/L. Patients responding to tocilizumab and needing further treatment were enrolled in an open-label extension phase for at least 48 weeks. The analysis was done by intention to treat. This study is registered with ClinicalTrials.gov, numbers NCT00144599 (for the open-label lead-in and double-blind phases) and NCT00144612 (for the open-label extension phase)., Findings: At the end of the open-label lead-in phase, ACR Pedi 30, 50, and 70 responses were achieved by 51 (91%), 48 (86%), and 38 (68%) patients, respectively. 43 patients continued to the double-blind phase and were included in the efficacy analysis. Four (17%) of 23 patients in the placebo group maintained an ACR Pedi 30 response and a CRP concentration of less than 15 mg/L compared with 16 (80%) of 20 in the tocilizumab group (p<0.0001). By week 48 of the open-label extension phase, ACR Pedi 30, 50, and 70 responses were achieved by 47 (98%), 45 (94%), and 43 (90%) of 48 patients, respectively. Serious adverse events were anaphylactoid reaction, gastrointestinal haemorrhage, bronchitis, and gastroenteritis., Interpretation: Tocilizumab is effective in children with systemic-onset juvenile idiopathic arthritis. It might therefore be a suitable treatment in the control of this disorder, which has so far been difficult to manage.

Henoch-Schonlein purpura (HSP) is an inflammatory vasculitis involving the skin, joints, gastrointestinal (GI) tract, and kidneys. This is the first case report describing a 5-year-old girl with HSP presenting duodenal involvement which might be associated with superior mesenteric artery syndrome (SMAS).

Food-dependent exercise-induced anaphylaxis (FEIAn) is a rare disease. The disease is classified into physical allergy and/or a subtype of food allergy. However, the pathophysiology and epidemiology are not well known. In this article we presented our studies of the epidemiology of FEIAn, in school students (elementary. junior-high, and high school) in Yokohama City and/or Kanagawa Prefecture, Japan. Also, we reviewed FEIAn cases in the literatures in Japan as well as in foreign countries. Six hundred and seven school nurses responded the questionnaire, 30 (Male:Female, 24:6) cases of FEIAn and 44 (M:F, 22:22) with exercise-induced anaphylaxis (EIAn) among 353977 students were reported. The frequency of FEIAn and EIAn was 0.0085% and 0.012%, respectively. Only one third of nurses had knowledge of FEIAn. We performed provocation tests in 11 of the 30 students with FEIAn and confirmed the diagnosis. We demonstrated transient increases in plasma histamine but not serum tryptase levels during the tests. The causative foods were mainly wheat and crustaceans in 170 Japanese cases and beans and wheat in foreign countries. As for exercise, ball games and running were more frequent sports in both groups. The age of first episode of FEIAn was 10 to 20 years old in most cases. Provocation test often fails to induce symptoms. In addition, we do not have the standard method. Now we are establishing the protocol. FEIAn and EIAn are relatively rare among school students in Japan. There is no evidence to prevent the onset of FEIAn with medicines. Therefore, to avoid serious outcomes and unnecessary restriction, we believe it is important to be familiar with the diseases for not only physicians, but also school nurses and teachers of physical education.

We report the case of a girl with steroids and cyclosporine (CsA) resistant focal segmental glomerulosclerosis (FSGS) whose proteinuria and hypoproteinaemia were dramatically resolved by pravastatin. She had been in a nephrotic condition for 6 years. Prednisolone, pulse methylprednisolone therapy, low-density lipoprotein (LDL) apheresis, CsA, cyclophosphamide and mizoribine (MZR) had proved to be ineffective. She was started on pravastatin for her hyperlipidaemia 6 and a half years from onset, in addition to the baseline therapy, which included CsA; remission of the nephrotic syndrome was unexpectedly attained after 10 months of treatment. The baseline therapy has not been changed since the inclusion of pravastatin. This case suggests that, in patients with hyperlipidaemia, the response to CsA could be restored by lowering cholesterol levels with statins. The decrease of cholesterol levels might have improved the pharmacokinetics of CsA in this patient. Furthermore, the anti-inflammatory and immuno-modulatory effects, recently attributed to statins, may also have been involved in the improvement experienced by our patient.

We encountered the case of a 4-year-old boy with thrombotic microangiopathy (TMA) of unknown etiology. Verotoxin-induced hemolytic uremic syndrome (HUS), Streptococcus-pneumoniae-related HUS, factor H deficiency, drug-induced thrombotic thrombocytopenic purpura (TTP), and ADAMTS13 (von Willebrand factor-cleaving protease; a disintegrin-like and metalloprotease with thrombospondin type 1 repeats)-related TTP were excluded. His condition was refractory to anticoagulants and plasma exchange, and his clinical course was catastrophic, with central nervous system symptoms and progressive renal failure. However, factual treatment of intravenous gamma globulin (IVIG) ended the hemolysis and resulted in a rise in platelet count. He fully recovered except for end-stage renal failure, but he underwent a successful renal transplant after peritoneal dialysis. He has not suffered a relapse of TMA or an allograft rejection for 4 years. IVIG might be an option for some patients with TMA of unknown etiology refractory to conventional treatment.

Norovirus is a common cause of gastroenteritis. We describe the case of a 23-month-old girl with encephalopathy possibly associated with norovirus infection. The viral genome was detected in stool, serum and cerebrospinal fluid (CSF) by reverse transcription polymerase chain reaction. This is the first report of encephalopathy potentially caused by norovirus as indicated by the presence of the virus genome in CSF.

Background: Anaphylaxis is one of the severest forms of allergic diseases. Some kinds of mushroom are known as causative allergens in food anaphylaxis. Matsutake mushroom (Tricholoma matsutake) is a typical edible mushroom available in autumn in Japan. We encountered an 8-year-old Japanese girl who developed anaphylaxis after ingesting matsutake mushrooms., Methods: We studied the case in detail, by measuring specific IgE antibodies and conducting skin tests, to confirm the diagnosis. We also detected seven cytokines and chemical mediators in the blood in order to study the pathophysiology of the anaphylaxis., Results: We diagnosed anaphylaxis caused by ingestion of matsutake mushrooms based on the following. A skin prick test showed a positive reaction to matsutake mushroom, and specific IgE antibody for matsutake mushroom extract was detected in the patient's serum by fluorometric ELISA. Blood levels of chemical mediators including histamine, ECP, tryptase and cytokines such as IL-6, IL-5 and IL-10 but not IFN-gamma also increased significantly during the allergic episode., Conclusions: We demonstrated that chemical mediators including histamine, tryptase and ECP as well as several cytokines were involved significantly during the episode of anaphylaxis. In addition, eosinophils as well as mast cells played significant roles in the anaphylaxis. Furthermore, CD4+CD25+ T regulatory cells that released IL-10 were likely activated during the anaphylaxis. Matsutake mushroom should be considered as a causative allergen in food anaphylaxis.

We studied the usefulness of interferon-gamma measurement reagent QuantiFERON-TB 2 G (QFT-2G), used to diagnose tubercle bacilli infections, as an indicator both for diagnosing primary tuberculosis (PTB) and for assessing therapeutic amorg pediatric Tuberculosis Outpatent cases effectiveness. Five cases showing typical PTB findings, such as cavitation, swollen lymph nodes, and nodular shadows at the pulmonary hilum, and diagnosed as tubercle bacillus infections, all showed positive reactions to QFT-2G, and in 3 asymptomatic cases without abnormalities detected in diagnostic imaging but QFT-2G-positive, one developed tuberculosis (TB) later. Among 12 patients who gave negative reactions to QFT-2G at their first visit and during observation from 6 months to 1 year, no TB occurrences was seen. Patients who were vaccinated for BCG and were tuberculin-positive showed negative reactions to QFT-2G, confirming that QFT-2G is not affected by BCG. One case of nontuberculous acid-fast bacilli in which Mycobacterium avium was detected was QFT-2G-negative. In 1 case, QFT-2G decreased as the patient's conditiorl improved. Without being influenced by BCG vaccination, QFT-2G demonstrated its usefulness in primary TB cases both for diagnosis and for assessing treatment effectiveness. Our results strougly suggested that QFT-2G is a potentially powerful tool with wide applications in diagnosis and assessment of treatment effectiveness in primary TB, even when bacterial elimination is low and diagnosis is difficult.

Objectives: Drug-induced hypersensitivity syndrome (HS) is a rare but life-threatening disease. We experienced carbamazepine-induced HS in a 14-year-old boy, who had cefaclor-induced cutaneous eruptions 15 months later. To clarify the mechanisms of HS and the differences between two diseases we studied this case in detail., Methods: We investigated the associated viral agents by polymerase chain reaction and the specific antibodies. We also studied the mechanism of diseases by measuring chemical mediators including cytokines, ECP and immunoglobulins., Results: The patient was diagnosed as having carbamazepine-induced HS associated with reactivation of human herpesvirus 6 based on the clinical course and laboratory data including drug-induced lymphocyte stimulation tests. Similarly, the diagnosis of cefaclor-induced eruption without any viral reactivation was made. Serum levels of IFN-gamma, IL-6, TNF-alpha, IL-5 and ECP were increased significantly at HS but mildly at cefaclor-induced eruptions. Furthermore, we detected transient hypogammaglobulinemia only at HS., Conclusions: This is the first report of anticonvulsant-induced HS followed by antibiotic-induced eruptions in a patient. In addition, we demonstrated difference in serum levels of inflammatory cytokines, immunoglobulins, activated eosinophils and viral reactivation between these diseases. This case would contribute to the understanding of the pathophysiology of adverse drug reactions including HS.

We reported three cases of childhood-onset male systemic lupus erythematosus (SLE), all of whom successfully treated with a combination of pulse methylprednisolone (mPSL) and pulse cyclophosphamide (IVCY). All of them had severe lupus nephritis and were complicated with other collagen diseases. Two cases were complicated with Sjögren syndrome (SS) and the other was complicated with both SS and anti-phospholipid syndrome (APS). After a combination of pulse mPSL and IVCY for a year, followed by oral predonisolone (PSL) and azathioprine (AZA), following up renal biopsy were performed in all cases, which showed histological improvement in glomerulonephritis. One case had flares a year later, but no flares were observed either in clinical symptoms or in laboratory examinations in the others. Their autoantibodies except anti-nuclear antibody (ANA) were eliminated. We suggest a combination of pulse mPSL and IVCY is effective for the patients who are suffering with severe lupus nephritis complicated with the other collagen diseases.

Abstract One month after treatment with γ-globulin for Kawasaki disease, an 18-month-old girl developed Evans' syndrome in addition to drug-induced hypersensitivity syndrome (DIHS) after a second γ-globulin treatment. She suffered from hyperbilirubinemia, hemolytic anemia, and thrombocytopenia. The findings and her clinical course involved plasma exchange and treatment with prednisolone, with good results. Peripheral lymphocyte stimulation tests indicated that γ-globulin was the likeliest cause of the DIHS. A real-time polymerase chain reaction test showed the human herpes virus (HHV)-6 genome in peripheral blood. We demonstrated that a primary infection or infection reactivation by the HHV-6 virus was involved in the development of γ-globulin-induced hypersensitivity and Evans' syndrome.

We analyzed the effects of three therapies on 30 patients with childhood systemic lupus erythematosus, and classified the patients into three groups. The therapies were as follows; Group A (8 cases), methylpredni-solone (mPSL) pulses plus oral prednisolone (PSL) alone, Group B (10 cases), mPSL pulses plus oral PSL and mizoribine (MZB) or azathioprine (AZP), Group C (12 cases), mPSL pulses and intravenous cyclophosphamide (IVCY) pulse therapy plus oral PSL and MZB or AZP. Three years after treatment, we compared the laboratory data (C3, C4, CH50 and anti-DNA antibody), the SLEDAI scores and numbers of relapses in these three groups. We demonstrated that group C had the best data, and this data indicated that the median C3, C4 and CH50 increased and that the median anti-DNA antibody and the mean of the numbers of relapses decreased. In conclusion, the combination of immunosuppressants and IVCY appeared to offer great benefits in childhood systemic lupus erythematosus.

Abstract Three cases of childhood-onset systemic lupus erythematosus (childhood SLE) with long-term remission are reported. These cases were not complicated with collagen diseases and had no SLE disease activity index scores either 3 or 5 years after onset. We suggest that some patients with childhood SLE may be able to abandon the maintenance therapy, and that careful observation is needed for each individual case. Uniform remission criteria based on clinical trials are needed.

Pediatricians are often faced with both bad temper and decreased daily activity in children with persistent nephrotic syndrome. These problems, which might affect both mental and physical development, have been explained as a consequence of general fatigue due to systemic edema and/or long-term hospitalization. However, other factors, such as cerebral hypoperfusion, may be involved. We experienced a case of a boy with steroid-resistant nephrotic syndrome who showed diffuse cerebral hypoperfusion on single photon emission computed tomography. Diffuse cerebral hypoperfusion dramatically resolved as the nephrotic syndrome remitted. His bad temper, decreased daily activity, and delay of speech and motor development also improved. In our patient, cerebral hypoperfusion might have been associated with his mental problems, physical problems, and delayed development of speech., (Copyright 2002 Elsevier Science B.V.)

Background: Systemic lupus erythematosus (SLE) is one of the major collagen diseases in childhood. However, the pathogenesis of this disease still remains unknown. The disease is known as a chronic inflammatory disease. Since oral and intravenous corticosteroid therapy has been introduced into the treatment of SLE, the prognosis of patients has improved significantly. However, it has now become clear that there are limitations in the effectiveness, as well as adverse reactions when corticosteroids therapy is administered for a long-term period. Therefore, we have been attempting to improve the maintenance therapy of child-onset SLE., Methods: We have proposed and tested a new type of combination therapy using prednisolone (PSL) and mizoribine (MZR) in pediatric patients with SLE for maintenance therapy after the induction of remission., Results: Our results showed that this combination therapy is more effective than the previous regimen. In addition, no significant side-effects were observed in our study., Conclusion: This combination therapy is still not perfect. Efforts should be continued to establish an optimal therapy regimen for child-onset SLE.

We experienced the case of a boy suffering from acute disseminated encephalomyelitis and concomitant acute glomerulonephritis. The multiple lesions observed on MR images, which located mainly in the cortical gray matter, quickly responded to methyl prednisolone pulse therapy. Renal biopsy confirmed the diagnosis of poststreptococcal acute glomerulonephritis. Streptococcus pyogenes was identified by pharyngeal culture, and the infection was serologically confirmed. We speculated that S. pyogenes infection was coincidentally involved in both diseases.