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1 Clinical Pediatric Hematology-Oncology Volume 23 ㆍ Number 2 ㆍ October 2016 CASE REPORT 장기간데플라자코트로치료한폐혈철소증소아환자 3 예 박규정ㆍ유영명ㆍ배기수ㆍ박준은 아주대학교의과대학소아과학교실 Three Cases of Pulmonary Hemosiderosis with Long-term Treatment of Deflazacort in Children Kyujung Park, M.D., Yeongmyong Yoo, M.D., Kisoo Pai, M.D., Ph.D. and Jun Eun Park, M.D., Ph.D. Department of Pediatrics, Ajou University School of Medicine, Suwon, Korea Idiopathic pulmonary hemosiderosis (IPH) is a rare respiratory disease with an unknown etiology, and is diagnosed with laboratory, radiology, and pathology tests. Chief complaints of IPH include hemoptysis, cough, and dyspnea. Since it is considered an immune-mediated disease, the first line of treatment is systemic corticosteroid therapy. The three cases reported here showed a decrease in ferritin level and improvement in the hemoglobin level with prednisolone treatment. However, long-term corticosteroid therapy may cause several side effects, particularly growth retardation and obesity, which can affect growing children. In the present study, all patients had cushingoid symptoms and obesity. Therefore, we switched to deflazacort (DFZ), which has lesser side-effects of weight gain. This report describes clinical courses of the disease and comparison of body mass index of three patients with IPH who took DFZ instead of prednisolone. DFZ was effective for IPH, and is useful for weight gain reduction. Key Words: Idiopathic pulmonary hemosiderosis, Deflazacort, Body mass index pissn / eissn Clin Pediatr Hematol Oncol 2016;23: Received on September 13, 2016 Revised on October 2, 2016 Accepted on October 9, 2016 Corresponding Author: Jun Eun Park Department of Pediatrics, Ajou University Hospital, Ajou University School of Medicine, 164 Worldcup-ro, Yeongtong-gu, Suwon 16499, Korea Tel: Fax: pedpje@ajou.ac.kr ORCID ID: orcid.org/ Introduction Idiopathic pulmonary hemosiderosis (IPH) is a disorder characterized by the triad of hemoptysis, diffuse parenchymal infiltrates on chest radiograph, and iron deficiency anemia. Pediatric IPH is a rare respiratory disease, with an incidence of about 0.24 to 1.23 cases per million, and usually occurs before the age of 10 years [1-3]. Despite its low incidence, the clinical course of IPH is variable, and death may occur suddenly after pulmonary hemorrhage [4]. Although its etiology remains unknown, IPH is considered to be an immune-mediated disease [5]. The first line treatment is usually systemic corticosteroid therapy, which is thought to decrease the frequency of pulmonary hemorrhage. However, long term corticosteroid use can cause serious side effects including hypertension, gastritis, diabetes, secondary infections, Cushing syndrome, and obesity. Furthermore, the use of corticosteroids could impair growth and development in children. Thus, Deflazacort (Calcort, Sanofi, 188
2 Deflazacort Therapy for Idiopathic Pulmonary Hemosiderosis DFZ), which is as effective as prednisone or methylprednisone, can be used as an alternative for corticosteroid [6,7]. In this study, we reviewed three cases of pediatric patients with IPH who were treated with DFZ to reduce the side effects of obesity that accompanies prednisolone use. Case Report 1) Case 1 A 6-year-old boy admitted to the emergency room with a hemoglobin (Hb) level of 4.6 g/dl, tested at a local clinic s laboratory, had taken iron supplements, following which his Hb level increased to 6.2 g/dl. Therefore, we diagnosed his condition as iron deficiency anemia. After 6 months, the patient had manifested dyspnea with anemia. He then underwent high-resolution computed tomography (HRCT) and lung biopsy for differential diagnosis of IPH and bronchiolitis obliterans organizing pneumonia (BOOP) (Fig. 1A). Intra-alveolar accumulation of hemosiderin-laden macrophages was identified in his lung biopsy specimen, and he was then diagnosed with IPH (Fig. 1B). The patient was started on methylprednisolone pulse therapy first, and the treatment was switched to prednisolone 2 mg/kg per day until he was 9 years old. When he had several viral infections, we increased his prednisolone dosage, which resolved the symptoms at once. However, his BMI increased from to kg/m 2 during 3 years of prednisolone therapy (Fig. 2). In addition, cushingoid symptoms appeared and worsened. Therefore, we switched from prednisolone to DFZ 0.25 mg/kg per day to reduce obesity and cushingoid symptoms. After starting DFZ, his BMI dramatically decreased from to (Fig. 2). When he had viral infections, he manifested symptoms of IPH. Thus, he was treated with prednisolone therapy, briefly. After the patient switched to DFZ, he was maintained on the treatment until now. 2) Case 2 A 3-year, 1-month old girl had pale skin color for 2 Fig. 2. Body mass index of three cases. Arrow indicates the onset time of deflazacort treatment. Fig. 1. (A) Chest High-resolution computed tomography (HRCT) showed predominant bilateral ground-glass opacity on right lung. (B) Right lung tissue showed intra-alveolar accumulation of hemosiderin-laden macrophage (Hematoxylin-eosin (H&E) staining, 200). Clin Pediatr Hematol Oncol 189
3 Kyujung Park, et al weeks and fever for 3 days. Thus, complete blood cell (CBC) count was performed, revealing Hb level of 6.0 g/dl. She underwent chest radiography, which showed multifocal infiltration on both lung fields and purified protein derivative skin test that showed a negative result (Fig. 3). Further, scattered non-predominant ground-glass opacity, especially dominant at the right upper lobe, was found on HRCT. Thus, we ruled out a diagnosis of BOOP or histiocytosis. She also underwent open lung biopsy, and the pathology showed focal accumulation of hemosiderinladen macrophages, suggestive of idiopathic hemosiderosis. She was therefore diagnosed with IPH, and treatment with prednisolone 2 mg/kg per day was initiated. Her condition aggravated several times during the course of steroid therapy when she developed recurrent upper respiratory infections. She was treated with prednisolone for 2 years and 5 months, but when she was 5 years 6 months old, she developed cushingoid symptom that worsened. Her BMI also increased from 22.6 to (Fig. 2). Therefore, her medication was switched from prednisolone to DFZ 2 mg/kg per day. After switching to DFZ, her BMI improved from to (Fig. 2). Thus, she continued DFZ treatment for 3 years, after which the treatment was discontinued. She visited the clinic for routine checkup, with no episode of IPH even without any treatment for 6 months. 3) Case 3 A 6-year 7-month-old girl presenting with fever for 4 days, vomiting, and bloody sputum for 2 months was admitted to the hospital. Her laboratory data showed severe anemia with an Hb level of 3.4 g/dl. Thus, she underwent chest radiography and HRCT, which showed symmetric opacity of geographic pattern on both lungs, without subpleural sparing evidence. Therefore, BOOP, desquamative interstitial pneumonia (DIP), or hypersensitivity pneumonia was suspected. We could not obtain pathology results since the patient refused lung biopsy, but typical HRCT finding and laboratory findings such as increased ferritin level, low Hb level, and microscopic hypochromic anemia indicated IPH. As she was diagnosed with IPH based on laboratory and radiology data, prednisolone (1 mg/kg per day) therapy was initiated. During the course of steroid therapy, she switched to methylprednisolone pulse therapy several times because of aggravation of respiratory symptoms. After 2 years and 6 months of therapy, prednisolone dose was tapered since her BMI had increased gradually from to (Fig. 2). As such, she started DFZ 0.8 mg/kg per day, but the dose was then tapered for 5 months. She was treated with DFZ for 7 months. Since no symptoms of IPH without any medication was reported when she came to the hospital for routine check-up, her DFZ treatment could be discontinued. Discussion Fig. 3. Chest radiograph of patient before treatment. Predominant ground-glass opacity (GGO) on right upper lobe, less GGO on left parahilar area. Idiopathic pulmonary hemosiderosis (IPH) is a rare disorder of unclear pathogenesis, with focal intraalveolar hemorrhage and thickening of alveolar basement membrane with interstitial fibrosis. It has an incidence of 0.24 to 1.23 cases per 100,000 children per year in selected populations [2,3]. The prognosis of pediatric IPH was measured by a 5-year survival rate, which was 86%, and the mortality rate was as high as 17% [4]. One retrospective study indicated long-term outcomes of IPH, which reports that prolonged survival is possible for children. 190 Vol. 23, No. 2, October 2016
4 Deflazacort Therapy for Idiopathic Pulmonary Hemosiderosis IPH evolved to an immune disorder, rheumatoid polyarthritis, or celiac disease [8]. IPH is diagnosed using laboratory, radiology, and pathology data from lung biopsy or bronchoalveolar lavage, with clinical symptoms such as hemoptysis, cough, and dyspnea. Further, long-term treatments had better outcomes than previous reports. Immunosuppressive treatment for acute episodes of IPH with steroids is effective, and following it with low doses may be beneficial [9]. However, dexamethasone administration may cause obesity and growth retardation, which are harmful for growing children [10]. In previous studies, the authors measured lower leg growth after administration of dexamethasone, which showed decreased velocity of leg length. DFZ, a methyl-oxazoline derivative of prednisolone, is an anti-inflammatory agent that is comparable to prednisolone. In other studies, DFZ appeared as effective as prednisolone in the long-term treatment of chronic sarcoidosis, and may have fewer side effects, especially on the bone [11]. Another study also revealed that patients with Duchenne muscular dystrophy treated with DFZ developed asymptomatic cataract, but other potential side effects such as hypertension, glucosuria, acne, infection, or bruising were not observed [12]. In the present study, the effectiveness of DFZ was similar with prednisolone without the effect of obesity (Table 1). Hemoglobin and ferritin levels improved in all three cases with DFZ, as compared to prednisolone. We compared the degree of obesity throughout the treatment of prednisolone and DFZ by using BMI values (Fig. 3). Normal range of BMI is kg/m 2 in children. During the treatment of prednisolone, BMI was greater than 23 kg/m 2 in Case 1 and 2. However, BMI dramatically decreased after DFZ administration (Fig. 2). This indicates that obesity reduced after switching treatment from prednisolone to DFZ. In case 3, after switching to DFZ, the treatment was maintained only for 7 months and discontinued thereafter. Although no further medication was administered, the patient s BMI increased. We assumed that her obesity had not improved dramatically, because she had taken prednisolone for long period of time and DFZ for only 7 months. In addition, she is now 12 years old, and has reached puberty. These two reasons may affect weight gain even after quitting all medications. All three patients improved after DFZ treatment. We also observed that two of three cases showed a trend of weight improvement after DFZ treatment. However, DFZ has several side effects. Previous studies reported that DFZ has common side effects of weight gain, height reduction, and cataract formation [13,14]. Other studies include vertebral fractures (except limb fractures), which can lead to stunted growth [15]. However, in this study, we followed them up for several years; all three patients had no side effects of cataract, or vertebral fractures. There were several limitations in this study. The total number of cases was too small to represent the general population. Second, the cases only included Korean children, which were not representative of other populations because of geographical and cultural differences. Lastly, it is necessary to follow them up in a long-term basis to investigate results after the puberty period. To conclude, the main treatment of IPH is systemic corticosteroid therapy to decrease the recurrence of pulmonary hemorrhage. However, the most common side effect of long-term prednisolone or dexamethasone treatment is obesity; therefore, we should consider the use of DFZ to reduce obesity side effect. Table 1. Laboratory data showing the effectiveness of deflazacort in idiopathic pulmonary hemosiderosis patients Hb g/dl/ Ferritin g/l Before diagnosis After steroid treatment Changed to deflazacort (after 1 month) After 2 months of deflazacort treatment Case 1 4.9/ / / /77.2 Case 2 6.7/ / / /31.2 Case 3 3.4/ / / /64.0 Hemoglobin and ferritin levels improved in all three cases treated with deflazacort, as compared to prednisolone. Hb, hemoglobin. Clin Pediatr Hematol Oncol 191
5 Kyujung Park, et al References 1. Chryssanthopoulos C, Cassimos C, Panagiotidou C. Prognostic criteria in idiopathic pulmonary hemosiderosis in children. Eur J Pediatr 1983;140: Kjellman B, Elinder G, Garwicz S, Svan H. Idiopathic pulmonary haemosiderosis in Swedish children. Acta Paediatr Scand 1984;73: Ohga S, Takahashi K, Miyazaki S, Kato H, Ueda K. Idiopathic pulmonary haemosiderosis in Japan: 39 possible cases from a survey questionnaire. Eur J Pediatr 1995;154: Saeed MM, Woo MS, MacLaughlin EF, Margetis MF, Keens TG. Prognosis in pediatric idiopathic pulmonary hemosiderosis. Chest 1999;116: Matsaniotis N, Karpouzas J, Apostolopoulou E, Messaritakis J. Idiopathic pulmonary haemosiderosis in children. Arch Dis Child 1968;43: Cacoub P, Chemlal K, Khalifa P, et al. Deflazacort versus prednisone in patients with giant cell arteritis: effects on bone mass loss. J Rheumatol 2001;28: Saviola G, Abdi Ali L, Shams Eddin S, et al. Compared clinical efficacy and bone metabolic effects of low-dose deflazacort and methyl prednisolone in male inflammatory arthropathies: a 12-month open randomized pilot study. Rheumatology (Oxford) 2007;46: Le Clainche L, Le Bourgeois M, Fauroux B, et al. Long-term outcome of idiopathic pulmonary hemosiderosis in children. Medicine (Baltimore) 2000;79: Kiper N, Göçmen A, Ozçelik U, Dilber E, Anadol D. Longterm clinical course of patients with idiopathic pulmonary hemosiderosis ( ): prolonged survival with low-dose corticosteroid therapy. Pediatr Pulmonol 1999;27: Gibson AT, Pearse RG, Wales JK. Growth retardation after dexamethasone administration: assessment by knemometry. Arch Dis Child 1993;69: Rizzato G, Riboldi A, Imbimbo B, Torresin A, Milani S. The long-term efficacy and safety of two different corticosteroids in chronic sarcoidosis. Respir Med 1997;91: Biggar WD, Gingras M, Fehlings DL, Harris VA, Steele CA. Deflazacort treatment of Duchenne muscular dystrophy. J Pediatr 2001;138: McAdam LC, Mayo AL, Alman BA, Biggar WD. The Canadian experience with long-term deflazacort treatment in Duchenne muscular dystrophy. Acta Myol 2012;31: Biggar WD, Harris VA, Eliasoph L, Alman B. Long-term benefits of deflazacort treatment for boys with Duchenne muscular dystrophy in their second decade. Neuromuscul Disord 2006;16: Houde S, Filiatrault M, Fournier A, et al. Deflazacort use in Duchenne muscular dystrophy: an 8-year follow-up. Pediatr Neurol 2008;38: Vol. 23, No. 2, October 2016
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