http://informahealthcare.com/mor ISSN 1439-7595 (print), 1439-7609 (online) Mod Rheumatol, 2013; Early Online: 1–3 © 2013 Japan College of Rheumatology DOI: 10.3109/14397595.2013.843753
CASE REPORT
A case of dermatomyositis with rhabdomyolysis, rescued by intravenous immunoglobulin
Mod Rheumatol Downloaded from informahealthcare.com by University of Connecticut on 01/06/14 For personal use only.
Fumitaka Mizoguchi1, Kazuki Takada2, Kinya Ishikawa3, Hidehiro Mizusawa3, Hitoshi Kohsaka1, and Nobuyuki Miyasaka1 1Department of Medicine and Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan, 2Office for Global Education and Career Development, International Exchange Center, Tokyo Medical and Dental University, Tokyo, Japan, and 3Department of Neurology and Neurological Sciences, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan
Abstract
Keywords
We describe a case of severe dermatomyositis (DM) complicated by rhabdomyolysis, acute tubular necrosis, and hemophagocytosis. The case failed to respond to corticosteroids, but showed rapid and significant improvement after the addition of intravenous immunoglobulin (IVIG).While the prognosis of DM is poor when it is complicated by rhabdomyolysis, the early administration of IVIG has the potential to be the cornerstone of its management.
Dermatomyositis, Hemophagocytosis, Rhabdomyolysis, IVIG
Introduction Rhabdomyolysis is caused by many conditions, such as trauma, metabolic myopathies, drugs, infections, endocrine disorders and autoimmune myopathies. It requires immediate intervention or treatment for underlying causes as well as aggressive fluid restoration to avoid acute tubular necrosis. Autoimmune myopathies are heterogeneous group of diseases including polymyositis (PM), dermatomyositis (DM), and inclusion body myositis [1]. Among them, DM and PM usually respond to glucocorticoids alone or a combination of glucocorticoids and immunosuppressive drugs. However, when they are complicated by rhabdomyolysis, the prognosis has been reported to be poor despite such treatments [2]. We herein describe a case of glucocorticoid-resistant severe DM complicated by rhabdomyolysis, acute tubular necrosis and hemophagocytosis, rescued by intravenous immunoglobulin (IVIG) therapy.
Case report A 24-year-old man with no significant medical history developed edematous purplish rash in his periorbital and malar areas as well as pain and weakness of the thigh muscles in the both legs. Thigh muscle weakness progressed over the following 2 weeks, and he was admitted to a hospital where his presenting serum creatine kinase (CK) level was noted to be 12,230 U/l. The diagnosis of DM was strongly suspected based on his facial rash, progressive muscle weakness and elevated serum CK, and he was initially
Correspondence to: Nobuyuki Miyasaka, MD, PhD, Department of Medicine and Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, 1-5-45, Yushima, Bunkyo-ku, Tokyo, 113-8510, Japan. Tel: 81-3-5803-5201, Fax: 813-5684-0057. E-mail: [email protected]
History Received 12 February 2013 Accepted 5 June 2013 Published online 31 October 2013
treated with methylprednisolone pulse therapy (500 mg/day for 3 days) followed by prednisolone 60 mg/day, together with aggressive hydration to avoid myoglobin-induced nephropathy. However, he showed minimal response to the initial treatment and continued to progress further, requiring a transfer to our hospital. Upon transfer, physical examination revealed periorbital edematous purplish rash, erythematous scaly rash on the extensor surface of the bilateral elbows and knees, periungual erythema, and pitting edema in his both legs. Proximal muscles of all four extremities and the neck flexor muscles were noted severely weak, and he had severe dysphagia. Laboratory examination revealed marked elevation of CK (40,700 U/l) and myoglobin ( 3,000 ng/ml) (Table 1). Anti-Jo-1 antibody was negative. His urine was dark brown colored, and was with proteinuria (4.8 g/24 h) and microscopic hematuria (RBC 30–40/hpf). Creatinine clearance was normal, but urine N-acetyl-β-D-glucosaminidase (NAG) level was increased (73.7 U/l). Electromyogram revealed the myogenic pattern. Magnetic resonance imaging of his thigh demonstrated high-intensity areas in T2-weighted and STIR images (Figure 1a, b). Muscle biopsy showed loss of cross striations in most fibers as well as fragmentation of the sarcoplasm in some fibers (Figure 1c). Inflammatory infiltrates, myofiber necrosis or regeneration were not observed. It was concluded that he had DM complicated by rhabdomyolysis, and methylprednisolone pulse therapy (500 mg/day for 3 days) was repeated, followed by oral prednisolone 80 mg/day. Muscle weakness, however, continued to worsen and he eventually became bed-bound and was unable even to turn over in bed. On the 5th day after transfer, serum urea nitrogen and creatinine increased (BUN 55 mg/dl, Cre 1.11 mg/dl). Since his urine NAG level was noted to be significantly increased on transfer, and no nephrotoxic drug was used, we concluded that he developed myoglobin-induced acute tubular necrosis. Therefore, prednisolone was increased to 100 mg/ day and IVIG therapy (32.5 g/day 5 days) was started. On the
2
F. Mizoguchi et al.
Mod Rheumatol, 2013; Early Online: 1–3
Table 1. Laboratory data on admission.
Mod Rheumatol Downloaded from informahealthcare.com by University of Connecticut on 01/06/14 For personal use only.
Urinalysis pH Specific gravity Protein Glucose Occult blood Sediment RBC WBC Cast Ccr U-pro NAG
8.5 1.044 4 – 3 30–49 50–99 – 113 4.8 73.7
/hpf /hpf ml/min g/day U/l
Blood cell count WBC Neu Ly RBC Hemoglobin Hematocrit Platelet Serology CRP ANA Anti-Jo-1 antibody
18,000 94 3 508 104 15.9 46.4 14.3 104
/μl % % /μl g/dl % /μl
2 mg/l – –
Chemistry Toral protein Albumin BUN Cre UA Na K Cl LDH AST ALT CK CK-MB CK-MM Myoglobin
6.1 2.8 16.9 0.3 3.1 132 4.1 94 1,977 1,183 384 40,700 3 95 3,000
g/dl g/dl mg/dl mg/dl mg/dl mEq/l mEq/l mEq/l IU/l IU/l IU/l IU/l % % ng/ml
hpf, high-power field; Ccr, creatinine clearance; NAG, N-acetyl-β-D-glucosaminidase; WBC, leukocytes; Neu, neutrophil; Ly, lymphocyte; RBC, erythrocytes; BUN, blood urea nitrogen; Cre, creatinine; UA, uric acid; LDH, lactate dehydrogenase; AST, aspartate aminotransferase; ALT, alanine aminotransferase; CK, creatine kinase; CRP, C-reactive protein; ANA, anti-nuclear antibody.
6th day, anemia (Hb 7.1 g/dl) and thrombocytopenia (Plt 7.7 104/μl) developed accompanied by significant elevation of serum ferritin level (1441 ng/ml) which strongly suggested hemophagocytic syndrome though bone marrow was not examined. On the 7th day, CK reached 202,410 U/ml, and he developed aspiration pneumonia. On the 8th day, however, CK and serum creatinine levels reached their peaks and started to decrease on the following day (Figure 2). His muscle strength gradually improved and, within a few weeks of physical therapy, he became ambulatory. Doses of prednisolone were gradually and uneventfully tapered, and prednisolone was eventually discontinued after 1 and a 1/2 years. He has since remained in remission.
Discussion Our DM patient, who was complicated by rhabdomyolysis, acute tubular necrosis and hemophagocytosis, was unresponsive to corticosteroids alone but responded favorably and promptly to the addition of IVIG therapy. It is reported that the prognosis of DM complicated by rhabdomyolysis is poor [2]. Retrospective
Figure 1. MRI and histological findings. MRI demonstrated high-intensity areas in T2-weighted imaging (A) and short tau inversion recovery imaging (B). Loss of cross striations in most fibers and fragmentation of the sarcoplasm were found in some fibers. No inflammatory infiltrates were observed. (hematoxylin and eosin staining) (C).
analysis of PM/DM patients revealed that four out of five patients of DM complicated by rhabdomyolysis and acute tubular necrosis required emergent hemodialysis and that all five died [2]. Although our patient did not reach the point where he required hemodialysis, he had the severity that was at least comparable to other DMassociated rhabdomyolysis cases reported in the literature. The level of peak CK in our patient was higher than any of the reported cases, and he developed hemophagocytosis. Efficacy of IVIG against DM has been well documented by randomized controlled trials [3]. However, experiences of IVIG in DM patients complicated by rhabdomyolysis have been scarce and the literature is limited to a single report of a case successfully treated with plasmapheresis and IVIG [4]. We reported our experience that suggests further the potential efficacy of IVIG against DM-associated rhabdomyolysis, especially given early in the course before patients require hemodialysis. Although it has not yet fully understood how IVIG ameliorates the inflammatory response in humans, the increased activation threshold of macrophages was proposed as a mechanism of anti-arthritic activity of IVIG [5]. It was reported that the shift of the threshold is due to upregulation of inhibitory FcγRIIB on macrophages which is induced by production of IL-33 and following IL-4 [6]. However, the rapid response to IVIG in our case suggests the direct effect of IVIG to suppress the effector function of macrophages. As rhabdomyolysis rarely complicates DM, we hypothesized that he had an underlying condition that predisposed him to rhabdomyolysis at the onset of DM. Specifically, we suspected carnitine palmitoyltransferase II (CPT-II) deficiency which could develop rhabdomyolysis as an initial clinical manifestation in adults [7]. We first examined his muscle CPT-II activity during rhabdomyolysis, which was decreased to 0.34 nmol/ mg/min (17% of the control samples). However, tandem mass spectrometry of his serum acylcarnitines performed at the time of remission showed a normal profile. In addition, we could not identify any mutation in his CPT2 gene. These results suggest that the decreased CPT-II activity noted during rhabdomyolysis was not due to CPT-II deficiency but was associated secondary with muscle damage. In conclusion, we report here a glucocorticoid-resistant severe DM complicated by rhabdomyolysis successfully treated with the addition of IVIG therapy. Our finding indicates that IVIG, especially given early, has the potential to be
A case of dermatomyositis with rhabdomyolysis 3
DOI 10.3109/14397595.2013.843753
Mod Rheumatol Downloaded from informahealthcare.com by University of Connecticut on 01/06/14 For personal use only.
Figure 2. Clinical course. CK: creatine kinase, Cre: creatinine IVIG: intravenous immunoglobulin, MPS: methylprednisolone, PSL: prednisolone.
a cornerstone in the treatment of this rare but potentially fatal complication of DM.
Acknowledgements We thank Masaki Takayanagi at Ciba Children’s Hospital for analyzing CPTII activity, Yosuke Shigematsu at Fukui University for serum acylcarnitine analysis, and Tetsuhiko Yasuno at Fukuoka University for gene analysis.
Conflict of interest H.K. has served as a consultant to Chugai Pharmaceutical and has received research grants from Eisai Pharmaceutical and Takeda Pharmaceutical. N.M. has received research grants from Abbott, Astellas Pharmaceutical, Banyu Pharmaceutical, Chugai Pharmaceutical, Daiichi Sankyo Pharmaceutical, Eisai Pharmaceutical, Janssen Pharmaceutical, Mitsubishi Tanabe Pharma Corporation, Takeda Pharmaceutical and Teijin Pharmaceutical. All other authors have declared no conflicts of interest.
References 1. Mammen AL. Autoimmune myopathies: autoantibodies, phenotypes and pathogenesis. Nat Rev Neurol. 2011;7(6):343–54. 2. Yen TH, Lai PC, Chen CC, Hsueh S, Huang JY. Renal involvement in patients with polymyositis and dermatomyositis. Int J Clin Pract. 2005;59(2):188–93. 3. Dalakas MC, Illa I, Dambrosia JM, Soueidan SA, Stein DP, Otero C, et al. A controlled trial of high-dose intravenous immuneglobulin infusions as treatment for dermatomyositis. N Engl J Med. 1993;329(27):1993–2000. 4. Fukunaga E, Kunishige M, Mitsui T, Endo I, Oshima Y, Ohnishi Y, et al. Severe dermatomyositis with rhabdomyolysis and paralytic ileus: a case successfully treated with plasmapheresis and intravenous immunoglobulin. Eur J Neurol. 2002;9(6):697–8. 5. Anthony RM, Wermeling F, Ravetch JV. Novel roles for the IgG Fc glycan. Ann N Y Acad Sci. 2012;1253:170–80. 6. Deschauer M, Wieser T, Zierz S. Muscle carnitine palmitoyltransferase II deficiency: clinical and molecular genetic features and diagnostic aspects. Arch Neurol. 2005;62(1):37–41.
CASE REPORT
A case of dermatomyositis with rhabdomyolysis, rescued by intravenous immunoglobulin
Mod Rheumatol Downloaded from informahealthcare.com by University of Connecticut on 01/06/14 For personal use only.
Fumitaka Mizoguchi1, Kazuki Takada2, Kinya Ishikawa3, Hidehiro Mizusawa3, Hitoshi Kohsaka1, and Nobuyuki Miyasaka1 1Department of Medicine and Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan, 2Office for Global Education and Career Development, International Exchange Center, Tokyo Medical and Dental University, Tokyo, Japan, and 3Department of Neurology and Neurological Sciences, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan
Abstract
Keywords
We describe a case of severe dermatomyositis (DM) complicated by rhabdomyolysis, acute tubular necrosis, and hemophagocytosis. The case failed to respond to corticosteroids, but showed rapid and significant improvement after the addition of intravenous immunoglobulin (IVIG).While the prognosis of DM is poor when it is complicated by rhabdomyolysis, the early administration of IVIG has the potential to be the cornerstone of its management.
Dermatomyositis, Hemophagocytosis, Rhabdomyolysis, IVIG
Introduction Rhabdomyolysis is caused by many conditions, such as trauma, metabolic myopathies, drugs, infections, endocrine disorders and autoimmune myopathies. It requires immediate intervention or treatment for underlying causes as well as aggressive fluid restoration to avoid acute tubular necrosis. Autoimmune myopathies are heterogeneous group of diseases including polymyositis (PM), dermatomyositis (DM), and inclusion body myositis [1]. Among them, DM and PM usually respond to glucocorticoids alone or a combination of glucocorticoids and immunosuppressive drugs. However, when they are complicated by rhabdomyolysis, the prognosis has been reported to be poor despite such treatments [2]. We herein describe a case of glucocorticoid-resistant severe DM complicated by rhabdomyolysis, acute tubular necrosis and hemophagocytosis, rescued by intravenous immunoglobulin (IVIG) therapy.
Case report A 24-year-old man with no significant medical history developed edematous purplish rash in his periorbital and malar areas as well as pain and weakness of the thigh muscles in the both legs. Thigh muscle weakness progressed over the following 2 weeks, and he was admitted to a hospital where his presenting serum creatine kinase (CK) level was noted to be 12,230 U/l. The diagnosis of DM was strongly suspected based on his facial rash, progressive muscle weakness and elevated serum CK, and he was initially
Correspondence to: Nobuyuki Miyasaka, MD, PhD, Department of Medicine and Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, 1-5-45, Yushima, Bunkyo-ku, Tokyo, 113-8510, Japan. Tel: 81-3-5803-5201, Fax: 813-5684-0057. E-mail: [email protected]
History Received 12 February 2013 Accepted 5 June 2013 Published online 31 October 2013
treated with methylprednisolone pulse therapy (500 mg/day for 3 days) followed by prednisolone 60 mg/day, together with aggressive hydration to avoid myoglobin-induced nephropathy. However, he showed minimal response to the initial treatment and continued to progress further, requiring a transfer to our hospital. Upon transfer, physical examination revealed periorbital edematous purplish rash, erythematous scaly rash on the extensor surface of the bilateral elbows and knees, periungual erythema, and pitting edema in his both legs. Proximal muscles of all four extremities and the neck flexor muscles were noted severely weak, and he had severe dysphagia. Laboratory examination revealed marked elevation of CK (40,700 U/l) and myoglobin ( 3,000 ng/ml) (Table 1). Anti-Jo-1 antibody was negative. His urine was dark brown colored, and was with proteinuria (4.8 g/24 h) and microscopic hematuria (RBC 30–40/hpf). Creatinine clearance was normal, but urine N-acetyl-β-D-glucosaminidase (NAG) level was increased (73.7 U/l). Electromyogram revealed the myogenic pattern. Magnetic resonance imaging of his thigh demonstrated high-intensity areas in T2-weighted and STIR images (Figure 1a, b). Muscle biopsy showed loss of cross striations in most fibers as well as fragmentation of the sarcoplasm in some fibers (Figure 1c). Inflammatory infiltrates, myofiber necrosis or regeneration were not observed. It was concluded that he had DM complicated by rhabdomyolysis, and methylprednisolone pulse therapy (500 mg/day for 3 days) was repeated, followed by oral prednisolone 80 mg/day. Muscle weakness, however, continued to worsen and he eventually became bed-bound and was unable even to turn over in bed. On the 5th day after transfer, serum urea nitrogen and creatinine increased (BUN 55 mg/dl, Cre 1.11 mg/dl). Since his urine NAG level was noted to be significantly increased on transfer, and no nephrotoxic drug was used, we concluded that he developed myoglobin-induced acute tubular necrosis. Therefore, prednisolone was increased to 100 mg/ day and IVIG therapy (32.5 g/day 5 days) was started. On the
2
F. Mizoguchi et al.
Mod Rheumatol, 2013; Early Online: 1–3
Table 1. Laboratory data on admission.
Mod Rheumatol Downloaded from informahealthcare.com by University of Connecticut on 01/06/14 For personal use only.
Urinalysis pH Specific gravity Protein Glucose Occult blood Sediment RBC WBC Cast Ccr U-pro NAG
8.5 1.044 4 – 3 30–49 50–99 – 113 4.8 73.7
/hpf /hpf ml/min g/day U/l
Blood cell count WBC Neu Ly RBC Hemoglobin Hematocrit Platelet Serology CRP ANA Anti-Jo-1 antibody
18,000 94 3 508 104 15.9 46.4 14.3 104
/μl % % /μl g/dl % /μl
2 mg/l – –
Chemistry Toral protein Albumin BUN Cre UA Na K Cl LDH AST ALT CK CK-MB CK-MM Myoglobin
6.1 2.8 16.9 0.3 3.1 132 4.1 94 1,977 1,183 384 40,700 3 95 3,000
g/dl g/dl mg/dl mg/dl mg/dl mEq/l mEq/l mEq/l IU/l IU/l IU/l IU/l % % ng/ml
hpf, high-power field; Ccr, creatinine clearance; NAG, N-acetyl-β-D-glucosaminidase; WBC, leukocytes; Neu, neutrophil; Ly, lymphocyte; RBC, erythrocytes; BUN, blood urea nitrogen; Cre, creatinine; UA, uric acid; LDH, lactate dehydrogenase; AST, aspartate aminotransferase; ALT, alanine aminotransferase; CK, creatine kinase; CRP, C-reactive protein; ANA, anti-nuclear antibody.
6th day, anemia (Hb 7.1 g/dl) and thrombocytopenia (Plt 7.7 104/μl) developed accompanied by significant elevation of serum ferritin level (1441 ng/ml) which strongly suggested hemophagocytic syndrome though bone marrow was not examined. On the 7th day, CK reached 202,410 U/ml, and he developed aspiration pneumonia. On the 8th day, however, CK and serum creatinine levels reached their peaks and started to decrease on the following day (Figure 2). His muscle strength gradually improved and, within a few weeks of physical therapy, he became ambulatory. Doses of prednisolone were gradually and uneventfully tapered, and prednisolone was eventually discontinued after 1 and a 1/2 years. He has since remained in remission.
Discussion Our DM patient, who was complicated by rhabdomyolysis, acute tubular necrosis and hemophagocytosis, was unresponsive to corticosteroids alone but responded favorably and promptly to the addition of IVIG therapy. It is reported that the prognosis of DM complicated by rhabdomyolysis is poor [2]. Retrospective
Figure 1. MRI and histological findings. MRI demonstrated high-intensity areas in T2-weighted imaging (A) and short tau inversion recovery imaging (B). Loss of cross striations in most fibers and fragmentation of the sarcoplasm were found in some fibers. No inflammatory infiltrates were observed. (hematoxylin and eosin staining) (C).
analysis of PM/DM patients revealed that four out of five patients of DM complicated by rhabdomyolysis and acute tubular necrosis required emergent hemodialysis and that all five died [2]. Although our patient did not reach the point where he required hemodialysis, he had the severity that was at least comparable to other DMassociated rhabdomyolysis cases reported in the literature. The level of peak CK in our patient was higher than any of the reported cases, and he developed hemophagocytosis. Efficacy of IVIG against DM has been well documented by randomized controlled trials [3]. However, experiences of IVIG in DM patients complicated by rhabdomyolysis have been scarce and the literature is limited to a single report of a case successfully treated with plasmapheresis and IVIG [4]. We reported our experience that suggests further the potential efficacy of IVIG against DM-associated rhabdomyolysis, especially given early in the course before patients require hemodialysis. Although it has not yet fully understood how IVIG ameliorates the inflammatory response in humans, the increased activation threshold of macrophages was proposed as a mechanism of anti-arthritic activity of IVIG [5]. It was reported that the shift of the threshold is due to upregulation of inhibitory FcγRIIB on macrophages which is induced by production of IL-33 and following IL-4 [6]. However, the rapid response to IVIG in our case suggests the direct effect of IVIG to suppress the effector function of macrophages. As rhabdomyolysis rarely complicates DM, we hypothesized that he had an underlying condition that predisposed him to rhabdomyolysis at the onset of DM. Specifically, we suspected carnitine palmitoyltransferase II (CPT-II) deficiency which could develop rhabdomyolysis as an initial clinical manifestation in adults [7]. We first examined his muscle CPT-II activity during rhabdomyolysis, which was decreased to 0.34 nmol/ mg/min (17% of the control samples). However, tandem mass spectrometry of his serum acylcarnitines performed at the time of remission showed a normal profile. In addition, we could not identify any mutation in his CPT2 gene. These results suggest that the decreased CPT-II activity noted during rhabdomyolysis was not due to CPT-II deficiency but was associated secondary with muscle damage. In conclusion, we report here a glucocorticoid-resistant severe DM complicated by rhabdomyolysis successfully treated with the addition of IVIG therapy. Our finding indicates that IVIG, especially given early, has the potential to be
A case of dermatomyositis with rhabdomyolysis 3
DOI 10.3109/14397595.2013.843753
Mod Rheumatol Downloaded from informahealthcare.com by University of Connecticut on 01/06/14 For personal use only.
Figure 2. Clinical course. CK: creatine kinase, Cre: creatinine IVIG: intravenous immunoglobulin, MPS: methylprednisolone, PSL: prednisolone.
a cornerstone in the treatment of this rare but potentially fatal complication of DM.
Acknowledgements We thank Masaki Takayanagi at Ciba Children’s Hospital for analyzing CPTII activity, Yosuke Shigematsu at Fukui University for serum acylcarnitine analysis, and Tetsuhiko Yasuno at Fukuoka University for gene analysis.
Conflict of interest H.K. has served as a consultant to Chugai Pharmaceutical and has received research grants from Eisai Pharmaceutical and Takeda Pharmaceutical. N.M. has received research grants from Abbott, Astellas Pharmaceutical, Banyu Pharmaceutical, Chugai Pharmaceutical, Daiichi Sankyo Pharmaceutical, Eisai Pharmaceutical, Janssen Pharmaceutical, Mitsubishi Tanabe Pharma Corporation, Takeda Pharmaceutical and Teijin Pharmaceutical. All other authors have declared no conflicts of interest.
References 1. Mammen AL. Autoimmune myopathies: autoantibodies, phenotypes and pathogenesis. Nat Rev Neurol. 2011;7(6):343–54. 2. Yen TH, Lai PC, Chen CC, Hsueh S, Huang JY. Renal involvement in patients with polymyositis and dermatomyositis. Int J Clin Pract. 2005;59(2):188–93. 3. Dalakas MC, Illa I, Dambrosia JM, Soueidan SA, Stein DP, Otero C, et al. A controlled trial of high-dose intravenous immuneglobulin infusions as treatment for dermatomyositis. N Engl J Med. 1993;329(27):1993–2000. 4. Fukunaga E, Kunishige M, Mitsui T, Endo I, Oshima Y, Ohnishi Y, et al. Severe dermatomyositis with rhabdomyolysis and paralytic ileus: a case successfully treated with plasmapheresis and intravenous immunoglobulin. Eur J Neurol. 2002;9(6):697–8. 5. Anthony RM, Wermeling F, Ravetch JV. Novel roles for the IgG Fc glycan. Ann N Y Acad Sci. 2012;1253:170–80. 6. Deschauer M, Wieser T, Zierz S. Muscle carnitine palmitoyltransferase II deficiency: clinical and molecular genetic features and diagnostic aspects. Arch Neurol. 2005;62(1):37–41.
