Fatal interstitial pneumonia in juvenile dermatomyositis

Nagai, Yayoi; Mizuno, Takahisa; Yoshizawa, Chikage; Ishikawa, Osamu

doi:10.1684/ejd.2010.0846

Cited by 9 publications

(4 citation statements)

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“…One study of diffuse ILD in 7 of 15 patients with either PM or DM found that affected patients had higher CPK and aldolase (), which is similar to our findings. A case report of a child with fatal ILD also observed elevated aldolase levels (). Elevation in aldolase is an indicator of increased risk of pulmonary disease in patients with IIMs, as evidenced by our evaluation showing that patients with both abnormal DL CO and abnormal TLC had significantly higher aldolase levels.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Function Tests in Idiopathic Inflammatory Myopathy: Association With Clinical Parameters in Children

Prestridge

Morgan

Ferguson

et al. 2013

Arthritis Care & Research

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Objective. To determine the association of decreased lung function in children with idiopathic inflammatory myopathies (IIMs) with specific clinical parameters. Methods. This study of 38 children ages 6 -23 years diagnosed with definite/probable IIM evaluated the association of myositis-specific/-associated antibodies (MSAs/MAAs), duration of untreated disease at diagnosis, Disease Activity Score for muscle (DAS-M), muscle-derived enzymes (aldolase, lactate dehydrogenase [LDH], aspartate transaminase, and creatine phosphokinase [CPK]), neopterin and von Willebrand factor antigen, and the Childhood Myositis Assessment Scale (CMAS) scores with data from pulmonary function testing (PFT). Results. Impaired PFTs were defined as total lung capacity (TLC) or diffusing capacity for carbon monoxide (DLCO) of <80% predicted. The PFTs documented that 37% of the children (14 of 38) had either decreased TLC or decreased DLCO; 5% (2 of 38) had both. Children with decreased TLC alone (7 [18%] of 38) were older both at the time of PFT and diagnosis, had anti-Jo-1 and anti-Scl-70 antibody, and had elevated levels of CPK and neopterin. Children with decreased DLCO alone (5 [13%] of 38) had a shorter duration of untreated disease at diagnosis, had higher DAS-M and total DAS, were positive for anti-Ro and anti-PL-12, had increased LDH, and had elevated levels of neopterin and aldolase, with low CMAS scores for items 1, 3, 10, 11, and 14. Conclusion. Assessment of PFTs in children with IIMs should be considered, since more than one-third of patients were found to be impaired. The presence of MSAs/MAAs, an elevated serum neopterin level (mean ؎ SD 12.4 ؎ 9.6 nmoles/liter, normal value <10.5), older age at diagnosis, and shorter duration of untreated disease at diagnosis suggest the presence of potential lung pathology.

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Section: Discussionmentioning

confidence: 99%

Pulmonary Function Tests in Idiopathic Inflammatory Myopathy: Association With Clinical Parameters in Children

Prestridge

Morgan

Ferguson

et al. 2013

Arthritis Care & Research

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“…There have been a few reports on patients with dermatomyositis/JDM-associated RP-ILD who were treated with PE but had poor outcomes [ 4 , 12 , 24 ]. Some experts suggest that PE can be considered in patients who are unresponsive to combination therapy with corticosteroids and immunosuppressive agents [ 4 ].…”

Section: Discussionmentioning

confidence: 99%

“…Furthermore, patients with anti-MDA-5 antibody-positive JDM and adult dermatomyositis are prone to developing RP-ILD [3,7]; in JDM specifically, RP-ILD has a poor prognosis with no established treatment. To study this further, we conducted a literature review of JDM with RP-ILD and found 18 cases (Table 1) [3,6,[8][9][10][11][12][13][14][15][16][17][18][19]. The search was performed in July 2021 using the PubMed/Medline database.…”

Section: Discussionmentioning

confidence: 99%

Successful rituximab treatment for severe rapidly progressive interstitial lung disease with anti-MDA5 antibody-positive juvenile dermatomyositis: a case report and literature review

et al. 2022

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Background Rapidly progressive (RP) interstitial lung disease (ILD) is a life-threatening complication of juvenile dermatomyositis (JDM); however, it is generally refractory to treatment; to the best of our knowledge, no evidence-based treatment has been established for RP-ILD yet. We present the case of a 2-year-old girl with RP-ILD who showed resistance to treatment with methylprednisolone, cyclosporine A, cyclophosphamide, immunoglobulin, and plasma exchange (PE) and was finally treated with extracorporeal membrane oxygenation. We further present a literature review of 18 cases of JDM with RP-ILD. Case presentation A 2-year-old girl presented with malar rash, mild muscle weakness, and weight loss for a few months before admission. She had a history of dry cough and dyspnea for a few days, followed by rapid respiratory failure. The patient was diagnosed with JDM with RP-ILD through physical examination (malar rashes and Gottron’s sign) and based on the finding of myositis on femoral magnetic resonance imaging, elevated levels of serum muscle enzymes, positive anti-melanoma differentiation-association gene 5 (MDA5) antibody (> 7,500 index), elevated level of Krebs von den Lungen-6 glycoprotein (KL-6; 3,420 U/mL), and extensive ground-glass opacities with consolidation in the bilateral lungs on chest high-resolution computed tomography. She received combination therapy, including methylprednisolone pulse therapy, followed by oral prednisolone and intravenous cyclosporine A, cyclophosphamide, and immunoglobulin. On day 11 of hospitalization, she was placed on ventilation support and PE was initiated. However, her respiratory condition continued to deteriorate and veno-venous extracorporeal membrane oxygenation was started on day 24 of hospitalization. Rituximab was administered on day 28. After 2 weeks of rituximab therapy initiation, her respiratory condition showed gradual improvements. Eventually, on day 52 of hospitalization, the patient could be weaned off extracorporeal membrane oxygenation. Finally, she was discharged with minimal ventilation support and no neurological complications 11 months after admission. Conclusions Our literature review suggest that JDM with RP-ILD has a high mortality rate. In JDM, rituximab may be a promising treatment option for RP-ILD. In the future, the efficacy of rituximab in the early phases of ILD should be investigated.

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“…There have been a few reports of DM/JDM-associated RP-ILD who were treated with PE, but resulted in poor outcomes 4,12,22 . Some experts suggest that PE might be considered in patients who are unresponsive to combination therapy with corticosteroids and immunosuppressive agents 4 .…”

Section: Discussionmentioning

confidence: 99%

Successful rituximab treatment for severe rapidly progressive interstitial lung disease with anti-MDA5 antibody-positive juvenile dermatomyositis: A case report and literature review

Nishi

Ogura

Tamai

et al. 2022

Preprint

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Background: Rapidly progressive interstitial lung disease (RP-ILD) is a life-threatening complication of juvenile dermatomyositis (JDM); however, it is generally refractory to treatment and no evidence-based treatment has yet been established for RP-ILD. We present the case of a 2-year-old girl with RP-ILD who was resistant to methylprednisolone, cyclosporine A, cyclophosphamide, immunoglobulin, and plasma exchange. Finally, she required extracorporeal membrane oxygenation, but rituximab contributed to the patient’s disease remission and survival. In addition, we conducted a literature review of 18 cases of JDM with RP-ILD.Case presentation: A 2-year-old girl developed malar rash, mild muscle weakness, and weight loss for a few months before admission. She presented with a history of dry cough and dyspnea for few days, followed by rapid respiratory failure. The diagnosis of JDM with RP-ILD was made by physical examination (malar rashes and Gottron’s sign) as well as by the finding of myositis on femoral magnetic resonance imaging, the elevation of serum muscle enzymes, positive anti-melanoma differentiation-association gene 5 antibody (>7,500 index), the elevation of Krebs von den Lungen-6 (3,420 U/mL), and the finding of extensive ground-glass opacities with consolidation in the bilateral lungs on chest high-resolution computed tomography. She received combination therapy, including methylprednisolone pulse therapy, followed by oral prednisolone, intravenous cyclosporine A, intravenous cyclophosphamide, and intravenous immunoglobulin. On the 11th day of her hospital stay, she was put on ventilation, and plasma exchange was started. However, her respiratory condition continued to deteriorate and veno-venous extracorporeal membrane oxygenation was started on day 24 of hospitalization. Rituximab was administered on day 28 of hospitalization. Two weeks after starting rituximab therapy, her respiratory condition gradually started to improve. Eventually, on day 52 of her hospital stay, she was able to wean off extracorporeal membrane oxygenation. Finally, 11 months after admission, she was discharged with minimal ventilatory support and no neurological complications.Conclusions: According to this literature review, JDM with RP-ILD has a high mortality rate. In JDM, rituximab could be a promising treatment option for RP-ILD. In the future, the effectiveness of rituximab in the early phases of ILD should be investigated.

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Fatal interstitial pneumonia in juvenile dermatomyositis

Cited by 9 publications

References 0 publications

Pulmonary Function Tests in Idiopathic Inflammatory Myopathy: Association With Clinical Parameters in Children

Pulmonary Function Tests in Idiopathic Inflammatory Myopathy: Association With Clinical Parameters in Children

Successful rituximab treatment for severe rapidly progressive interstitial lung disease with anti-MDA5 antibody-positive juvenile dermatomyositis: a case report and literature review

Successful rituximab treatment for severe rapidly progressive interstitial lung disease with anti-MDA5 antibody-positive juvenile dermatomyositis: A case report and literature review

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