scholarly journals Spontaneous Pneumomediastinum due to Anti-Melanoma Differentiation-Associated Protein 5 Requiring a Bilateral Lung Transplant

2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Amrit Singh Jhajj ◽  
James Hok Shun Yeung ◽  
Fergus To
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Immunosuppressive Therapy ◽  
Lung Transplant ◽  
Medical Literature ◽  
Rare Complication ◽  
Immunosuppressive Agents ◽  
Spontaneous Pneumomediastinum ◽  
Autoimmune Myositis ◽  
Bilateral Lung

Anti-melanoma differentiation-associated protein 5 (anti-MDA5) is a subset of dermatomyositis associated with respiratory complications, in which rapidly progressive interstitial lung disease (RPILD) is commonly cited, and spontaneous pneumomediastinum (SPM) is a rare complication. In medical literature, aggressive immunosuppressive therapy has been the mainstay of anti-MDA5-associated SPM management. Here, we report the first MDA5 case with SPM which was successfully treated with a double-lung transplant. We present a 48-year-old male who presented with multiple constitutional symptoms such as fevers, weight loss, malaise, and arthralgias, in association with erythroderma over the ears and fingers. Imaging of the chest demonstrated peripheral airspace disease, and myositis-specific serology returned positive for anti-Jo1 (medium-positive), anti-Ro52 (high-positive), and anti-MDA5 (weak-positive) autoantibodies. Therefore, the patient was begun on immunosuppressive therapy as the leading diagnosis included autoimmune myositis, possibly antisynthetase syndrome with interstitial lung disease (ILD). A year later, the patient presented with progressive shortness of breath, widespread macular erythematous facial rash, and new erythematous ulcerations over the fingertips. Imaging demonstrated a new SPM at this juncture. As the patient’s respiratory status continued to decline despite the use of immunosuppressive agents, a double-lung transplant was performed. Therefore, we propose that lung transplantation should be considered early in MDA5-SPM.

scholarly journals Adolescent-onset anti-MDA5 antibody-positive juvenile dermatomyositis with rapidly progressive interstitial lung disease and spontaneous pneumomediastinum: a case report and literature review

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Tsz-Wing Yeung ◽  
Kai-Ning Cheong ◽  
Yu-Lung Lau ◽  
Kei-Chiu Niko Tse
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Immunosuppressive Therapy ◽  
High Mortality ◽  
Inflammatory Myopathy ◽  
Unknown Etiology ◽  
Rapid Progression ◽  
Muscle Involvement ◽  
Spontaneous Pneumomediastinum ◽  
Increased Risk

Abstract Background Dermatomyositis with positive anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody has a distinct phenotype associated with small hand joint arthritis, mucocutaneous ulceration, palmar papules and less muscle involvement. It is also associated with increased risk of rapidly progressive interstitial lung disease (RP-ILD) and has a high mortality rate in adults. There is evidence that cases complicated with spontaneous pneumomediastinum (PNM) have an increase in mortality. While most of the evidence for this rare disease is derived from the adult literature, we report a case diagnosed in an adolescent complicated with both RP-ILD and PNM with a good outcome after aggressive immunosuppressive therapy. Our case also illustrates the potential challenges in diagnosis of this condition in the setting of non-specific clinical manifestations, the need for a high index of suspicion, and the importance of testing for myositis-specific antibodies (MSA) early to aid in diagnosis given the risk of rapid progression in these patients. Case presentation A 16-year-old Chinese female presented with fever and cough for 1 day, and finger swelling for 3 weeks. Physical examination revealed arthritis of fingers and wrists, ulcers and palmar papules over fingers, hyperpigmentation of interphalangeal joints, and rash over the neck. The diagnosis of dermatomyositis was made 1 month later with the onset of malar rash, Gottron’s papules, calcinosis and myalgia. The diagnosis was supported by the presence of anti-MDA5 antibody and evidence of inflammatory myopathy on magnetic resonance imaging. In retrospect, she already had interstitial lung disease at first presentation manifested as cough and opacity on chest radiograph, which was later confirmed with chest computed tomography. She was treated according to adult guidelines with steroid and calcineurin inhibitor. Her disease was resistant to initial therapy and was complicated by RP-ILD and spontaneous PNM. Intensive immunosuppressive therapy including cyclophosphamide and rituximab were required to induce remission. Conclusions Recognition of distinct clinical features of anti-MDA5 antibody-positive dermatomyositis and testing for MSA is crucial in patients with skin ulceration and abnormal pulmonary findings of unknown etiology, as prompt diagnosis with early aggressive treatment and anticipation of complications could make a difference in the outcome of this disease with high mortality.

scholarly journals Adolescent-Onset Anti-MDA5 Antibody-Positive Juvenile Dermatomyositis with Rapidly Progressive Interstitial Lung Disease and Spontaneous Pneumomediastinum: A Case Report and Literature Review

2021 ◽  
Author(s):  
Tsz Wing Yeung ◽  
Kai Ning Cheong ◽  
Yu Lung Lau ◽  
Kei Chiu Niko Tse
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Immunosuppressive Therapy ◽  
High Mortality ◽  
Inflammatory Myopathy ◽  
Diagnostic Challenge ◽  
Muscle Involvement ◽  
Spontaneous Pneumomediastinum ◽  
Small Hand ◽  
Joint Arthritis

Abstract Background:Dermatomyositis with positive anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody has a distinct phenotype associated with small hand joint arthritis, mucocutaneous ulceration, and less muscle involvement. It is reported to be associated with rapidly progressive interstitial lung disease (RP-ILD) and have a high mortality rate in adult studies. There is evidence that cases complicated with spontaneous pneumomediastinum (PNM) have an increase in mortality. Most of the evidence of this rare disease is derived from adult studies. We report a case in adolescent age complicated with both RP-ILD and PNM with a good disease outcome after aggressive immunosuppressive therapy. We illustrate the diagnostic challenge and the importance of test for the myositis-associated antibodies (MSA).Case presentation:A 16-year-old Chinese female presented with fever and cough for one day, and finger swelling for three weeks. Physical examination revealed arthritis of fingers and wrists, ulcers and palmar papules over fingers, hyperpigmentation of interphalangeal joints, rash over the neck and calcinosis over external ears. The diagnosis of dermatomyositis was made one month later until the onset of malar rash, Gottron’s papules and myalgia. The diagnosis was supported by the presence of anti-MDA5 antibody and evidence of inflammatory myopathy on magnetic resonance imaging without performing muscle biopsy or electromyography. In retrospect, she already had interstitial lung disease at first presentation manifested as cough and opacity on chest radiograph, which was later confirmed with chest computed tomography and pulmonary function test. She was treated according to adult guidelines with calcineurin inhibitor and steroid. Her disease was steroid-resistant, which was complicated with RP-ILD and spontaneous PNM. Intensive immunosuppressive therapy including cyclophosphamide and rituximab were required to induce remission.Conclusions:Recognition of distinct clinical features including mucocutaneous ulceration and test for MSA are important for prompt diagnosis of anti-MDA5 antibody-positive dermatomyositis, as early aggressive treatment and anticipation of complications could make a difference in the outcome of this disease with high mortality.

scholarly journals The First Successful Heart-Lung Transplant in a Korean Child with Humidifier Disinfectant-Associated Interstitial Lung Disease

2016 ◽  
Vol 31 (5) ◽  
pp. 817 ◽  
Author(s):  
Won Kyoung Jhang ◽  
Seong Jong Park ◽  
Eun Lee ◽  
Song I Yang ◽  
Soo Jong Hong ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Lung Transplant ◽  
Heart Lung Transplant ◽  
Korean Child

Increasing Opportunity for Lung Transplant in Interstitial Lung Disease With Pulmonary Hypertension

2018 ◽  
Vol 106 (6) ◽  
pp. 1812-1819 ◽  
Author(s):  
Scott Chicotka ◽  
Felipe E. Pedroso ◽  
Cara L. Agerstrand ◽  
Erika B. Rosenzweig ◽  
Darryl Abrams ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Lung Transplant

scholarly journals The lungs were on fire: a pilot study of 18F-FDG PET/CT in idiopathic-inflammatory-myopathy-related interstitial lung disease

2021 ◽  
Author(s):  
Junyu Liang ◽  
Heng Cao ◽  
Yinuo Liu ◽  
Bingjue Ye ◽  
Yiduo Sun ◽  
...  
Keyword(s):  
Lymph Node ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Ct Scan ◽  
Mediastinal Lymph Node ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Pet Ct ◽  
Bilateral Lung ◽  
Pet Ct Scan

Abstract Background: Interstitial lung disease (ILD) and its rapid progression (RP) were main contributors to unfavorable outcome of idiopathic inflammatory myopathy (IIM) patients. This study aimed at identifying the clinical value of PET/CT scan in IIM-ILD patients as well as constructing a predicting model for RP-ILD.Methods: Adult IIM-ILD patients who were hospitalized at four divisions of the First Affiliated Hospital, Zhejiang University School of Medicine (FAHZJU) from Jannuary 1st 2017 to December 31st 2020 were reviewed. PET/CT scan as well as other factors of patients who met the inclusion and exclusion criteria were collected and analyzed.Results: A total of 61 IIM-ILD patients were finally enrolled into this study. Twenty-one patients (34.4%) developed RP-ILD and 24 patients (39.3%) died in follow-up. After false discovery rate (FDR) correction, percent-predicted diffusing capacity of the lung for carbon monoxide (DLCO%, P=0.014), bilateral lung mean standard uptake value (SUVmean, P=0.014) and abnormal mediastinal lymph node (P=0.045) were significantly different in comparison between RP-ILD and non-RP-ILD groups. A “DLM” model was hereby established by including the above three values to predict RP-ILD with a cutoff value of ≥2 and an area under the curve (AUC) of 0.905. Higher bilateral lung SUVmean (P=0.019) and spleen SUVmean (P=0.011) were observed in IIM-ILD patients who died within three months, and a moderate correlation was recognized between the two values. Conclusions: Elevated bilateral lung SUVmean and abnormal mediastinal lymph node were associated with RP-ILD in IIM-ILD patients. The “DLM” model was valuable in predicting RP-ILD and demanded further evaluation.

scholarly journals Risk factors associated with Pneumocystis jirovecii pneumonia in juvenile myositis in North America

Rheumatology ◽  
2020 ◽  
Author(s):  
Sara E Sabbagh ◽  
Jessica Neely ◽  
Albert Chow ◽  
Marietta DeGuzman ◽  
Jamie Lai ◽  
...  
Keyword(s):  
Risk Factors ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Immunosuppressive Therapy ◽  
Inflammatory Myopathy ◽  
Severe Infection ◽  
Idiopathic Inflammatory Myopathy ◽  
Pneumocystis Jirovecii ◽  
Pneumocystis Jirovecii Pneumonia ◽  
The Usa

Abstract Objectives Pneumocystis jirovecii pneumonia (PJP) is associated with significant morbidity and mortality in adult myositis patients; however, there are few studies examining PJP in juvenile myositis [juvenile idiopathic inflammatory myopathy (JIIM)]. The purpose of this study was to determine the risk factors and clinical phenotypes associated with PJP in JIIM. Methods An research electronic data capture (REDCap) questionnaire regarding myositis features, disease course, medications and PJP infection characteristics was completed by treating physicians for 13 JIIM patients who developed PJP (PJP+) from the USA and Canada. Myositis features and medications were compared with 147 JIIM patients without PJP (PJP–) from similar geographic regions who enrolled in National Institutes of Health natural history studies. Results PJP+ patients were more often of Asian ancestry than PJP– patients [odds ratio (OR) 8.7; 95% CI 1.3, 57.9]. Anti- melanoma differentiation associated protein 5 (MDA5) autoantibodies (OR 12.5; 95% CI 3.0, 52.4), digital infarcts (OR 43.8; 95% CI 4.2, 460.2), skin ulcerations (OR 12.0; 95% CI 3.5, 41.2) and interstitial lung disease (OR 10.6; 95% CI 2.1, 53.9) were more frequent in PJP+ patients. Before PJP diagnosis, patients more frequently received pulse steroids, rituximab and more immunosuppressive therapy compared with PJP– patients. Seven PJP+ patients were admitted to the intensive care unit and four patients died due to PJP or its complications. Conclusions PJP is a severe infection in JIIM that can be associated with mortality. Having PJP was associated with more immunosuppressive therapy, anti-MDA5 autoantibodies, Asian race and certain clinical features, including digital infarcts, cutaneous ulcerations and interstitial lung disease. Prophylaxis for PJP should be considered in juvenile myositis patients with these features.

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