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Inclusion Body Myositis: A Case Presenting with Respiratory Failure and Autopsy Findings Leading to the Hypothesis of a Paraneoplastic Cause
Patient: Female, 48 Final Diagnosis: Inclusion body myositis Symptoms: Shortness of breath • weakness Medication: — Clinical Procedure: Biopsy Specialty: Neurology OBJECTIVE: Rare disease BACKGROUND: Sporadic inclusion body myositis (IBM) is the most common acquired myopathy seen in adults aged over...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
International Scientific Literature, Inc.
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5490508/ https://www.ncbi.nlm.nih.gov/pubmed/28642454 http://dx.doi.org/10.12659/AJCR.903566 |
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author | Dardis, Christopher Antezana, Ariel Tanji, Kurenai Maccabee, Paul |
author_facet | Dardis, Christopher Antezana, Ariel Tanji, Kurenai Maccabee, Paul |
author_sort | Dardis, Christopher |
collection | PubMed |
description | Patient: Female, 48 Final Diagnosis: Inclusion body myositis Symptoms: Shortness of breath • weakness Medication: — Clinical Procedure: Biopsy Specialty: Neurology OBJECTIVE: Rare disease BACKGROUND: Sporadic inclusion body myositis (IBM) is the most common acquired myopathy seen in adults aged over 50 years, with a prevalence estimated at between 1 and 70 per million. Weakness of the diaphragm with loss of vital capacity is almost universal in IBM. This is almost always asymptomatic. When respiratory complications occur, they are most often due to aspiration. Respiratory failure due to diaphragmatic weakness is exceptionally rare, particularly as the presenting symptom of the disease. It is not currently considered to be a paraneoplastic syndrome. CASE REPORT: Our patient presented with hypercarbic respiratory failure. This is the first such reported case without signs of weakness elsewhere of which we are aware. We suspected IBM based on her history of progressive weakness and findings on electromyography. There was a delay of 5 years in obtaining biopsy for confirmation, during which she presented with recurrent episodes of respiratory failure despite using non-invasive ventilation. An autopsy revealed the presence of papillary thyroid carcinoma with spread to local lymph nodes. On the basis that these co-morbidities are unlikely to have occurred by chance (we estimate 1×10(−17)), we hypothesize that IBM may be a paraneoplastic condition. We acknowledge that proof would require demonstrating a pathogenic antibody. CONCLUSIONS: IBM should be considered in older patients (age >45) presenting with otherwise unexplained respiratory failure. A workup for possible malignancy in this setting appears reasonable. |
format | Online Article Text |
id | pubmed-5490508 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | International Scientific Literature, Inc. |
record_format | MEDLINE/PubMed |
spelling | pubmed-54905082017-07-05 Inclusion Body Myositis: A Case Presenting with Respiratory Failure and Autopsy Findings Leading to the Hypothesis of a Paraneoplastic Cause Dardis, Christopher Antezana, Ariel Tanji, Kurenai Maccabee, Paul Am J Case Rep Articles Patient: Female, 48 Final Diagnosis: Inclusion body myositis Symptoms: Shortness of breath • weakness Medication: — Clinical Procedure: Biopsy Specialty: Neurology OBJECTIVE: Rare disease BACKGROUND: Sporadic inclusion body myositis (IBM) is the most common acquired myopathy seen in adults aged over 50 years, with a prevalence estimated at between 1 and 70 per million. Weakness of the diaphragm with loss of vital capacity is almost universal in IBM. This is almost always asymptomatic. When respiratory complications occur, they are most often due to aspiration. Respiratory failure due to diaphragmatic weakness is exceptionally rare, particularly as the presenting symptom of the disease. It is not currently considered to be a paraneoplastic syndrome. CASE REPORT: Our patient presented with hypercarbic respiratory failure. This is the first such reported case without signs of weakness elsewhere of which we are aware. We suspected IBM based on her history of progressive weakness and findings on electromyography. There was a delay of 5 years in obtaining biopsy for confirmation, during which she presented with recurrent episodes of respiratory failure despite using non-invasive ventilation. An autopsy revealed the presence of papillary thyroid carcinoma with spread to local lymph nodes. On the basis that these co-morbidities are unlikely to have occurred by chance (we estimate 1×10(−17)), we hypothesize that IBM may be a paraneoplastic condition. We acknowledge that proof would require demonstrating a pathogenic antibody. CONCLUSIONS: IBM should be considered in older patients (age >45) presenting with otherwise unexplained respiratory failure. A workup for possible malignancy in this setting appears reasonable. International Scientific Literature, Inc. 2017-06-23 /pmc/articles/PMC5490508/ /pubmed/28642454 http://dx.doi.org/10.12659/AJCR.903566 Text en © Am J Case Rep, 2017 This work is licensed under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0 (https://creativecommons.org/licenses/by-nc-nd/4.0/) ) |
spellingShingle | Articles Dardis, Christopher Antezana, Ariel Tanji, Kurenai Maccabee, Paul Inclusion Body Myositis: A Case Presenting with Respiratory Failure and Autopsy Findings Leading to the Hypothesis of a Paraneoplastic Cause |
title | Inclusion Body Myositis: A Case Presenting with Respiratory Failure and Autopsy Findings Leading to the Hypothesis of a Paraneoplastic Cause |
title_full | Inclusion Body Myositis: A Case Presenting with Respiratory Failure and Autopsy Findings Leading to the Hypothesis of a Paraneoplastic Cause |
title_fullStr | Inclusion Body Myositis: A Case Presenting with Respiratory Failure and Autopsy Findings Leading to the Hypothesis of a Paraneoplastic Cause |
title_full_unstemmed | Inclusion Body Myositis: A Case Presenting with Respiratory Failure and Autopsy Findings Leading to the Hypothesis of a Paraneoplastic Cause |
title_short | Inclusion Body Myositis: A Case Presenting with Respiratory Failure and Autopsy Findings Leading to the Hypothesis of a Paraneoplastic Cause |
title_sort | inclusion body myositis: a case presenting with respiratory failure and autopsy findings leading to the hypothesis of a paraneoplastic cause |
topic | Articles |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5490508/ https://www.ncbi.nlm.nih.gov/pubmed/28642454 http://dx.doi.org/10.12659/AJCR.903566 |
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