Logo American Journal of Case Reports

Call: 1.631.629.4328
Mon-Fri 10 am - 2 pm EST

Contact Us

Logo American Journal of Case Reports Logo American Journal of Case Reports Logo American Journal of Case Reports

25 April 2017 : Case report  USA

A Rare Case of Necrotizing Myopathy and Fibrinous and Organizing Pneumonia with Anti-EJ Antisynthetase Syndrome and SSA Antibodies

Challenging differential diagnosis, Unusual or unexpected effect of treatment, Unexpected drug reaction, Educational Purpose (only if useful for a systematic review or synthesis), Rare coexistence of disease or pathology

Muhammad Kashif1AEF, Divya Arya2BEF, Masooma Niazi3CDE, Misbahuddin Khaja1ABCDEF*

DOI: 10.12659/AJCR.903540

Am J Case Rep 2017; 18:448-453

Abstract

BACKGROUND: Idiopathic inflammatory myopathies are autoimmune disorders that can involve the skin, joints, muscles, and lungs. The most common of these disorders are dermatomyositis, polymyositis, overlap syndrome, and inclusion body myositis. Necrotizing autoimmune myopathy is an idiopathic inflammatory myopathy that is rarely associated with Sjögren’s syndrome. The most common lung findings associated with anti-EJ antisynthetase syndrome are nonspecific interstitial pneumonia and usual interstitial pneumonia; this condition is rarely associated with fibrinous and organizing pneumonia.

CASE REPORT: Here, we present a rare case of necrotizing myopathy and fibrinous and organizing pneumonia in a 34-year-old African American man with Sjögren’s syndrome and anti-EJ antibodies. The patient’s presenting symptoms were cough and proximal muscle weakness of the extremities. He had elevated serum creatine kinase level, aldolase level, and erythrocyte sedimentation rate. Myositis panel was positive for anti-EJ antibodies. Chest radiography was consistent with bilateral interstitial infiltrates. CT chest showed patchy bilateral infiltrates. Quadriceps muscle biopsy revealed widespread necrotic fibers and lung biopsy showed fibrinous and organizing pneumonia. The patient responded well to immunoglobulin therapy, mycophenolate, and prednisone, which resulted in complete resolution of bilateral infiltrates and improved muscle pain and weakness.

CONCLUSIONS: Myopathies are characterized by myalgia and muscle weakness due to muscle fiber dysfunction and are associated with autoimmune diseases. Histopathological features may differ in idiopathic inflammatory myopathies. It is important to recognize the rare association of anti-EJ autoantibodies with necrotizing myopathy and interstitial lung disease, which responds well to methylprednisolone and intravenous immunoglobulin.

Keywords: Lung Diseases, Interstitial, myositis, Sjogren's Syndrome

Add Comment 0 Comments

In Press

12 Feb 2024 : Case report  Germany

Diagnostic Challenges and Imaging Considerations for Intraparotid Facial Nerve Schwannoma: A Case Report an...

Am J Case Rep In Press; DOI: 10.12659/AJCR.942870  

0:00

12 Feb 2024 : Case report  USA

Erdheim-Chester Disease Occult on Radiographs and CT but Visible on MRI and PET

Am J Case Rep In Press; DOI: 10.12659/AJCR.941169  

0:00

12 Feb 2024 : Case report  USA

Surgical Treatment of Spontaneous Superficial Temporal Artery Arteriovenous Malformation: A Case Report

Am J Case Rep In Press; DOI: 10.12659/AJCR.942839  

13 Feb 2024 : Case report  Bahrain

Warfarin Woes: A Rare Case of Hemoperitoneum with Intramural Small Bowel Hematoma

Am J Case Rep In Press; DOI: 10.12659/AJCR.943519  

Most Viewed Current Articles

10 Jan 2022 : Case report  Germany

A Report on the First 7 Sequential Patients Treated Within the C-Reactive Protein Apheresis in COVID (CACOV...

DOI :10.12659/AJCR.935263

Am J Case Rep 2022; 23:e935263

19 Jul 2022 : Case report  Saudi Arabia

Atlantoaxial Subluxation Secondary to SARS-CoV-2 Infection: A Rare Orthopedic Complication from COVID-19

DOI :10.12659/AJCR.936128

Am J Case Rep 2022; 23:e936128

05 Mar 2022 : Case report  Brazil

Acute Limb Ischemia After Self-Injection of Crushed Morphine Tablets into the Radial Artery: Role of Infrar...

DOI :10.12659/AJCR.935336

Am J Case Rep 2022; 23:e935336

02 Apr 2022 : Case report  Saudi Arabia

Infective Endocarditis Caused by Pseudomonas luteola in a Pediatric Patient. A Case Report and Review of Li...

DOI :10.12659/AJCR.935743

Am J Case Rep 2022; 23:e935743

Your Privacy

We use cookies to ensure the functionality of our website, to personalize content and advertising, to provide social media features, and to analyze our traffic. If you allow us to do so, we also inform our social media, advertising and analysis partners about your use of our website, You can decise for yourself which categories you you want to deny or allow. Please note that based on your settings not all functionalities of the site are available. View our privacy policy.

American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923