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Patient With Pulmonary Symptoms, Dysphagia, and Raynaud Disease

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 73-year-old man with a history of gastroesophageal reflux disease and Raynaud disease but no history of cigarette smoking presented to the emergency department with 4 weeks of dysphagia and a 4.54-kg (10-lb) weight loss over 3 months. He had no nausea, vomiting, abdominal pain, melena, or hematochezia but reported a cough productive of yellow sputum, dyspnea on exertion, and fatigue. The patient had been treated for presumed pneumonia with 3 courses of azithromycin over the prior 3 months due to infiltrates on a chest radiograph. He reported no known ill contacts or recent travel outside the US. At presentation, his heart rate and blood pressure were normal, and oxygen saturation was 95% on room air. Physical examination revealed a maculopapular rash over his neck and chest (Figure, left panel) and bibasilar crackles on lung auscultation. Laboratory testing showed a white blood cell count of 14.3 × 103/μL (reference, 4.2-9.1 × 103/μL); creatine kinase level, 2060 U/L (34.40 μkat/L) (reference, 44-196 U/L [0.73-3.27 μkat/L]), aldolase level, 18 U/L (0.30 μkat/L) (reference, <8 U/L [<0.13 μkat/L]), C-reactive protein level, 30 mg/L (reference, <8 mg/L), and erythrocyte sedimentation rate, 35 mm/h (reference, <20 mm/h). Antinuclear antibody immunofluorescent assay findings were positive. Results from testing for HIV, hepatitis B, and hepatitis C were negative. A barium swallow study showed no visualized aspiration. Endoscopy revealed clean-based esophageal and duodenal ulcers and nonspecific gastritis. Chest computed tomography (CT) showed bibasilar pulmonary consolidations and ground glass opacities (Figure, right panel).

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A 73-year-old man with a history of gastroesophageal reflux disease and Raynaud disease but no history of cigarette smoking presented to the emergency department with 4 weeks of dysphagia and a 4.54-kg (10-lb) weight loss over 3 months. He had no nausea, vomiting, abdominal pain, melena, or hematochezia but reported a cough productive of yellow sputum, dyspnea on exertion, and fatigue. The patient had been treated for presumed pneumonia with 3 courses of azithromycin over the prior 3 months due to infiltrates on a chest radiograph. He reported no known ill contacts or recent travel outside the US. At presentation, his heart rate and blood pressure were normal, and oxygen saturation was 95% on room air. Physical examination revealed a maculopapular rash over his neck and chest (Figure, left panel) and bibasilar crackles on lung auscultation. Laboratory testing showed a white blood cell count of 14.3 × 103/μL (reference, 4.2-9.1 × 103/μL); creatine kinase level, 2060 U/L (34.40 μkat/L) (reference, 44-196 U/L [0.73-3.27 μkat/L]), aldolase level, 18 U/L (0.30 μkat/L) (reference, <8 U/L [<0.13 μkat/L]), C-reactive protein level, 30 mg/L (reference, <8 mg/L), and erythrocyte sedimentation rate, 35 mm/h (reference, <20 mm/h). Antinuclear antibody immunofluorescent assay findings were positive. Results from testing for HIV, hepatitis B, and hepatitis C were negative. A barium swallow study showed no visualized aspiration. Endoscopy revealed clean-based esophageal and duodenal ulcers and nonspecific gastritis. Chest computed tomography (CT) showed bibasilar pulmonary consolidations and ground glass opacities (Figure, right panel).

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Article Information

Corresponding Author: Priya Agarwala, MD, NYU Langone Health, 259 First St, Mineola, NY 11501 (Priya.agarwala@NYUlangone.org).

Published Online: July 27, 2023. doi:10.1001/jama.2023.13766

Conflict of Interest Disclosures: None reported.

Additional Information: We thank the patient for providing permission to share his information.

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Zhao  N , Jiang  W , Wu  H ,  et al.  Clinical features, prognostic factors, and survival of patients with antisynthetase syndrome and interstitial lung disease.   Front Immunol. Published online August 10, 2022. doi:10.3389/fimmu.2022.872615Google ScholarCrossref
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Huang  K , Aggarwal  R.   Antisynthetase syndrome: a distinct disease spectrum.   J Scleroderma Relat Disord. 2020;5(3):178-191. doi:10.1177/2397198320902667PubMedGoogle ScholarCrossref
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AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 credit toward the CME [and Self-Assessment requirements] of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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