A 65-year-old woman comes to the emergency department due to worsening cough and difficulty breathing. The patient had similar symptoms a month ago and was found to have right-sided pneumonia. The symptoms resolved with antibiotic treatment but recurred 2 days ago. During the previous hospitalization, the patient also reported progressive muscle weakness and fatigue of several months' duration. She subsequently underwent muscle biopsy, which revealed dermatomyositis; oral prednisone therapy was initiated 2 weeks ago. She still has significant muscle weakness. The patient also has had poor oral intake and frequent coughing while eating, and has lost 6.8 kg (15 lb) over the last 3 months. The patient feels mildly depressed but has had no suicidal ideation. She is a lifelong nonsmoker and does not drink alcohol.
Temperature is 38.6 C (101.5 F), blood pressure is 122/78 mm Hg, pulse is 89/min, and respirations are 20/min. Bronchial breath sounds and rhonchi are present over the right lung base. Muscle strength is moderately decreased in the proximal muscles. There is a scaly skin rash over the extensor surfaces of both hands.
Laboratory results are as follows:
Chest x-ray shows a right lower lobe lung infiltrate.
Which of the following is most likely responsible for this patient's pulmonary symptoms?

Question

A 65-year-old woman comes to the emergency department due to worsening cough and difficulty breathing.  The patient had similar symptoms a month ago and was found to have right-sided pneumonia.  The symptoms resolved with antibiotic treatment but recurred 2 days ago.  During the previous hospitalization, the patient also reported progressive muscle weakness and fatigue of several months' duration.  She subsequently underwent muscle biopsy, which revealed dermatomyositis; oral prednisone therapy was initiated 2 weeks ago.  She still has significant muscle weakness.  The patient also has had poor oral intake and frequent coughing while eating, and has lost 6.8 kg (15 lb) over the last 3 months.  The patient feels mildly depressed but has had no suicidal ideation.  She is a lifelong nonsmoker and does not drink alcohol.
Temperature is 38.6 C (101.5 F), blood pressure is 122/78 mm Hg, pulse is 89/min, and respirations are 20/min.  Bronchial breath sounds and rhonchi are present over the right lung base.  Muscle strength is moderately decreased in the proximal muscles.  There is a scaly skin rash over the extensor surfaces of both hands.
Laboratory results are as follows:
 Chest x-ray shows a right lower lobe lung infiltrate.
Which of the following is most likely responsible for this patient's pulmonary symptoms?

Quizplus · Accepted Answer

11ec193a_9726_4d64_853d_c7720ea2ddb9_MD0002_00 Dermatomyositis can cause weakness of the oropharyngeal and striated muscles in the upper third of the esophagus. This may lead to dysphagia for both solids and liquids and aspiration with recurrent pneumonia, as seen in this patient. Associated findings may include hoarseness and a nasal quality to the voice. Dermatomyositis-related dysphagia can be evaluated with manometry and a videofluoroscopic swallowing study, which typically shows pharyngeal pooling and nasal regurgitation. Treatment initially involves patient education on aspiration risk, elevation of the head of the bed, and semi-thick diets. Dysphagia may respond to treatment of the underlying myopathy with systemic glucocorticoids, although this is not well studied. Patients who are refractory to conservative measures may require a nasopharyngeal or gastric feeding tube for nutrition and airway protection. (Choice B) Impaired mucociliary clearance is common in smokers and is also seen in a variety of disorders, including Sjögren syndrome, cystic fibrosis, and primary ciliary dyskinesia. This patient is a lifelong nonsmoker and does not have any of these conditions. Although patients with impaired mucociliary clearance have an increased incidence of pulmonary infections, recurrent right lower lobe pneumonia suggests aspiration. (Choice C) Mycobacterial infection typically presents with upper-lobe disease (reactivation tuberculosis) or perihilar infiltrates (primary tuberculosis) with hilar adenopathy. This patient's initial improvement with antibiotics and chest x-ray findings make this less likely. (Choice D) Necrotizing vasculitis is seen in polyarteritis nodosa, eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome), rheumatoid arthritis, systemic lupus erythematosus, scleroderma, and granulomatosis with polyangiitis (formerly Wegener granulomatosis), but is not common in dermatomyositis. Vasculitis also often causes acute/chronic kidney injury, which is not seen in this patient. (Choice E) Dermatomyositis is associated with interstitial lung disease, including interstitial pneumonia. However, examination typically shows fine ("Velcro") crackles, and imaging would show diffuse reticulonodular interstitial opacities, predominantly at the lung bases. Educational objective: Dermatomyositis can cause weakness of the oropharyngeal and striated muscles in the upper third of the esophagus. This may lead to dysphagia for both solids and liquids and aspiration with recurrent pneumonia. __________ References: Review article: the gastrointestinal complications of myositis. (http://www.ncbi.nlm.nih.gov/pubmed/19886949) Oropharyngeal dysphagia in dermatomyositis: associations with clinical and laboratory features including autoantibodies. (http://www.ncbi.nlm.nih.gov/pubmed/27167831)

A 65-Year-Old Woman Comes to the Emergency Department Due to Worsening