A 45-year-old woman comes to the office due to a 3-month history of progressive exertional dyspnea and nonproductive cough. She has had no chest pain or pedal edema but reports intermittent episodes of difficulty swallowing. The patient has a history of hypertension and severe gastroesophageal reflux and has had Raynaud phenomenon for 2 years. She does not use tobacco or alcohol. Blood pressure is 142/86 mm Hg and pulse is 80/min. Physical examination shows the skin over her face, hands, arms, and trunk to be thick and firm. Scattered crackles are heard on lung auscultation. Which of the following is the most likely cause of this patient's current symptoms?

Question

A 45-year-old woman comes to the office due to a 3-month history of progressive exertional dyspnea and nonproductive cough.  She has had no chest pain or pedal edema but reports intermittent episodes of difficulty swallowing.  The patient has a history of hypertension and severe gastroesophageal reflux and has had Raynaud phenomenon for 2 years.  She does not use tobacco or alcohol.  Blood pressure is 142/86 mm Hg and pulse is 80/min.  Physical examination shows the skin over her face, hands, arms, and trunk to be thick and firm.  Scattered crackles are heard on lung auscultation.  Which of the following is the most likely cause of this patient's current symptoms?

Quizplus · Accepted Answer

11ec25c8_d52a_edda_bc81_e3826bd52cdb_MD0003_00 This patient with progressive dyspnea and nonproductive cough has most likely developed interstitial lung disease (ILD) due to underlying systemic sclerosis (SS). Of the 2 subtypes of SS, diffuse cutaneous SS (dcSS) carries the stronger association with internal organ involvement, and ILD occurs in approximately 40% of cases. Considerable overlap exists in the clinical manifestations of dcSS and limited cutaneous SS (lcSS). Although vascular manifestations are more prominent in lcSS, Raynaud phenomenon is common in both subtypes. In addition, esophageal dysmotility with gastroesophageal reflux is commonly seen in both subtypes. In contrast, skin thickening involving the trunk is characteristic of dcSS. ILD also occurs in patients with lcSS, but less frequently and typically less extensively than in those with dcSS. The stronger association of dcSS with internal organ involvement leads to an overall worse prognosis compared to lcSS. (Choice A) Chemical pneumonitis refers to damage to the lower airways due to aspiration of gastric contents (eg, gastric acid). Patients often present acutely in respiratory distress and have a history of dysphagia (eg, stroke) or reduced consciousness (eg, alcoholism). This patient's dysphagia puts her at risk for chemical pneumonitis, but her progressive dyspnea and features of SS (eg, Raynaud phenomenon) make ILD more likely. (Choice C) Cardiac involvement in SS can occur in the form of myocardial fibrosis, typically leading to ventricular diastolic dysfunction. Patients have symptoms of heart failure (eg, dyspnea, peripheral edema); absence of peripheral edema makes the diagnosis less likely. (Choice D) Bronchiolitis obliterans is an obstructive lung disease caused by proliferative narrowing of medium and small bronchioles. It can occur following viral infection or inhalation injury and is also associated with rheumatologic disease. However, it is rare in patients with SS and is more likely to present with wheezing (rather than crackles) on lung auscultation. (Choice E) Restriction of chest movement affecting respiration is most commonly seen in patients with obesity hypoventilation syndrome. It may occur in dcSS due to extensive thoracic skin fibrosis but is quite rare. Educational objective: Systemic sclerosis (SS) is divided into diffuse cutaneous and limited cutaneous subtypes. Although there is considerable overlap in disease manifestations between the 2 subtypes, internal organ involvement (eg, interstitial lung disease) is more common and more extensive in diffuse cutaneous SS, leading to a worse prognosis. References: Interstitial lung disease in systemic sclerosis: pathophysiology, current and new advances in therapy. (http://www.ncbi.nlm.nih.gov/pubmed/22506881)

A 45-Year-Old Woman Comes to the Office Due to a 3-Month