A 38-year-old woman with no past medical history presents with fatigue, diffuse joint pain, and difficulty breathing upon exertion. The symptoms have developed gradually over the past few months. On review of systems, she also endorses difficulty swallowing and an increased sensitivity to cold temperatures, which make her fingers turn blue. Her vitals show a temperature of 37.3°C, blood pressure of 132/86 mmHg, heart rate of 82 beats per minute, and respiratory rate of 20 breaths per minute. Dry crackles are heard during auscultation primarily over the middle of the lung fields. On skin examination, the patient has skin tightening around the mouth and hands. There are scattered telangiectasias as well as distal calcifications of the fingertips with some ulcerations. An initial workup with laboratory values and imaging is ordered, and pulmonary function testing is performed.
What is the most common cause of death in patients with this disease?
Pulmonary disease. This patient has systemic sclerosis (scleroderma) and is presenting with findings suggestive of CREST syndrome (Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasias), which is more commonly associated with the limited form of the disease. The diffuse form usually has central skin involvement (upper arms and chest) rather than just acral involvement. Patients with both the limited and the diffuse form have an increased risk of pulmonary fibrosis, which is seen in this patient. Pulmonary function tests will show a restrictive pattern and a loss in total lung volume. The most common cause of death is from pulmonary fibrosis and pulmonary hypertension. (A) Cardiac disease may include pericarditis, myocardial fibrosis, conduction abnormalities, and changes secondary to pulmonary hypertension from lung disease. These findings are less common than lung findings and are not the most common cause of death. (C) Renal disease can present as scleroderma renal crisis, which is acute in onset and rapidly progressive. Before dialysis, this was a major cause of death. (D) Secondary infections of the diseased lungs may occur but is not the most common cause of death.
A 59-year-old woman presents with a 2-month history of left shoulder pain and arm weakness. Her past medical history and family history are unremarkable, and she only takes a daily multivitamin. She drinks 2 glasses of wine daily and smokes a pack of cigarettes daily, which she has done since the age of 18. On examination, her vitals are normal. The lungs are clear bilaterally but there is decreased diaphragmatic excursion on the left. On neurologic examination, her right pupil is larger than the left; when a light is shone into the right eye, the pupils become equal in size.
Which of the following is true regarding this condition?
There may be atrophy and weakness of the intrinsic muscles of the hand. This patient has Pancoast syndrome, which is caused by an apical tumor in the superior sulcus that causes symptoms based on the structures on which it impinges. (A) The tumor may involve the C8 and T1 nerve roots, which leads to atrophy and weakness of the intrinsic hand muscles. Tumors in this location can also impinge on the sympathetic chain, causing ipsilateral Horner syndrome. (C) This patient shows signs of Horner syndrome (left-sided ptosis and miosis); loss of sympathetics to the face would also cause ipsilateral (left-sided, not right-sided) anhidrosis and flushing of the face, since the loss of norepinephrine to cutaneous vessels leads to vasodilation. (D) In some cases, the tumor can also compress the superior vena cava (SVC) and cause SVC syndrome, which can manifest with a feeling of facial fullness, distended neck veins, and facial plethora. It would not cause vascular congestion of the abdomen or lower extremities. This patient also has involvement of the phrenic nerve, manifested by decreased diaphragmatic excursion on the left side. For the shelf examination, whenever there is a vignette with a confusing constellation of findings, try to explain them all with one diagnosis; if you do not understand one part of the presentation (such as the phrenic nerve palsy), then focus on the rest of the findings.
A 65-year-old man is brought in to the hospital by his wife because of fatigue and progressive confusion. His laboratory values are shown below.
The patient is admitted and given hypertonic saline. A lung mass is subsequently found during chest imaging. This type of cancer is UNLIKELY to cause which of the following physical examination findings?
Ptosis and anisocoria. Small cell lung cancer commonly causes the syndrome of inappropriate ADH (SIADH), which is seen in this patient with severe hyponatremia as a result of excessive water retention. Ptosis (eyelid droop) with miosis (causing anisocoria, or asymmetry of pupil sizes) is seen in Horner syndrome, with one cause being non–small cell lung cancer that impinges on the sympathetic chain. (B, C, D) These answer choices are more commonly associated with small cell lung cancer than with Horner syndrome. Moon facies, central adiposity, and striae are manifestations of Cushing syndrome, which is caused by ectopic production of ACTH. Vascular congestion is seen in superior vena cava (SVC) syndrome, which is caused by a mass obstructing the SVC that leads to increased venous pressure in the face and upper extremities. Symmetric muscle weakness, especially with initial use (vs. fatigability in myasthenia gravis) suggests Lambert–Eaton syndrome with antibodies directed at presynaptic calcium channels.
A 32-year-old man is brought in by paramedics after being involved in a motor vehicle collision. He has multiple fractures and is hypotensive and tachycardic. Heart sounds are normal, but his neck veins are distended and there are decreased breath sounds on the left side with tracheal shift to the right.
Which of the following should be done immediately?
Needle thoracostomy in the second intercostal space along the midclavicular line. This patient has a tension pneumothorax, which may occur after trauma, mechanical ventilation, or in patients with underlying pulmonary disease. The diagnosis is highly suggested by the findings of decreased breath sounds on the affected side, tracheal shift away from the affected side, elevated jugular venous pressure, and hypotension. It is caused by a rupture in the visceral pleura that creates a flap, acting as a oneway valve that causes air trapping between the visceral and parietal pleura with each inspiration. This increases the pressure in the intrapleural space, causing collapse of the affected lung and compression of the other lung and the mediastinum. If it progresses, cardiac output will decrease and hypotension will result. If there are findings on history or examination that suggest this diagnosis, immediate decompression with needle thoracostomy should be performed. (A) This is an emergency; immediate management is lifesaving and should not be delayed for diagnostic confirmation with imaging. (B) Chest tube placement should occur after needle thoracostomy, but this takes time to perform and therefore is not the initial step. (D) Thoracotomy is a last ditch effort in patient resuscitation and is not a good option here. Indications for emergency thoracotomy include penetrating or blunt trauma with hemodynamic instability despite fluid resuscitation en route to the Emergency Department. Patients with severe intrathoracic processes such as cardiac tamponade, a large air embolism, or major intrathoracic bleeding from large vessels will benefit most from this procedure.
A 33-year-old woman presents with progressive shortness of breath. The symptoms started with exertion, but now she has dyspnea at rest. She has no previous medical problems, no family history of heart or lung disease, and she has never smoked. On examination, she has a holosystolic murmur and an S4, both heard along the left sternal border, and there is jugular venous distention. Her lungs are clear bilaterally with no wheezes or rales. Her lower extremities are nontender and symmetric in size. She is admitted and given supplemental oxygen. A chest x-ray shows mild dilatation of the pulmonary arteries, and the lung parenchyma is normal in appearance.
What is the most likely diagnosis?
: Pulmonary arterial hypertension. Pulmonary hypertension can be a primary disease process or secondary to other disease processes. Pulmonary arterial hypertension is an idiopathic form of pulmonary hypertension that occurs in the absence of another disease process. It is more common in women, typically manifests around the mid-30’s, and has a poor prognosis. If the patient were to undergo right heart catheterization, the right atrial, right ventricular, and pulmonary artery pressures would be elevated with a normal PCWP. The mean pulmonary artery pressure would be >25 mmHg (normal range 9 to 18 mmHg). The clues to the diagnosis include the symptom of progressive dyspnea, evidence of right heart strain/failure (tricuspid regurgitation and right-sided S4), and a chest x-ray showing enlarged pulmonary arteries without any other lung disease.
(B, C, D) These are all causes of secondary pulmonary hypertension. The patient has no rales on lung examination, making left heart failure less likely. She has no clinical evidence of a DVT, and there are no suggested risk factors for her being in a hypercoagulable state, making pulmonary embolism less likely. COPD is also unlikely given that she does not have a smoking history and has no wheezes on examination.
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