A Cystic Fibrosis Patient with Hemoptysis From an Unusual Cause

Sugeet Jagpal, MD

Fellow

Division of Pulmonary and Critical Care Medicine

Rutgers University Robert Wood Johnson Medical School

New Brunswick, New Jersey

Vyacheslav Gendel, MD

Division of Interventional Radiology

Rutgers University Robert Wood Johnson Medical School

New Brunswick, New Jersey

John L. Nosher, MD

Division of Interventional Radiology

Rutgers University Robert Wood Johnson Medical School

New Brunswick, New Jersey

Lakshmi Uppaluri, MBBS

Assistant Professor

Department of Pediatrics

Rutgers University Robert Wood Johnson Medical School

New Brunswick, New Jersey

A Caucasian child had Cystic Fibrosis (CF) diagnosed at birth with a positive sweat chloride test, and required surgery as a neonate for meconium ileus. A liver biopsy at three months of age demonstrated focal biliary cirrhosis, and the patient suffered from esophageal varices that required sclerotherapy until eighteen months of age. From the age of two onwards the patient did not have further clinical manifestations of cirrhosis. Her pulmonary symptoms were not significant, and imaging demonstrated mild bronchiectasis of the right middle and lower lobes. Genotyping was done during adolescence, and she was found to have one copy of ΔF508 while the second mutation remained unknown.

After being lost to follow up for several years, the patient presented to our emergency room at the age of seventeen with nausea and dizziness.

The vitals on admission revealed mild sinus tachycardia with a pulse of 90/minute, systolic blood pressure between 90-100 mm Hg range, but no tachypnea, pain or fever. Exam demonstrated a thin female who was developmentally normal for her age. She did not have any overt manifestations of cirrhosis, with no spider angiomas, palmer erythema, or asterexis noted. Her lung exam was positive for mild crackles at the right lung base, and she did not have clubbing. Abdominal exam was benign with normoactive bowel sounds.

Orthostatics were attempted and rapidly aborted when the patient vomited a moderate amount of bright red blood. Blood work demonstrated that her hemoglobin and platelets had decreased compared to prior labs (9 gm/dl Hg and platelets 100,000/ µL), and she was admitted to the pediatric intensive care unit. She was given packed red blood cells, esomeprazole and octreotide drips were administered, and endoscopy was planned.

Answer: C

The cystic fibrosis transmembrane receptor (CFTR) is located on the apical membrane of the cholangiocyte, and with loss of function in the receptor there is decreased bile flow and inspissation of bile. This leads to an obstructive process, and eventually to bile plugs, bridging fibrosis, and obliteration of bile ducts. Fibrosis of the liver leads to the development of portal hypertension, which can cause esophageal varices, portosystemic shunts, and pulmonary complications such as portopulmonary hypertension or hepatopulmonary syndrome (1, 2).

Despite the loss of function of CFTR, only 10% of CF patients develop clinically significant liver disease (1). It is thought there is a second, and possibly third, hit that is required prior to progression. Exposure to a hepatotoxin, or presence of other genes damaging the liver in combination with loss of CFTR function leads to release of proinflammatory and fibrogenic mediators and activation of inflammatory cells, including portal fibroblasts, which lead to formation of peribiliary fibrosis.

History Continued:

The patient was taken to the endoscopy suite the next morning, and was intubated for an upper endoscopy. Three esophageal varices were banded, and subsequently the patient was noted to have frank hemoptysis from the endotracheal tube.

Answer: A

Ninety percent of hemoptysis originates from the bronchial artery, and the pulmonary artery accounts for 5% (3). Tuberculosis remains the leading cause of hemoptysis worldwide. Other conditions include bronchiectasis, especially in cystic fibrosis patients, and bronchitis.

The patient was taken from the endoscopy suite to the interventional radiology suite for a bronchial arteriogram, with the assumption that her hemoptysis was secondary to bronchiectasis. Bronchoscopy could have been considered for this patient, but given the non-focal computed tomography of the chest in the past and the belief that there would not be an endobronchial source for intervention it was not considered to be high yield.

Answer: D

Bronchial artery embolization (BAE) is considered the most effective non-surgical treatment in massive hemoptysis because of immediate and long-term results (3, 4). Selective angiography should be performed initially to locate the bleeding bronchial artery before the injection of particles (polyvinyl alcohol foam, isobutyl-2-cyanoacrylate, absorbable gelatin pledgets, or Gianturco steel coils) (4).

For our patient, the bronchial angiogram demonstrated evidence of peripheral arteriovenous shunts – findings that are suggestive of hepatopulmonary syndrome (Figure 1a) as opposed to bleeding from bronchiectasis. Hemoptysis is not a common finding for hepatopulmonary syndrome, but when it occurs it is thought that the vascular malformations are a result of the hypertensive portal system (5). The patient had cross-sectional imaging after her procedure, which verified that she had cirrhosis with portal hypertension as well as areas of arteriovenous shunting (Figure 1b). She also demonstrated evidence of hypoxia, with a PaO2 of 47 on 50% FiO2.

History Continued:

She was extubated two days post procedure, but required approximately a week of supplemental oxygen prior to being discharged home on room air. She did not require oxygen with exertion.

Figure 1

A (left): Right bronchial arteriogram demonstrating atypical dilatation of the peripheral arterial branches and significant arteriovenous shunting.

B (right): Axial CT slice of the chest demonstrating bilateral peripheral nodularities, consistent with pulmonary arterial branches with areas of arteriovenous shunting. Also noted is a relative lack of bronchiectasis typically seen in patients with cystic fibrosis.

The American Thoracic Society improves global health by advancing research, patient care, and public health in pulmonary disease, critical illness, and sleep disorders. Founded in 1905 to combat TB, the ATS has grown to tackle asthma, COPD, lung cancer, sepsis, acute respiratory distress, and sleep apnea, among other diseases.

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	A.	Agenesis of the liver Incorrect!
	B.	Dietary deficiency from lack of absorption of vitamins Incorrect!
	C.	Loss of function of transmembrane receptor on cholangiocyte Correct!
	D.	Medication side effect from CF medications Incorrect!

	A.	Bronchial artery Correct!
	B.	Pulmonary artery Incorrect!
	C.	GI tract Incorrect!
	D.	AV malformation Incorrect!

	A.	Lateral decubitus position Incorrect!
	B.	Endobronchial tamponade Incorrect!
	C.	Conservative management Incorrect!
	D.	Bronchial artery embolization Correct!

	A.	Bronchial wall thickening Incorrect!
	B.	Pronounced vascular dilatation of blood vessels Correct!
	C.	Pulmonary hypertension Incorrect!
	D.	Epithelial cell destruction Incorrect!

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