August 2014 Pulmonary Case of the Month: A Physician's Job is Never Done

Elijah Poulos, MD^*

Kristine Saunders, MD^†

Pulmonary and Critical Care Medicine*

Department of Pathology^†

Phoenix VA Medical Center

Phoenix, AZ

History of Present Illness

A 75-year-old man presented with recurrent minimally productive cough, dyspnea, fatigue, low-grade fevers, and weight loss in November 2013. The patient had been treated twice as an outpatient with antibiotics in the previous 6 weeks for pneumonia.

PMH, FH, SH

The patient has a history of obstructive sleep apnea but is not compliant with his prescribed continuous positive airway pressure. He also as a history of obesity, dyslipidemia, and peripheral vascular disease.

There is no significant family history.  

He is a retired brick layer with a 50 pack-year smoking history but quit a few weeks prior to admission.  He drinks a case of beer/week.

Physical Examination

VS stable. There were no significant findings on physical examination.

Radiography

A chest radiograph (Figure 1) was performed.

Figure 1. Admission PA (Panel A) and lateral (Panel B) chest radiograph.

What should be done next? (Click on the correct answer to proceed to the next panel)

1. Bronchoscopy with bronchoalveolar lavage
2. Bronchoscopy with transbronchial biopsy
3. Needle biopsy
4. Thoracentesis
5. Video-assisted thorascopic surgery (VATS)

Reference as: Poulos E, Saunders K. August 2014 pulmonary case of the month: a physician's job is never done. Southwest J Pulm Crit Care. 2014;9(2):59-67. doi: http://dx.doi.org/10.13175/swjpcc098-14 PDF

July 2014 Pulmonary Case of the Month: Where Did It Come From?

Colin B. Fitterer, MD

James M. Parish, MD

Mayo Clinic Arizona

Scottsdale, AZ

History of Present Illness

A 67 year old man presented with worsening cough and shortness of breath. He has a history of metastatic colon cancer first diagnosed in 2010. He was treated with radiation and chemotherapy (FOLFOX) but unfortunately developed new pulmonary nodules in October, 2013 which were metastatic colon cancer on biopsy. In February 2014 he developed a right parietal brain mass which was resected. Thoracic CT scan at that time showed progression of the pulmonary nodules. He has also noted a 30 pound weight loss over the past 6 months and an enlarging right supraclavicular lymph node.

PMH, FH, SH

In addition to the colon cancer, he has previous diagnoses of type 2 diabetes mellitus, hypertension, allergic rhinitis, and vitamin D deficiency. He is married and a recently retired railroad engineer. He has no history of tobacco use. There is a positive family history of lung cancer but no colon cancer.

Physical Examination

Vital Signs:  Temperature 36.8, pulse 98, respirations 18, blood pressure 144/70, SpO2 91% on 3 L via nasal cannula.

Pertinent findings include:

• A large firm and fixed right supraclavicular lymph node that is nonpainful on palpation.
• Diminished breath sounds across all right posterior lung fields with dullness to percussion. 
• Palpable liver edge is palpable approximately 2cm below the right costal margin.

Laboratory Analysis

Admission laboratory values include a hemoglobin of 11.1 g/dL but with a normal white blood cell count and platelet count. Electrolytes, blood urea nitrogen, creatinine, and liver enzymes were all within normal limits.  Serum chemistries are within normal limits.

Radiography

A chest x-ray (Figure 1A) and chest CT (Figure 1B) were performed.

Figure 1. Admission AP (Panel A) and representative image from the thoracic CT scan (Panel B).

Which of the following is the best interpretation of the radiographic findings? (Click on the correct answer to proceed to the next panel)

1. Large right pleural effusion
2. Right lung atelectasis
3. Right lung pneumonia
4. Right lung pulmonary edema
5. None of the above

Reference as: Fitterer CB, Parish JM. July 2014 pulmonary case of the month: where did it come from? Southwest J Pulm Crit Care. 2014;8(6):1-7. doi: http://dx.doi.org/10.13175/swjpcc080-14 PDF

June 2014 Pulmonary Case of the Month: "Petrified"

Steven W. Purtle, MD

University of Colorado Hospital, Denver, CO

steven.purtle@ucdenver.edu

History of Present Illness

A 52 year old expatriated Iraqi man presents to pulmonary clinic with complaints of chronic dyspnea. While a young man living in Iraq, he had been disqualified from service in the Iraqi Air Force after a screening chest x-ray was found to be abnormal. He had no respiratory symptoms at the time of his disqualification, and he remained asymptomatic for the next twenty five years. Beginning five years ago, he had an insidious onset of breathlessness and exertional intolerance. Over the past several years, he has developed diffuse pleuritic chest pain, non-productive cough, and fatigue. He denies any fevers, chills, night sweats, arthralgias, rash, or visual symptoms. After moving to Denver, Colorado three years ago, he has developed a continuous oxygen requirement of two liters per minute.

PMH, FH, SH

He has no significant past medical or family history. While living in Iraq, he worked as a photographer, but he is currently unemployed. He is a lifelong non-smoker and uses no illicit drugs. He has never had any pets. He denies any exposure to inorganic dusts.

Medications

None

Physical Examination

Physical examination reveals a thin, middle-aged man in no acute distress. Vital signs were notable for an oxygen saturation of 90% on 2 liters per minute of supplemental oxygen. Pulmonary examination was notable for fine inspiratory crackles heard best at the bilateral bases. There was no clubbing or peripheral edema. The remainder of his physical examination was unremarkable.

Laboratory Analysis

Serum chemistries are within normal limits. Complete blood count shows a normal white blood cell count, hematocrit, and platelet count.

Pulmonary Function Tests

Pulmonary functions tests are shown in Figure 1.

Radiography

A chest x-ray (Figure 2) and chest CT (Figures 3 and 4) were performed.

Figure 2. Admission AP (Panel A) and lateral (Panel B) chest x-ray.

Figure 3. Static thoracic CT images displayed in lung windows (Panels A-D) and soft tissue windows (Panels E-H).

Figure 4. Movies of thoracic CT scan in lung windows (Panel A, top) and soft tissue windows (Panel B, bottom).

Which of the following features best describes the pattern seen on the patient’s chest CT? (Click on the correct answer to proceed to the next panel)

1. Diffuse microcalcifications
2. Honeycombing
3. Mosaicism
4. Patchy ground glass opacifications
5. Pleural plaques

Reference as: Purtle SW. June 2014 pulmonary case of the month: "petrified". Southwest J Pulm Crit Care. 2014;8(6):299-304. doi: http://dx.doi.org/10.13175/swjpcc069-14 PDF

May 2014 Pulmonary Case of the Month: Stress Relief

Robert W. Viggiano, MD

Department of Pulmonary Medicine

Mayo Clinic Arizona

Scottsdale, AZ

History of Present Illness

A 62 year old man was referred for an abnormal CT scan of the chest. He was found to have an abnormality in the lung as an incidental finding on a CT scan of the abdomen done 6 months earlier for abdominal pain. A CT-guided needle biopsy was performed but revealed only scant tissue and no diagnosis was made.

The patient was asymptomatic without dyspnea, wheezing or cough. He had no fevers, chills, history of pneumonia or sinus disease. He denied any symptoms of gastroesophageal reflux disease (GERD), regurgitation, dysphagia or aspiration.

PMH, FH, SH

The patient had a small melanoma excised from his arm several months earlier. Family history was noncontributory. He smoked a pack per day for 7 years but quit over 30 years earlier. He does not drink.

Medications

• Vitamins
• Mineral oil laxative

Physical Examination

Physical examination was unremarkable.

Radiography

A CT scan of the chest was performed (Figure 1).

Figure 1. Representative images from the thoracic CT scan. Panels A-E: lung windows. Panels F-J: Corresponding soft tissue windows.

The thoracic CT shows which of the following abnormalities? (Click on the correct answer to proceed to the next panel)

1. Left lower lobe mass
2. Mediastinal mass
3. Right middle lobe mass
4. 1 and 3
5. All of the above

Reference as: Viggiano RW. Pulmonary case of the month: stress relief. Southwest J Pulm Crit Care. 2014;8(5): . doi: http://dx.doi.org/10.13175/swjpcc046-14 PDF

Giant Cell Myocarditis: A Case Report and Review of the Literature

Nathan Spence, MD

Karen Niehaus, MD

Leonardo Macias, MD

Bart Cox, MD

University of New Mexico Hospital

Albuquerque, New Mexico, United States

Introduction 

First described by Saltykow in 1905 (1), Giant cell myocarditis (GCM) is a rare but highly lethal disease. Until the 1980s the diagnosis of GCM was determined at autopsy (2). It often affects young patients (mean age of 42.6 + 12.7 years),  and appears to occur in men and women equally. The occurrence of GCM in minority patients has not been previously described (3). The most common presenting symptom is heart failure (75%), though ventricular tachycardia (14%), chest pain with ECG findings of acute myocardial infarction (6%) and complete heart block (5%) may also occur. Treatment often involves an immunosuppressive regimen as a bridge to heart transplantation. The prevalence of GCM is known primarily from autopsy studies (i.e., 0.051% in India, 0.007% in England,  and 0.023% in Japan) (4-6). In the largest GCM observational study yet published, the rate of death or cardiac transplantation was 89 percent, with a median survival of 5.5 months from the onset of symptoms to the time of death or transplantation (3). Few cases with the successful treatment of GCM have been reported (7). Here we describe a case of GCM in a Hispanic female, the first to our knowledge, in which immediate diagnosis and initiation of an immunosuppressive regimen led to a favorable hospital course, whereby she was clinically stabilized and able to be transferred for transplant evaluation.

Case

A 56-year-old Hispanic female with a past medical history significant only for hypertension presented to our emergency department for evaluation of a non-productive cough over the last 3 days, which was associated with a headache, runny nose, myalgias, nausea, vomiting, chest pain, increased dyspnea on exertion, and lower extremity edema. On initial evaluation, heart rate was 86, blood pressure was 131/84, temperature was 37.4 degrees Celsius, oxygen saturation 96% on room air. Physical exam revealed a patient in moderate distress, a pericardial friction rub, clear lungs, and trace lower extremity edema. Laboratory testing revealed a leukocytosis of 12,800/mm³ (normal < 10,600/mm³), troponin I of 3.020 ng/mL (normal < 0.06 ng/mL), N-Terminal-Pro-BNP of 9,775 pg/mL (normal < 125 pg/mL), elevated ESR of 43 mm/hr (normal < 15mm/hr), elevated CRP of 3.6mg/dL (normal < 1 mg/dL), and a mild eosinophilia of 6% (normal < 5%).  Respiratory viral panel was negative. Chest X-Ray revealed a globular cardiac silhouette without definite evidence of congestion. Twelve-lead electrocardiogram revealed normal sinus rhythm with a new left bundle branch block. Cardiac catheterization revealed no significant coronary stenosis, with a left ventricular end diastolic pressure (LVEDP) of 22 mmHg. Upon admission to the floor, diuresis and titration of guideline based medications for dilated cardiomyopathy were begun, but were promptly discontinued due to development of hypotension. Transthoracic echocardiography (TTE) displayed severe hypokinesis of the basal inferoseptum and inferior wall of the left ventricle. Estimated ejection fraction was 35%, with mild to moderate mitral regurgitation. Blood pressure stabilized on day 2 of admission. Cardiac MR (CMR) with gadolinium was ordered, which did not show definite myocardial delayed enhancement (i.e., no evidence of infarction, myocarditis. See Figure 1).

Figure 1 Cardiac Magnetic Resonance Imaging. (A) Two chamber delayed post-gadolinium inversion recovery view. (B) Two chamber delayed post-gadolinium phase sensitive inversion recovery view.

On day 3 of hospitalization, the patient suddenly developed complete heart block, became hypotensive and confused. As a result, a temporary venous pacemaker (TVP) was placed, and endomyocardial biopsy (EMB) was performed. Five specimens were obtained. Pulmonary capillary wedge pressure was measured at 32 mmHg, with a Fick cardiac output 3.01 L/min and cardiac index of 1.77 L/min/m². Due to persistent hypotension in the cath lab, a dopamine drip was begun, a Swan-Ganz catheter was placed, and the patient was transferred to the Medical Intensive Care Unit for further hemodynamic monitoring and treatment. That afternoon, GCM was diagnosed by pathology (see Figure 2).

Figure 2 Pathology. (A) Infiltration of cardiac muscle tissue by an inflammatory infiltrate. (B) Massive myocyte necrosis with giant cells among the inflammatory infiltrate. (C) Rare eosinophils are seen among the inflammatory infiltrate. (D) Giant cell among the inflammatory infiltrate.

An immunosuppressive regimen of corticosteroids, azathioprine,  and cyclosporine was promptly initiated. Thereafter, over the course of 3 days, clinical symptoms  and hemodynamics improved significantly. TVP was removed, inotropic support was weaned off, and ACE inhibitor and diuretics were titrated. Beta-blockers were withheld out of concern for recent complete heart block and use of inotropic support. On hospital day 10, the patient was transferred, in stable condition, to be evaluated for heart transplantation, and/or mechanical circulatory support. At the outside center, she had an ICD placed for primary prevention, was maintained on the three drug immunosuppression regimen, continued to do well clinically, and was listed for transplant.

Discussion

Several autoimmune disorders have been associated with GCM, which include inflammatory bowel disease, thyroiditis, and thymoma (8). Our patient did not have a history of autoimmune disease, and the laboratory tests to detect such during her hospitalization were negative. Evidence suggests that GCM is an autoimmune disorder dependent on CD4-positive T lymphocytes and anti-myosin autoantibodies (8). Early diagnosis leading to appropriate treatment, as in our case, appears to be imperative for a favorable clinical outcome. Treatment with a combination of immunosuppressives has been shown to improve survival, compared with those not receiving immunosuppressive regimens (12.3 months vs. 3.0 months, p=0.001) (3). If patients live long enough to receive heart transplantation, longer term survival is possible. For that reason, it is a Class I (level of evidence B) guideline recommendation to perform EMB in the setting of unexplained, new-onset heart failure of < 2 weeks' duration associated with a normal sized or dilated left ventricle in addition to hemodynamic compromise (9). The sensitivity of EMB for GCM is 80% to 85% in subjects who subsequently die or undergo heart transplantation (2). Therefore, in the appropriate clinical setting, EMB may drastically alter treatment and provide important prognostic information. The pathological hallmark of GCM is the presence of multinucleated giant cells and a lymphocytic inflammatory infiltrate, associated with myocyte necrosis (10-12). CMR may display delayed myocardial enhancement to support the diagnosis of myocarditis, though a non-diagnostic study, as in our case, does not rule it out.   

We encountered, to our knowledge, the first case of GCM in a patient of Hispanic ethnicity, who presented with the classic associated symptoms of heart failure, hemodynamic collapse, and complete heart block, and whose clinical course was favorably improved by early diagnosis and initiation of an immunosuppressive regimen. CMR did not identify myocarditis. However, this case illustrates the importance of including GCM in the differential diagnosis when a patient presents with suggestive clinical features and is not responding to current evidence based treatment for acute decompensated heart failure.

References

1. Saltykow S. Uber Diffuse Myokarditis. Virchows Archiv fur Pathologische Anatomie. 1905;182:1-39. [CrossRef]
2. Shields RC, Tazelaar HD, Berry GJ, Cooper LT. The role of right ventricular endomyocardial biopsy for idiopathic giant cell myocarditis. J Card Fail. 2002;8:74-88. [CrossRef] [PubMed] 
3. Cooper LT, Berry GJ, Shabetai R. Idiopathic giant-cell myocarditis - natural history and treatment. Multicenter Giant Cell Myocarditis Study Group Investigators. N Engl J Med. 1997;336:1860-6.[CrossRef] [PubMed]
4. Vaideeswar P, Cooper L. Giant cell myocarditis: clinical and pathological disease characteristics in an indian population. Cardiovasc Pathol. 2013;22:70-4. [CrossRef] [PubMed]  
5. Whitehead R. Isolated myocarditis. Brit Heart J 1965;27:220-30. [CrossRef] [PubMed]
6. Okada R, Wakafuji S. Myocarditis in autopsy. Heart Vessels 1985; Suppl 1:23-9. [CrossRef]
7. Desjardins V, Pelletier G, Leung TK, Waters D. Successful treatment of severe heart failure caused by idiopathic giant cell myocarditis. Can J Cardiol. 1992;8:788-92. [PubMed] 
8. Cooper L, ElAmm C. Giant Cell Myocarditis: Diagnosis and treatment. Herz. 2012;37:632-6. [CrossRef] [PubMed] 
9. Cooper LT, Baughman KL, Feldman AM, et al. The role of endomyocardial biopsy in the management of cardiovascular disease. A scientific statement from the American heart association, the American college of cardiology, and the European society of cardiology. J Am Coll Cardiol. 2007;50(19):1914-31. [CrossRef] [PubMed] 
10. Davies M, Pomerance A, Teare R. Idiopathic giant cell myocarditis - a distinctive clinico-pathological entity. Br Heart J. 1975;37:192-5. [CrossRef] [PubMed] 
11. Davidoff R, Palacios I, Southern J, Fallon JT, Newell J, Dec GW. Giant cell versus lymphocytic myocarditis. A comparison of their clinical features and long-term outcomes. Circulation. 1991;83:953-61. [CrossRef] [PubMed] 
12. Ren H, Poston RS Jr, Hruban RH, Baumgartner WA, Baughman KL, Hutchins GM. Long survival with giant cell myocarditis. Mod Pathol. 1993;6:402-7. [PubMed] 

Reference as: Spence N, Niehaus K, Macias L, Cox B. Giant cell myocarditis: a case report and review of the literature. Southwest J Pulm Crit Care. 2014;8(4):247-51. doi: http://dx.doi.org/10.13175/swjpcc052-14 PDF