WOW: OHSU Represents at ACP

OHSU residents (current and former) represent at National ACP in New Orleans last week. Congratulations to all of those who presented!

 Photo collage featuring Kimberly Chesteen, Kathy Wunderle, Elise Larson, Jessica Dreicer, Hayden Oldham, Meagan Herda (alumna), Jacob Nelson (alumnus), Jessica Morton (alumna) and Renee Honeyfield (alumna).  Photos courtesy of Avi O'Glasser and Kimberly Chesteen. 

Photo collage featuring Kimberly Chesteen, Kathy Wunderle, Elise Larson, Jessica Dreicer, Hayden Oldham, Meagan Herda (alumna), Jacob Nelson (alumnus), Jessica Morton (alumna) and Renee Honeyfield (alumna).

Photos courtesy of Avi O'Glasser and Kimberly Chesteen. 

Can you spot the esteemed former OHSU IM Program Director and longtime beloved VA faculty member?

Cardiac Amyloidosis

Thanks Dr. Ellis for presenting a case of cardiac amyloidosis. For those who missed it, the case was of a 70 y/o iron man participant who developed exercise-induced fatigue. His labs were unremarkable, and his EKG was notable for new TWIs in the anterolateral leads. Echo showed left atrial enlargement and RVSP of 41. His left heart cath was clean but cardiopulmonary exercise testing suggested chronotropic incompetence, leading to pacemaker placement. Despite multiple adjustments to the pacer, he continued to have symptoms and sought further counsel. In the end, cardiac MRI showed late gadolinium enhancement, and subsequent endomyocardial biopsy revealed cardiac amyloid.

Key points:

  • Classic presentation for cardiac amyloidosis is HFpEF
  • Key physical exam findings may include periorbital bruising and macroglossia
 Left: small periorbital bruise; Right: macroglossia  Images courtesy of JACC 2016 article entitled  AL (light-chain) Cardiac Amyloidosis: A Review of Diagnosis and Therapy

Left: small periorbital bruise; Right: macroglossia

Images courtesy of JACC 2016 article entitled AL (light-chain) Cardiac Amyloidosis: A Review of Diagnosis and Therapy

  • Approximately 50% of patients will have low voltage on ECG
  • Cardiac MRI shows late gadolinioum enhancement over the entire subendocardial circumference
 MR to aid in diagnosis of cardiac amyloid and estimate or amyloid burden.  Images courtesy of JACC 2016 article entitled  AL (light-chain) Cardiac Amyloidosis: A Review of Diagnosis and Therapy

MR to aid in diagnosis of cardiac amyloid and estimate or amyloid burden.

Images courtesy of JACC 2016 article entitled AL (light-chain) Cardiac Amyloidosis: A Review of Diagnosis and Therapy

  • Definitive diagnosis requires endomyocardial biopsy with Congo red staining but consider less invasive non-cardiac biopsy before obtaining endomyocardial biopsy

Acute Hepatitis B

Thanks to Dr. Xu for presenting a case of acute hepatitis B infection. This allowed for several clinical pearls and some review. First, Hepatitis B is decreasing in the USA. And in 2016, there were only 20 reported cases in Oregon. The decrease is likely as a result of vaccinations. Note, there is a new FDA approved two-dose HBV vaccination available called HEPLISAV-B (click here to read more about ). Remember that transmission is via blood and body fluid exposure. Therefore, make sure to ask about their practices and test partners/close contacts. For additional review of both clinical course and epidemiology, please refer to the CDC. A few other points from today:

1) Differential Diagnosis of Transaminases in the 1000s

  • ischemic: Budd Chiari (look for other evidence of end organ damage)
  • acute viral (HAV, HBV, etc)
  • drugs/toxins (often Tyelnol)
  • other less common etiologies include: exacerbation of autoimmune hepatitis and HELLP syndrome

2) Hepatitis B Typical Serologic Course

 Courtesy of the  CDC

Courtesy of the CDC

 Courtesy of the  CDC

Courtesy of the CDC

3) Treatment: in a non-cirrhotic patient with acute HBV infection, a 2017 Cochrane Review showed that there is poor quality evidence to suggest that treatment vs. no treatment decreased the rate of progression to chronic infection. Thus, expectant managment is fine in most immunocompetent adults. However, if treatment is chosen, it may be useful to avoid lamivudine which has a higher rate of progression to chronic HBV than did the groups treated either with entecavir or placebo. Of note, the exclusion criteria were liver diease, liver failure, drug/alcohol abuse and immunosuppression. So, the case discussed would have been excluded.

Idiopathic Interstitial Pneumonia

Thank you to Dr. Burnett who presented a complex pulmonary case in Intern Report today. The case allows for a refresher on idiopathic interstitial pneumonias and BAL results.

Idiopathic insterstitial pneumonias are a subset of diffuse instersitial lung disease with an inflammatory infiltrate in the interstitial compartment occasionally accompanied by fibrosis. Interstitial pneumonia can be idiopathic or associated with other conditions such as connective tissue disease, HIV, drugs or hypersensitivity. Idiopathic interstitial pneumonias include: idiopathic pulmonary fibrosis, idiopathic nonspecific insterstitial pneumonia (NSIP), respiratory bronchiolitis-interstitial lung disease, desquamative instersitial pnuemonia, cryptogenic organizing pneumonia and acute interstitial pneumonia (AIP). Interestingly, AIP is rapidly progressive and histologically indistinguishable from ARDS. Diagnosis can be aided by imaging such as CXR and HRCT. For information on the radiographic and pathologic findings, please refer to the article in Current Problems in Diagnostic Radiology. It is also useful to compare prior imaging to assess for rate of change. In this case, a BAL was also performed. And with a negative infectious and rheumatologic evaluation, the patient was diagnosed with idiopathic interstitial pneumonia and treated successfully with steroids.

For further reading on IIPs, ple

 American Thoracic Society guidelines on BAL interpretation in interstitial lung disease

American Thoracic Society guidelines on BAL interpretation in interstitial lung disease

WOW: SW Hope "Feed the Hungry" 5K

A special thanks to everyone who participated in the 3rd annual SW Hope "Feed the Hungry" 5K Race this past weekend coordinated in part by our very own Kristy Duggan!

The 2018 SW Hope 5K raised $20,278 which will purchase 81,112 pounds of food!! For all the race details, check out their website!

Shout out to: Volunteers: Cate Edgell, Michael Gardner Runners: Pat Liu (won 2nd place for women!), Hunter Spencer, Emilio Sulpizio, the entire O'Glasser family, and Brian Ricci!

And, special thanks to the many residents who donated to a great cause! You all are our Win of The Week!!

Amyloidosis and MM

Dr. Myers presented a case today of abdominal fullness and LE edema ultimately found to have endomyocardial biopsy proven AL amyloidosis and multiple myeloma. This case prompted further investigation into the cardiac considerations when contemplating therapy for multiple myeloma (MM). This topic is discussed in a 2016 article in the American College of Cardiology along with a highlight of new MM therapies that do not have a strong cardiac signal. They conclude that as MM treatments continue to evolve (chimeric antigen T-cell therapy, bromodomain inhibitors, etc) that the field also needs to improve managment of cardiovascular risks and prevention strategies especially in light of the increased cardiovascular risk in this cohort at baseline.

 Review of CV risk factors in patients with MM from a  2016 ACC  article. 

Review of CV risk factors in patients with MM from a 2016 ACC article. 

AIN after Combo Immune-Checkpoint Inhibitors

 A and B: urine sediment with granular cast (A) and WBC cast (B) suggestive of AIN. C: non-pruritis morbilliform erythematous rash all of which may be seen in a patient treated with nivolumab and ipilimumab for metastatic melanoma. Please refer to the article in  Clinic Kidney Journal  for more information. 

A and B: urine sediment with granular cast (A) and WBC cast (B) suggestive of AIN. C: non-pruritis morbilliform erythematous rash all of which may be seen in a patient treated with nivolumab and ipilimumab for metastatic melanoma. Please refer to the article in Clinic Kidney Journal for more information. 

Today's case highlights the ever changing frontier of medicine with the use of immunotherapies. Immune-checkpoint inhibitors are being used more and more frequently as anti-cancer therapy. And it has become common to look for the emergence of an autoimmune phenotype (the "-itis": hypophysitis, pneumonitis, colitis, hepatitis, etc). Dr. Oldham shared a case of acute interstitial nephritis (AIN) in the setting of combination immunotherapy with nivolumab and ipilimumab for metastatic melanoma.

Recall that nivolumab and pembrolizumab are anti-PD-1 therapies and, ipilimumab is an anti-CTA-4 therapy. On these therapies, immune-related adverse events occur in up to 60% of patients per reports. Often they are mild to moderate. However, with the utilization of combination immunotherapies, additional adverse events are being reported and at increasing rates. One recent study of nivolumab and ipilimumab combination therapy for melanoma in NJEM reported higher adverse events for dual therapy (55%) compared to either therapy alone (16.3% for nivolumab and 27.3% for ipilimumab). Notably however, severe renal impairment was rare. Yet, in the last two years there are several case reports of AIN associated with combination immunotherapies.

 Figure from  NJEM  article

Figure from NJEM article

One group from Brigham and Women speculates that this may be synergy between the two blocked pathways allowing for untethered cytotoxic effects.

While more information is necessary, this adds to the potential side effect profile of anti-cancer immunotherapies. And, it highlights the necessary vigilience we must have in order to properly diagnose and treat these individuals.

Do You Fancy Birds?

Dr. Duggan presented a case of "bird fancier's lung" which is a hypersensitvity pneumonitis. Common symptoms include: weight loss, fever, shortness of breath, dry cough, chest discomfort, anorexia, chills and extreme fatigue.

bird lung.PNG

It is diagnosed if there are 3 or more of the following:

  • known exposure
  • compatible exam, imaging and/or PFT findings
  • BAL with lymphocytosis
  • positive inhalation test
  • hisopathology demonstrating noncaseating granulomas

Once the diagnosis is made the treatement is: antigen removal and then more antigen removal and then maybe some steroids. Of note, steroids do not seem to alter long-term prognosis after review of a 1992 randomized, double-blind, placebo-controlled trial of 36 indviduals with Farmer's Lung published in the American Review of Respiratory Disease. Thus, steroids may be an adjunct to antigen removal if there are persistent symptoms or hypoxia. Additionally, this case is a reminder to ask about exposures- if we don't ask, we don't know!