Skip to content
  • About
  • Contact
  • Contribute
  • Book
  • Careers
  • Podcast
  • Recommended
  • Speaking
  • All
  • Physician
  • Practice
  • Policy
  • Finance
  • Conditions
  • .edu
  • Patient
  • Meds
  • Tech
  • Social
  • Video
    • All
    • Physician
    • Practice
    • Policy
    • Finance
    • Conditions
    • .edu
    • Patient
    • Meds
    • Tech
    • Social
    • Video
    • About
    • Contact
    • Contribute
    • Book
    • Careers
    • Podcast
    • Recommended
    • Speaking

MKSAP: 38-year-old woman with fatigue and dyspnea on exertion

mksap
Conditions
July 13, 2013
4 Shares
Share
Tweet
Share

Test your medicine knowledge with the MKSAP challenge, in partnership with the American College of Physicians.

A 38-year-old woman is evaluated for a 6-month history of progressive fatigue and dyspnea on exertion. Over the past 2 years she has noted episodes of hand swelling, joint pain, blue color change of her fingers with cold exposure, and difficulty swallowing. She takes no medications.

On physical examination, temperature is 36.9 °C (98.4 °F), blood pressure is 126/84 mm Hg, pulse rate is 88/min, and respiration rate is 18/min. Cardiac examination reveals an accentuated pulmonic component of S2 and a grade 2/6 holosystolic murmur. Muscle strength is normal.

Laboratory studies reveal a serum creatine kinase level of 312 units/L, an antinuclear antibody titer of 1:1280 (speckled pattern), and high positive anti-U1-ribonucleoprotein antibodies.

Pulmonary function tests show a DLCO of 55% of predicted with normal FEV1 and lung volumes. Chest radiograph is normal. Electrocardiogram reveals right axis deviation. Echocardiogram shows 2+ tricuspid regurgitation, an enlarged right atrium, normal left and right ventricular function, and an estimated systolic pulmonary artery pressure of 40 mm Hg. High-resolution CT of the chest is normal. Ventilation/perfusion scan results are normal.

Which of the following is the most appropriate next step in management?

A. Muscle biopsy
B. Radionuclide stress test
C. Repeat echocardiography and pulmonary function tests in 1 year
D. Right heart catheterization

MKSAP Answer and Critique

The correct answer is D. Right heart catheterization. This item is available to MKSAP 16 subscribers as item 26 in the Rheumatology section.

Right heart catheterization is appropriate for this patient to evaluate for pulmonary arterial hypertension (PAH). She has symptoms and signs suggestive of an underlying diagnosis of mixed connective tissue disease (MCTD), which is characterized by overlapping clinical features of systemic lupus erythematosus, polymyositis, and systemic sclerosis, with high titers of antinuclear and anti-U1-ribonucleoprotein (RNP) antibodies. PAH may also occur, which is the most common disease-related cause of death in patients with MCTD.

This patient has Raynaud phenomenon, hand swelling, joint pain, dysphagia, high-titer speckled antinuclear antibodies, and high-titer RNP antibodies, all of which are typical findings of MCTD. This patient’s symptoms of dyspnea on exertion, isolated low DLCO, and elevated estimated systolic pulmonary artery pressure on echocardiogram all suggest PAH. Therefore, right heart catheterization is indicated.

Despite the patient’s mildly elevated serum creatine kinase level, muscle biopsy is not necessary, because her muscle strength is normal. Patients with MCTD may have subclinical myositis, but respiratory muscle weakness causing dyspnea would be a late finding associated with more profound myositis.

Radionuclide stress test is not indicated for this patient, whose abnormal DLCO and systolic pulmonary artery pressure on echocardiogram are not suggestive of ischemic cardiac disease as the cause for her dyspnea.

In patients with confirmed MCTD without cardiopulmonary symptoms and with normal baseline pulmonary function testing and echocardiogram, yearly repeat tests are appropriate for routine monitoring. It is an inappropriate choice for this symptomatic patient with findings highly suggestive of PAH.

Key Point

  • Pulmonary arterial hypertension is the most common disease-related cause of death in patients with mixed connective tissue disease.

This content is excerpted from MKSAP 16 with permission from the American College of Physicians (ACP). Use is restricted in the same manner as that defined in the MKSAP 16 Digital license agreement. This material should never be used as a substitute for clinical judgment and does not represent an official position of ACP. All content is licensed to KevinMD.com on an “AS IS” basis without any warranty of any nature. The publisher, ACP, shall not be liable for any damage or loss of any kind arising out of or resulting from use of content, regardless of whether such liability is based in tort, contract or otherwise.

Prev

MKSAP: 26-year-old man with fever and lower abdominal pain

July 13, 2013 Kevin 0
…
Next

Finding and keeping great front office staff

July 13, 2013 Kevin 4
…

Tagged as: Pulmonology

Post navigation

< Previous Post
MKSAP: 26-year-old man with fever and lower abdominal pain
Next Post >
Finding and keeping great front office staff

More by mksap

  • a desk with keyboard and ipad with the kevinmd logo

    MKSAP: 26-year-old man with back pain

    mksap
  • a desk with keyboard and ipad with the kevinmd logo

    MKSAP: 36-year-old man with abdominal cramping, diarrhea, malaise, and nausea

    mksap
  • a desk with keyboard and ipad with the kevinmd logo

    MKSAP: 52-year-old woman with osteoarthritis of the right hip

    mksap

More in Conditions

  • The surprising medical mystery of a “good” Hitler: How a rescued kitten revealed a rare movement disorder

    Teresella Gondolo, MD
  • The power of coaching for physicians: transforming thoughts, changing lives

    Kim Downey, PT
  • Unlocking the secrets of cancer conferences: an end-of-life counselor’s journey among pharmaceutical giants

    Althea Halchuck, EJD
  • An obstetrician-gynecologist reveals the truth about reproductive planning and how to navigate society’s expectations

    Yuliya Malayev, DO, MPH
  • Nose-brain connection: The surprising link between allergies and mental health revealed

    Kara Wada, MD
  • Is the rise in mental illness due to greater awareness or a true increase in incidence?

    Zahid Awan, MD
  • Most Popular

  • Past Week

    • How chronic illness and disability are portrayed in media and the importance of daily choices for improved quality of life

      Juliet Morgan and Meghan Jobson | Physician
    • Why affirmative action is crucial for health equity and social justice in medicine

      Katrina Gipson, MD, MPH | Policy
    • The power of coaching for physicians: transforming thoughts, changing lives

      Kim Downey, PT | Conditions
    • A patient’s perspective on the diminishing relationship between doctors and patients

      Michele Luckenbaugh | Conditions
    • Emulating Michael Jordan’s winning mindset: a path to success for health care professionals and entrepreneurs

      Harvey Castro, MD, MBA | Physician
    • How biased language and stigmatizing labels affect patient care and treatment

      Joan Naidorf, DO | Physician
  • Past 6 Months

    • The growing threat to transgender health care: implications for patients, providers, and trainees

      Carson Hartlage | Policy
    • It’s time to replace the 0 to 10 pain intensity scale with a better measure

      Mark Sullivan, MD and Jane Ballantyne, MD | Conditions
    • “Is your surgeon really skilled? The hidden threat to public safety in medicine.

      Gene Uzawa Dorio, MD | Physician
    • Breaking point: the 5 reasons American doctors are dreaming of walking away from medicine

      Amol Shrikhande, MD | Physician
    • Breaking the cycle of racism in health care: a call for anti-racist action

      Tomi Mitchell, MD | Policy
    • Revolutionize your practice: the value-based care model that reduces physician burnout

      Chandravadan Patel, MD | Physician
  • Recent Posts

    • From skydiving to saving lives: a surgeon’s journey on adversity, passion, and perseverance [PODCAST]

      The Podcast by KevinMD | Podcast
    • How can there be joy in medicine if there is no joy in Mudville?

      Arthur Lazarus, MD, MBA | Physician
    • Unveiling the intricate link between housing costs and health care

      Harvey Castro, MD, MBA | Policy
    • Physician entrepreneurs offer hope for burned out doctors

      Cindy Rubin, MD | Physician
    • Uncovering the truth about racial health inequities in America: a book review

      John Paul Mikhaiel, MD | Policy
    • Why electronic health records are failing patients: the dark side of copy and paste [PODCAST]

      The Podcast by KevinMD | Podcast

Subscribe to KevinMD and never miss a story!

Get free updates delivered free to your inbox.


Find jobs at
Careers by KevinMD.com

Search thousands of physician, PA, NP, and CRNA jobs now.

Learn more

View 5 Comments >

Founded in 2004 by Kevin Pho, MD, KevinMD.com is the web’s leading platform where physicians, advanced practitioners, nurses, medical students, and patients share their insight and tell their stories.

Social

  • Like on Facebook
  • Follow on Twitter
  • Connect on Linkedin
  • Subscribe on Youtube
  • Instagram

CME Spotlights

From MedPage Today

Latest News

  • 'Medically Relevant to Saving the Life of Your Patient': What We Heard This Week
  • Want to Solve the Nurse Shortage?
  • Why Are Female Doctors Sued Nearly Half as Often as Male Doctors?
  • What Drug Did FDA Just Approve for COVID?
  • PET Scan for Alzheimer's Dx; Predicting Colon Cancer Survival

Meeting Coverage

  • No Access to Routine Healthcare Biggest Barrier to HPV Vaccination
  • Trial Results Spark Talk of Curing More Metastatic Cervical Cancers
  • Cross-Border Collaboration Improves Survival in Pediatric Leukemia Patients
  • Monoclonal Antibody Reduced Need For Transfusions in Low-Risk MDS
  • Less-Invasive Surgery for Pancreatic Cancer Proves Safe, Effective
  • Most Popular

  • Past Week

    • How chronic illness and disability are portrayed in media and the importance of daily choices for improved quality of life

      Juliet Morgan and Meghan Jobson | Physician
    • Why affirmative action is crucial for health equity and social justice in medicine

      Katrina Gipson, MD, MPH | Policy
    • The power of coaching for physicians: transforming thoughts, changing lives

      Kim Downey, PT | Conditions
    • A patient’s perspective on the diminishing relationship between doctors and patients

      Michele Luckenbaugh | Conditions
    • Emulating Michael Jordan’s winning mindset: a path to success for health care professionals and entrepreneurs

      Harvey Castro, MD, MBA | Physician
    • How biased language and stigmatizing labels affect patient care and treatment

      Joan Naidorf, DO | Physician
  • Past 6 Months

    • The growing threat to transgender health care: implications for patients, providers, and trainees

      Carson Hartlage | Policy
    • It’s time to replace the 0 to 10 pain intensity scale with a better measure

      Mark Sullivan, MD and Jane Ballantyne, MD | Conditions
    • “Is your surgeon really skilled? The hidden threat to public safety in medicine.

      Gene Uzawa Dorio, MD | Physician
    • Breaking point: the 5 reasons American doctors are dreaming of walking away from medicine

      Amol Shrikhande, MD | Physician
    • Breaking the cycle of racism in health care: a call for anti-racist action

      Tomi Mitchell, MD | Policy
    • Revolutionize your practice: the value-based care model that reduces physician burnout

      Chandravadan Patel, MD | Physician
  • Recent Posts

    • From skydiving to saving lives: a surgeon’s journey on adversity, passion, and perseverance [PODCAST]

      The Podcast by KevinMD | Podcast
    • How can there be joy in medicine if there is no joy in Mudville?

      Arthur Lazarus, MD, MBA | Physician
    • Unveiling the intricate link between housing costs and health care

      Harvey Castro, MD, MBA | Policy
    • Physician entrepreneurs offer hope for burned out doctors

      Cindy Rubin, MD | Physician
    • Uncovering the truth about racial health inequities in America: a book review

      John Paul Mikhaiel, MD | Policy
    • Why electronic health records are failing patients: the dark side of copy and paste [PODCAST]

      The Podcast by KevinMD | Podcast

MedPage Today Professional

An Everyday Health Property Medpage Today
  • Terms of Use | Disclaimer
  • Privacy Policy
  • DMCA Policy
All Content © KevinMD, LLC
Site by Outthink Group

MKSAP: 38-year-old woman with fatigue and dyspnea on exertion
5 comments

Comments are moderated before they are published. Please read the comment policy.

Loading Comments...