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
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.

ADVERTISEMENT

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

ADVERTISEMENT

ADVERTISEMENT

ADVERTISEMENT

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

  • Why sleep is the missing pillar in modern health care

    Carlos Nunez, MD
  • How a family’s strength led to a successful kidney transplant

    C. Nicole Swiner, MD
  • Beyond the TikTok hype: Rebuilding trust in evidence-based weight loss medicine

    Sarah White, APRN
  • How deep transcranial magnetic stimulation is transforming mental health care

    Muhamad Aly Rifai, MD
  • Nurses aren’t eating their young — we’re starving the profession

    Adam J. Wickett, BSN, RN
  • What if medicine had an exit interview?

    Lynn McComas, DNP, ANP-C
  • Most Popular

  • Past Week

    • When did we start treating our lives like trauma?

      Maureen Gibbons, MD | Physician
    • When credibility is your only asset: the cautionary tale of DrKoop.com [PODCAST]

      The Podcast by KevinMD | Podcast
    • Medicalizing burnout misses the real problem

      Jessie Mahoney, MD | Physician
    • How robotics are reshaping the future of vascular procedures

      David Fischel | Conditions
    • Why gambling addiction is America’s next health crisis

      Safina Adatia, MD | Conditions
    • The man in seat 11A survived, but why don’t our patients?

      Dr. Vivek Podder | Physician
  • Past 6 Months

    • Why tracking cognitive load could save doctors and patients

      Hiba Fatima Hamid | Education
    • The silent toll of ICE raids on U.S. patient care

      Carlin Lockwood | Policy
    • What the world must learn from the life and death of Hind Rajab

      Saba Qaiser, RN | Conditions
    • Why recovery after illness demands dignity, not suspicion

      Trisza Leann Ray, DO | Physician
    • Physician patriots: the forgotten founders who lit the torch of liberty

      Muhamad Aly Rifai, MD | Physician
    • Addressing the physician shortage: How AI can help, not replace

      Amelia Mercado | Tech
  • Recent Posts

    • When credibility is your only asset: the cautionary tale of DrKoop.com [PODCAST]

      The Podcast by KevinMD | Podcast
    • Why sleep is the missing pillar in modern health care

      Carlos Nunez, MD | Conditions
    • How a family’s strength led to a successful kidney transplant

      C. Nicole Swiner, MD | Conditions
    • The food-drug interaction risks your doctor may be missing

      Frank Jumbe | Meds
    • Beyond the TikTok hype: Rebuilding trust in evidence-based weight loss medicine

      Sarah White, APRN | Conditions
    • The weaponization of rules: How regulatory overreach puts physicians and health care at risk

      Kayvan Haddadan, MD | Physician

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

ADVERTISEMENT

ADVERTISEMENT

ADVERTISEMENT

ADVERTISEMENT

  • Most Popular

  • Past Week

    • When did we start treating our lives like trauma?

      Maureen Gibbons, MD | Physician
    • When credibility is your only asset: the cautionary tale of DrKoop.com [PODCAST]

      The Podcast by KevinMD | Podcast
    • Medicalizing burnout misses the real problem

      Jessie Mahoney, MD | Physician
    • How robotics are reshaping the future of vascular procedures

      David Fischel | Conditions
    • Why gambling addiction is America’s next health crisis

      Safina Adatia, MD | Conditions
    • The man in seat 11A survived, but why don’t our patients?

      Dr. Vivek Podder | Physician
  • Past 6 Months

    • Why tracking cognitive load could save doctors and patients

      Hiba Fatima Hamid | Education
    • The silent toll of ICE raids on U.S. patient care

      Carlin Lockwood | Policy
    • What the world must learn from the life and death of Hind Rajab

      Saba Qaiser, RN | Conditions
    • Why recovery after illness demands dignity, not suspicion

      Trisza Leann Ray, DO | Physician
    • Physician patriots: the forgotten founders who lit the torch of liberty

      Muhamad Aly Rifai, MD | Physician
    • Addressing the physician shortage: How AI can help, not replace

      Amelia Mercado | Tech
  • Recent Posts

    • When credibility is your only asset: the cautionary tale of DrKoop.com [PODCAST]

      The Podcast by KevinMD | Podcast
    • Why sleep is the missing pillar in modern health care

      Carlos Nunez, MD | Conditions
    • How a family’s strength led to a successful kidney transplant

      C. Nicole Swiner, MD | Conditions
    • The food-drug interaction risks your doctor may be missing

      Frank Jumbe | Meds
    • Beyond the TikTok hype: Rebuilding trust in evidence-based weight loss medicine

      Sarah White, APRN | Conditions
    • The weaponization of rules: How regulatory overreach puts physicians and health care at risk

      Kayvan Haddadan, MD | Physician

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...