Worsening Dyspnea in Scleroderma: What Gave It Away?

1Cleveland Clinic, Cleveland, OH
2Tulane University, New Orleans, LA

Meeting: Hospital Medicine 2011, May 10-13, Dallas, Texas.

Abstract number: 229

Case Presentation:

A 48‐year‐old man with Raynaud's and recently diagnosed CREST syndrome presented with 1 month of exercise intolerance and progressive shortness of breath, with no chest pain, peripheral edema, weight gain, cough, fever, or chills. He denied sick contact or recent travel. Subsequently, he began having episodes of abdominal pain with melena and dizziness but never passed out. He was on long‐term low‐dose prednisone and mycophenolate mofetil along with omeprazole for gastroesophageal reflux disease. His family history was positive for lupus in his father and colon cancer in his grandmother. Outpatient work up for interstitial lung disease, pulmonary hypertension, and heart failure was negative. This included a chest CT scan, pulmonary function tests (PFTS), and a 2‐D echocardiogram. He was afebrile, with normal vital signs. He had a normal cardiopulmonary examination but epigastric tenderness with no rebound. Admission hemoglobin was 7.8 g/dL, down from 14.5 g/dL at the time of CREST diagnosis 5 months prior. Iron studies revealed iron deficiency. He was transfused with 2 units of packed red blood cells and was started on an esomeprazole drip for suspected upper gastrointestinal bleed. The patient underwent esophagogastroduodenoscopy, which revealed a classic “watermelon” distribution of angioectasia in the stomach antrum, consistent with gastric antral vascular ectasia (GAVE). The associated ectasia was cauterized with argon plasma coagulation and later switched to oral esomeprazole. The patient's hemoglobin stabilized, and his symptoms of melena and dyspnea both improved before discharge.

GAVE after argon plasma coagulation (APC).

Discussion:

Scleroderma, specifically CREST syndrome, has gastrointestinal complications that can be easily missed and respond poorly to conventional treatment. This case demonstrates the recognized association between CREST syndrome and GAVE disease and illustrates an uncommon cause of dyspnea in the setting of limited systemic sclerosis. Those with diffuse cutaneous disease tend to develop GAVE sooner than those with limited and may need more therapeutic treatments such as argon plasma coagulation in combination with traditional immunosuppressants. Symptomatic vascular or gastrointestinal involvement of this disease is typically pigeonholed to Raynaud's or dysphagia, respectively, and associated dyspnea is often attributed to pulmonary or cardiac involvement. However, appreciating this association can lead to earlier recognition and treatment, as well as prevention of avoidable testing or complications, especially in those with normal PFTs and echocardiograms.

Conclusions:

The hospitalist has to recognize that dyspnea in patients with CREST syndrome is not always due to primary lung pathology. We describe a case where dyspnea was associated with gastrointestinal bleeding, which should be recognized as an association of scleroderma.

Disclosures:

M. C. Alraies ‐ none; M. Auron ‐ none; P. Parikh ‐ none; A. Hamid Alraiyes ‐none

To cite this abstract:

Alraies M, Auron M, Parikh P, Alraiyes A. Worsening Dyspnea in Scleroderma: What Gave It Away?. Abstract published at Hospital Medicine 2011, May 10-13, Dallas, Texas. Abstract 229. Journal of Hospital Medicine. 2011; 6 (suppl 2). https://www.shmabstracts.com/abstract/worsening-dyspnea-in-scleroderma-what-gave-it-away/. Accessed July 19, 2019.

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