A 57-year-old woman comes to the physician because of severe heartburn. She has a history of gastroesophageal reflux disease treated with lansoprazole; however, lately the medication seems to provide little relief. She was also seen in the clinic one year ago for joint pains which responded to non-steroidal anti-inflammatory agents. She suffers from chronic back pain. She denies any abdominal pain, weight loss, or black stools. She has no family history of gastrointestinal cancer.
Her blood pressure is 110/65 mm Hg, pulse is 74/min, and respirations are 14/min. Examination shows clear lung fields. Extremity examination reveals skin hardening of the fingertips as well as a few whitish lumps measuring 1 cm each over both forearm surfaces. Small red blanching lesions are seen over her face.
Laboratory work-up shows the following:

Which one of the following disorders is the patient at greatest risk for developing?

Question

A 57-year-old woman comes to the physician because of severe heartburn.  She has a history of gastroesophageal reflux disease treated with lansoprazole; however, lately the medication seems to provide little relief.  She was also seen in the clinic one year ago for joint pains which responded to non-steroidal anti-inflammatory agents.  She suffers from chronic back pain.  She denies any abdominal pain, weight loss, or black stools.  She has no family history of gastrointestinal cancer.
Her blood pressure is 110/65 mm Hg, pulse is 74/min, and respirations are 14/min.  Examination shows clear lung fields.  Extremity examination reveals skin hardening of the fingertips as well as a few whitish lumps measuring 1 cm each over both forearm surfaces.  Small red blanching lesions are seen over her face.
Laboratory work-up shows the following:

Which one of the following disorders is the patient at greatest risk for developing?

Quizplus · Accepted Answer

Systemic sclerosis (SSc) is classified into limited and diffuse forms primarily based on the distribution of skin involvement. In limited cutaneous SSc (LcSSc), skin sclerosis is restricted mainly to the hands and distal forearm and, to a lesser extent, the face and neck. In the diffuse cutaneous form (DcSS), skin sclerosis extends to the chest, abdomen, and upper arms. This patient's presentation is consistent with CREST syndrome, a variant of the limited cutaneous form. These patients are anti-centromere antibody positive and carry a more favorable prognosis (in comparison to DcSS). They do not develop renal failure or the cardiac disease that can be seen with DcSS (Choices A & B); however, they are more prone to developing pulmonary hypertension, often without interstitial fibrosis. In contrast, patients with DcSS may develop severe forms of interstitial lung disease. CREST syndrome (Choice C) Cirrhosis is associated with hemochromatosis but not with SSc or the CREST variant. (Choice D) Patients with CREST syndrome do not carry a heightened risk for developing lymphoma, but they do have an increased risk for developing lung cancer. Educational objective: Patients with CREST syndrome are more likely to develop pulmonary hypertension (often without interstitial fibrosis) and less likely to develop the renal and cardiac disease seen in the diffuse cutaneous form of systemic sclerosis. __________ References: The pulmonary pathologic manifestations of the CREST syndrome. (http://www.ncbi.nlm.nih.gov/pubmed/2186993)

A 57-Year-Old Woman Comes to the Physician Because of Severe