A 34-year-old woman complains bitterly of heartburn. Physical examination reveals healing lesions of the fingertips that she says were small ulcers, and there are small areas of telangiectasias on her face. Esophageal manometry reveals a decrease in the expected amplitude of smooth muscle contraction. Lower esophageal sphincter tone is subnormal, but relaxes normally with swallowing. Which of the following
statements regarding this condition is most likely correct?
(A) characterized by systemic signs of inflammation
(B) predominantly treated symptomatically
© characterized by a poor prognosis
(D) usually more frequent in men
(E) characterized by death secondary to a renal crisis
solution:
These findings are characteristic of scleroderma. If the disease is limited, the prognosis is not necessarily poor. The limited form is characterized by calcinosis, Raynaud’s (often with distal ulceration), esophageal motility disorder, sclerodactyly, and telangiectasia. It has a female preponderance. Renal disease can be severe, but is not the most common cause of death. Esophageal symptoms should be treated aggressively.