MKSAP Quiz: aching in joints of hands and knees
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A 28-year-old woman develops a 2-month history of aching in the joints in her hands and knees. Her fingertips turn white and become numb with exposure to cold. In addition, she has experienced postprandial and occasional burning nocturnal chest discomfort for the past 3 months. She does not have shortness of breath, exertional chest pain, or digital ulcers. Medical history is unremarkable, and family history is remarkable only for an aunt with systemic lupus erythematosus. She takes no medications.
On physical examination, pulse rate is 78/min, respiration rate is 18/min, and blood pressure is 100/60 mm Hg. There is skin thickening of the face, chest, abdomen, arms, hands, and feet. Lungs are clear to auscultation, and cardiac examination reveals a normal S1 and S2 with no gallops or murmurs. Abdominal examination is unremarkable with no organomegaly. There is no evidence of synovitis, but flexion contractures are present at the proximal interphalangeal joints and elbows. The wrists have reduced flexion. Tendon friction rubs are present around the ankles.
The complete blood count, basic metabolic profile, and urinalysis are normal. Antinuclear antibody assay is positive (titer of 1:640; speckled pattern), and an anti-double-stranded DNA antibody assay is negative.
In addition to a calcium-channel blocker trial, which of the following is the most appropriate management for this patient?
A. Prednisone
B. Omeprazole
C. Intravenous pulse cyclophosphamide
D. Azathioprine
MKSAP Answer and Critique
The correct answer is B) Omeprazole. This item is available online to MKSAP 14 subscribers in the Rheumatology section, Item 28.
Combination therapy with a proton-pump inhibitor, such as omeprazole, and a calcium-channel blocker is the most appropriate treatment in this clinical scenario. This patient has aggressive new-onset diffuse cutaneous systemic sclerosis with extensive skin involvement. Therapy for scleroderma involves systematic management of end-organ involvement. Therefore, therapy with a proton-pump inhibitor or, alternately, a histamine-2 receptor antagonist is indicated to treat this patient's symptoms of gastroesophageal reflux. In addition, a calcium-channel blocker is indicated to manage this patient's Raynaud's phenomenon.
Because scleroderma is not characterized by an inflammatory disorder, neither high-dose corticosteroids nor immunosuppressive agents are used in this setting. Moreover, even moderate-dose corticosteroid therapy may be associated with normotensive renal crisis in patients with scleroderma. In one study of 140 patients with “scleroderma renal crisis,” 11% of these patients had normal blood pressure but a rapid decline in renal function. These patients also were more likely to have microangiopathic hemolytic anemia and thrombocytopenia and were more likely to receive higher doses of prednisone during the 2 months immediately preceding renal crisis. Low-dose corticosteroids are indicated to treat inflammatory arthritis associated with scleroderma if first-line therapy is not effective. This patient has aggressive skin involvement, but no treatments for skin involvement in scleroderma are documented in clinical trials to be disease modifying. Therefore, cyclophosphamide and azathioprine would not be appropriate in this patient.
Key Point
- There is no treatment for scleroderma that is disease modifying.Therapy for scleroderma involves systematic management of end-organ involvement.In patients with scleroderma, high-dose corticosteroid therapy may be associated with normotensive renal crisis.