A 60-year-old woman is being evaluated for headache and hypertension. For the past week, she has felt tired and has had a progressive, generalized headache. She denies fever, chills, photophobia, or neck stiffness. Her general health has been good with the exception of 6 months of persistent pains and swelling in both hands. During this time, her fingers felt puffy and she recently noted some tiny pits appearing on her finger tips. She is also troubled by heartburn and mild dyspnea with exertion. She does not smoke or drink alcohol. She takes calcium, vitamin D, and a multivitamin. Over the past few days, she has also been taking acetaminophen and ibuprofen.
Her blood pressure is 210/111 mm Hg, pulse is 90/min, and respirations are 16/min. She looks her stated age and appears uncomfortable. Her ocular fundi reveal no papilledema. Her neck is supple. Cardiac and abdominal exams are within normal limits. She has bibasilar fine end-inspiratory crackles on pulmonary exam. There are a few small digital fingertip pits.
Her laboratory studies are shown below.

Her chest-x ray shows bibasilar reticular fibrosis. Her peripheral blood smear reveals schistocytes.
Which of the following is the best initial treatment for this patient?

Question

A 60-year-old woman is being evaluated for headache and hypertension.  For the past week, she has felt tired and has had a progressive, generalized headache.  She denies fever, chills, photophobia, or neck stiffness.  Her general health has been good with the exception of 6 months of persistent pains and swelling in both hands.  During this time, her fingers felt puffy and she recently noted some tiny pits appearing on her finger tips.  She is also troubled by heartburn and mild dyspnea with exertion.  She does not smoke or drink alcohol.  She takes calcium, vitamin D, and a multivitamin.  Over the past few days, she has also been taking acetaminophen and ibuprofen.
Her blood pressure is 210/111 mm Hg, pulse is 90/min, and respirations are 16/min.  She looks her stated age and appears uncomfortable.  Her ocular fundi reveal no papilledema.  Her neck is supple.  Cardiac and abdominal exams are within normal limits.  She has bibasilar fine end-inspiratory crackles on pulmonary exam.  There are a few small digital fingertip pits.
Her laboratory studies are shown below.

Her chest-x ray shows bibasilar reticular fibrosis.  Her peripheral blood smear reveals schistocytes.
Which of the following is the best initial treatment for this patient?

Quizplus · Accepted Answer

This patient's presentation (joint pains, finger puffiness, digital tip pits, gastroesophageal reflux, and basilar interstitial lung disease) is most consistent with scleroderma. Severe and life-threatening renal disease develops in 10-20% of patients with the cutaneous form of scleroderma, known as scleroderma renal crisis (clinical features shown below). 11ec193a_98f4_56dd_853d_831e9d8f0cb6_MD0002_00 Intense renal vasoconstriction stimulates the release of renin and generation of angiotensin II, which worsens the hypertension and leads to progressive renal dysfunction. Angiotensin converting enzyme inhibitors can decrease progression of scleroderma renal failure, even in the presence of significant renal dysfunction (as in this patient). Captopril is preferred due to its rapid onset of action and extensive reported use in scleroderma renal crisis. The treatment should be continued even if renal function initially worsens, and some patients may need dialysis following long-term control of blood pressure with angiotensin converting enzyme inhibitors. (Choices B and C) Clonidine and Metoprolol are not first-line agents for hypertensive emergencies and do not prevent the effects of angiotensin II on renal vasoconstriction. (Choice D) Nifedipine can lower blood pressure quickly and may treat Raynaud's phenomena (seen in some scleroderma patients), but it is not effective for scleroderma renal crisis. (Choice E) Malignant hypertension and scleroderma renal crisis are often associated with microangiopathic hemolytic anemia and platelet destruction. In patients with TTP-HUS, marked thrombocytopenia and anemia are seen. This patient has severe hypertension, pulmonary fibrosis, and cutaneous findings which are not seen with TTP-HUS. As a result, there is no indication for plasmapheresis in this patient. Educational objective: A subset of patients with diffuse cutaneous systemic sclerosis develop scleroderma renal crisis, characterized by acute kidney injury (no active urinary sediment), accelerated hypertension, and microangiopathic hemolytic anemia. Treatment involves prompt initiation of an angiotensin converting enzyme inhibitor (even in patients with advanced renal dysfunction). __________ References: Scleroderma renal crisis: Patient characteristics and long-term outcome. (http://www.ncbi.nlm.nih.gov/pubmed/17601770) Outcome of renal crisis in systemic sclerosis: Relationship to availability of angiotensin converting enzyme inhibitors. (http://www.ncbi.nlm.nih.gov/pubmed/2382917) Systemic sclerosis sine scleroderma: demographic, clinical and serologic featured and survival in forty-eight patients. (http://www.ncbi.nlm.nih.gov/pubmed/10693887)

A 60-Year-Old Woman Is Being Evaluated for Headache and Hypertension