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Scleroderma is a challenging disease to manage as it affects multiple organ systems. The hallmark of scleroderma
is clinical heterogeneity with subsets that vary in the degree of disease expressions, organ involvement, and prognosis.
The 2 cases to follow illustrate the diverse nature of the disease. Following the cases there is a competency based curriculum with
hyperlinks to images, and references that will help you answer questions relevant to the cases.
A 35 year old female presents with a 2 year history of Raynaud's phenomenon. She reports gastroesophageal reflux for a similar duration of time.
She also has noted that her skin is diffusely pruritic for the past year. Physical examination reveals a P 90 BP 160/90. HEENT is
remarkable for furrowing around the mouth and decreased oral aperture. Cardiac exam is normal. Pulmonary exam is normal. Skin reveals
sclerodactyly with digital pitting scars. There is mild diffuse skin tightening including the proximal upper and lower extremities, abdomen and chest.
What physical examination findings are concerning in this patient and why?
A 55 year old female with a 15 year history of Raynaud's phenomenon presents with increasing shortness of breath. She has noticed
tightening of the skin on her finger tips with intermittent digital ulcerations. She additionally reports hard bumps over the elbows
and red spots on her face and neck. Physical exam confirms the presence of sclerodactyly, digital pitting scars, calcinosis over the
elbows, and telangiectasias over the face and chest. Cardiac exam reveals a regular rate and rhythm and a loud P2 component of S2.
What is the diagnosis and what is a possible etiology for her shortness of breath? How would you evaluate it?
(Answer questions 1 – 5 on a piece a paper. Find Answer Key at the bottom on the page.)
1) Which is the next best step in the management of this patient?
2) Which of the following antibodies would be predictive of the development of hypertensive renal crisis in this patient?
The patient returns 1 month after the intial visit because her home BP readings have been high. She continues
to have active Raynauds phenomenon. On examination P 80 BP 160/90.
3) The next best step in the management would include:
4) Which of the following antibodies would be most likely present in this patient?
5 years after the intitial evaluation the patient presents with increasing dyspnea on exertion for the past 4 months.
Physical exam confirms stable findings of sclerodactyly, digital pitting scars, calcinosis over the elbows, and telangiectasias
over the face and chest. Pulmonary examination is clear to auscultation. Cardiac exam reveals a regular rate and rhythm and a
loud P2 component of S2. Pulmonary function tests reveal normal Lung volumes, and a DLCO of 40% of predicted.
5) The most likely cause for the dyspnea is?
1) The correct answer is E.
Home blood pressure is the correct answer and is a very important screening test in patients with early diffuse scleroderma to assure diagnosis of hypertension which could lead to hypertensive renal crisis. Cytoxan is used for treatment of active inflammatory pulmonary disease which has not been established in this patient. Right heart catheterization would be performed if there were additional signs and symptoms of pulmonary HTN including shortness of breath, Low DLCO on pulmonary function tests, or increased right sided pressures on echocardiogram.The patient denies shortness of breath, and has not yet had PFTs or echocardiogram. Bosentan is used for the treatment of pulmonary HTN which has not been established in this patient. Ankle MRI is not necessary, as a tendon friction rub is a clinical finding seen in patients with diffuse scleroderma, and does not need further evaluation.
2) The correct answer is E.
Anti RNA polymerase-3 is found in patients with diffuse scleroderm, and correlates with the development of hypertensive renal crisis and is the correct answer Anti centromere ab is see in the clinical subset of patients with with limited cutaneous disease as in this patient, Anti Jo1 is present in patients with antisynthetase syndrome a subset of the inflammatory myopathies Anti Mi-2 is found in a subset of patients with dermatomyositis. Anti SCL-70 is associated with diffuse scleroderma, and the presence of interstitial lung disease.
3) The correct answer is A.
This patient has hypertension in the setting diffuse scleroderma, and is at risk of developing renal crisis. Continue the Nifedipine and add Enalapril is the correct answer. Addition of the ACE inhibitor will help to prevent the development of hypertensive renal crisis, and is the drug category of choice. Discontinue the Nifedipine and add Verapamil would not protect the patient against hypertensive renal crisis. Continue the Nifedipine and add Atenolol would not protect the patient against hypertensive renal crisis. Discontinue Nifedipine and add CLonidine, again would not prevent renal crisis
4) The correct answer is A.
Anti centromere ab is seen in the clinical subset of patients with with limited cutaneous disease as in this patient, and is the correct answer Anti Jo1 is present in patients with antisynthetase syndrome a subset of the inflammatory myopathies. Anti Mi-2 is found in a subset of patients with dermatomyositis. Anti SCL-70 is associated with diffuse scleroderma, and the presence of interstitial lung disease. Anti RNA polymerase-3 is found in patients with diffuse scleroderma , and correlates with the development of hypertensive renal crisis.
5) The correct answer is D.
Pulmonary HTN is the correct answer, and is the likely diagnosis to explain dyspnea on exertion in a patient with long standing limited scleroderma. There are no physical exam features to suggest congestive heart failure, ie PND, LE edema, JVD, or rales Anemia can be seen in chronic disease, and can lead to dyspnes on exertion, but would not explain the abnormal P2 or DLcO. Interstitial lung disease would cause decreased lung volumes and a restrictive pattern on PFTs not seen in this patient.
Last updated February 2015.