Correct!
3. Systemic sclerosis
Hypertrophic osteoathropathy is one of a number of conditions that can affect the digits in the form of digital clubbing, but the description of the patient’s finger and hand findings is not consistent with this disorder. The yellow nail syndrome consists of the combination of yellow dystrophic nails, peripheral lymphedema and bronchiectasis with recurrent lower respiratory tract infections. This disorder is quite rare, and the physical examination findings are not consistent with this disorder. Furthermore, the basal bronchiectasis at thoracic CT in this patient is due to fibrotic disease (traction bronchiectasis), not intrinsic destruction of the airway walls resulting in bronchial dilation. Macrodactyly, or focal gigantism, represents enlargement of one or more digits, often the fingers, due to excessive growth of the underlying bone and soft tissue. This disorder is typically congenital in nature, often occurring in the context of neurofibromatosis, vascular malformations, multiple enchondromatosis, or even tuberous sclerosis, but does not fit with the patient’s physical examination findings. Finally, autoimmune thyroiditis may cause thyroid acropachy, which presents as digital swelling, clubbing, and periostitis, typically in the context of the ophthalmopathy and dermopathy associated with thyroid diseases- this patient does not have those stigmata and the physical examination findings do not fit with thyroid acropachy. However, the patient’s physical examination findings, thoracic CT findings, and antibody profile fit well with systemic sclerosis.
The patient was diagnosed with systemic sclerosis and pirfenidone was discontinued and mycophenolate mofetil and oxygen were started. Transthoracic echocardiography was performed, which showed normal left ventricular size and function and an estimated mean pulmonary arterial pressure of 43 mmHg with an estimated right ventricular systolic pressure of 63 mmHg. The right ventricular size and function were thought to be normal.
Which of the following statements represents the most appropriate next step for the managementof this patient? (Click on the correct answer to proceed to the eighth and final page)