What are the pulmonary recommendations for a patient with a history of scleroderma (systemic sclerosis), presenting with dysphagia, a negative swallow study, and a computed tomography (CT) scan of the chest showing an 8mm calcified nodule, subsegmental atelectasis, and subcarinal calcified nodules?

From the Guidelines

From a pulmonary standpoint, I strongly recommend close monitoring of the 8mm calcified nodule with follow-up CT imaging in 6-12 months to assess for any changes in size or characteristics, as the risk of malignancy, although low, cannot be entirely ruled out 1. For patients with scleroderma and dysphagia, even with a negative swallow study, I suggest pulmonary function tests (PFTs) including DLCO to establish baseline lung function, as interstitial lung disease is common in scleroderma 1. The subcarinal calcified nodules likely represent benign granulomatous disease from prior infection and typically don't require specific intervention. For the subsegmental atelectasis, deep breathing exercises and incentive spirometry 10 times hourly while awake can help prevent progression. Consider pulmonary rehabilitation if there are any functional limitations. Given the risk of pulmonary hypertension in scleroderma patients, an echocardiogram would be valuable to assess right heart function, as recommended by the updated EULAR guidelines for the treatment of systemic sclerosis 1. These recommendations are based on the increased risk of pulmonary complications in scleroderma patients, including interstitial lung disease, pulmonary hypertension, and aspiration pneumonia, even when swallow studies appear normal. Key considerations include:

• Monitoring for ILD progression with PFTs and HRCT chest, as conditionally recommended by the American College of Rheumatology and American College of Chest Physicians guideline 1
• Screening for ILD with HRCT chest and PFTs, given the low sensitivity of chest radiography and the complementary information provided by these tests 1
• The use of novel therapies, such as mycophenolate mofetil, nintedanib, rituximab, and tocilizumab, for the treatment of skin fibrosis and ILD, as recommended by the updated EULAR guidelines 1

From the FDA Drug Label

1. 1 Clinical Trials in Pulmonary Arterial Hypertension (PAH) Acute Hemodynamic Effects Acute intravenous infusions of epoprostenol for up to 15 minutes in patients with idiopathic or heritable PAH or PAH associated with scleroderma spectrum of diseases (PAH/SSD) produce dose-related increases in cardiac index (CI) and stroke volume (SV) and dose-related decreases in pulmonary vascular resistance (PVR), total pulmonary resistance (TPR), and mean systemic arterial pressure (SAPm).

The patient has a history of scleroderma and dysphasia, but the swallow study is negative. The CT chest shows an 8mm calcified nodule, subsegmental atelectasis, and subcarinal calcified nodules. From a pulmonary standpoint, the recommendations are not directly addressed in the provided drug label for a patient with this specific presentation. However, considering the patient's history of scleroderma, which is associated with PAH/SSD, and the presence of pulmonary nodules and atelectasis, it is essential to consider the potential for pulmonary arterial hypertension (PAH). The use of epoprostenol may be considered in patients with PAH/SSD, as it has been shown to improve hemodynamic parameters and exercise capacity in these patients 2. However, the decision to use epoprostenol should be made on a case-by-case basis, taking into account the patient's overall clinical presentation and medical history. It is crucial to consult with a pulmonologist or a specialist in PAH to determine the best course of treatment for this patient.

From the Research

Pulmonary Recommendations for Scleroderma Patient

• The patient's history of scleroderma and current symptoms, including dysphasia, require careful evaluation from a pulmonary standpoint 3, 4.
• The presence of an 8mm calcified nodule, subsegmental atelectasis, and subcarinal calcified nodules on the CT chest scan should be considered in the context of scleroderma-related interstitial lung disease (SSc-ILD) 5.
• Given the patient's swallow study is negative, the focus should be on managing potential pulmonary complications associated with scleroderma, such as pulmonary hypertension and interstitial lung disease 6, 7.
• The patient should undergo a comprehensive work-up, including:
  • Pulmonary function testing
  • High-resolution thoracic CT scanning
  • ECG
  • Echocardiography
  • Right-heart catheterization if pulmonary artery hypertension is suspected
• Immunotherapy, such as mycophenolate mofetil or cyclophosphamide, may be considered as first-line therapy for SSc-ILD, with the goal of slowing disease progression 5.
• The patient's diffusion capacity and forced vital capacity should be closely monitored, as significant declines in these values can indicate poor prognosis 7.
• Lung transplantation may be considered for selected patients with SSc-ILD, although careful assessment for co-morbidities is crucial 5.