The guideline presents initial treatment options, with mycophenolate, tocilizumab and rituximab in terms of first-line ILD therapy expected to be the most prescribed for systemic sclerosis, but with cyclophosphamide, nintedanib or azathioprine as additional options that may make sense in specific situations. Some slight differences exist in the recommendations for therapeutic treatments for different subtypes.
Dr. Johnson pointed out the evidence used to recommend one treatment over another was of very low certainty. Comorbidities, extra-pulmonary disease activity, patient preferences or other factors may lead clinicians to make different choices.
Dr. Khanna highlighted one key difference in treatment approaches among different SARDs: Short-term glucocorticoids are conditionally recommended as part of treatment for other types of SARDs, but for systemic sclerosis, the strong recommendation is against their use. This is due to risks of triggering renal crisis in this condition and lack of data demonstrating their efficacy in treating systemic sclerosis-related ILD.
Clinical Question: How often should this patient be rescreened?
Per the guideline, the conditional recommendation is that this patient should be screened again with pulmonary function testing within another three to six months, with less frequent screenings once the disease is stable, and with ambulatory desaturation testing every three to 12 months. High resolution CT can also be used if needed, e.g., to distinguish worsening symptoms from other possible etiologies.
Dr. Johnson underscored that ambulatory desaturation testing is not synonymous with the 6-minute-walk test. Rheumatologists may not be comfortable with the former test themselves, but they can collaborate with pulmonologists, who should become contributors to patient care if someone is diagnosed with an ILD.
Clinical Case Continued
The patient was prescribed mycophenolate mofetil at 500 mg twice a day, increasing to 3,000 mg/day over six weeks. The patient’s fatigue and pleuritis had improved at six-month follow-up, with a minimal improvement in her Rodnan skin score. She had developed minimal dyspnea on exertion and mild cough. Her pulmonary function tests had all dropped slightly.
By the time of another follow-up three months later, her pulmonary function tests had dropped more (e.g., forced vital capacity had fallen to 2.34 L from 2.62 L at pre-treatment; diffusion capacity had dropped to 65% from 73% pretreatment). She continued to display high inflammatory markers.
Clinical Question: Is a treatment change indicated? What approach should be used?
Dr. Khanna emphasized how important it is to follow these patients closely. He explained that this patient was clearly displaying a progression in her ILD, which in this case he confirmed via high-resolution chest CT, and she needed further treatment.
