The connective tissue diseases (CTD) are frequently associated with Interestitial lung diseases (ILD). The
presence of an ILD in a patient with CTD is associated with a higher morbidity and mortality. Management of a patient
with connective tissue interstitial lung disease (CTD-ILD) depends on disease severity with a focus on improving quality
of life. Therapeutic interventions include medications, which aim to prevent progression to fibrosis and decrease
inflammation or alveolitis, supplemental oxygen; and pulmonary rehabilitation. Comorbidities including gastroesophageal
reflux disease (GERD) and obstructive sleep apnea should be addressed in all CTD-ILD patients. Appropriate
referral to support groups, lung transplantation centers, and palliative care are considerations for all patients with CTDILD.
The management of a patient with CTD-ILD is a multidisciplinary personalized process that is driven by the specific
disease, clinical pattern, and associated comorbidities.
Keywords: Collagen vascular disease, connective tissue disease, immunosuppressive therapy, interstitial lung disease, oxygen,
pulmonary rehabilitation, supportive care.
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