Abstract Introduction Interstitial lung disease (ILD) is the most common cause of death in patients with systemic sclerosis (SSc), accounting for at least 30% of deaths. Pulmonary involvement develops with similar pathophysiology to dermal manifestations, beginning with inflammatory changes that progress to fibrosis and vascular injury. Early treatment can improve outcomes and prevent progression of fibrosis, however, this can be delayed in cases where infection may confound the picture and preclude the use of immunosuppressive therapy. This case describes a case of rapidly progressive ILD in the setting of a new diagnosis of systemic sclerosis. Case presentation A 41-year-old man with a history of osteoarthritis presented with progressive dyspnea and pleuritic chest pain for one month. Associated symptoms included depigmentation and unintentional weight loss. High-resolution CT revealed nonspecific chronic ground glass changes and bilateral interstitial opacities. He remained without signs of infection. Autoimmune pathology was suspected. Pulmonary function tests revealed a restrictive pattern. Bronchial lavage revealed an elevated white count, a nonspecific finding. He developed rapidly progressing respiratory failure requiring multiple hospitalizations and the need for near continuous BiPAP. Labs revealed a positive anti-topoisomerase antibody (Scl70). He was diagnosed with systemic scleroderma. He developed worsening esophageal dysmotility due to the progression of his scleroderma, resulting in chronic aspiration pneumonitis, further worsening his respiratory status. His infectious workup was notable for an indeterminate fungitell. As a result of his rapidly worsening clinical picture and quality of life, a multidisciplinary decision between patient and his specialists was made to begin steroids and rituximab despite the risk of underlying fungal infection to slow his disease progression. Medications were transitioned to mycophenolate, however he was ultimately lost to follow up. Discussion This case highlights the diagnostic and therapeutic challenges for SSc-ILD, particularly when infectious workup is indeterminate. ILD occurs in 80% of SSc patients, and approximately 25% can progress to clinically debilitating disease. Identification of disease specific antibodies associated with severe manifestations of systemic sclerosis, such as anti-topoisomerase I (Scl-70), is crucial as it is associated with a higher risk of rapidly progressive ILD. Timely initiation of treatment with immunosuppressive therapy has been shown to slow pulmonary function decline and improve survival. Due to the use of immunosuppressive medications, overlapping infectious concerns can delay treatment and remain a barrier to optimal outcomes. Given the significant morbidity and mortality associated with SSc-ILD, early detection be life saving, and improve quality of life outcomes. This abstract is funded by: None
Bacani et al. (Fri,) studied this question.