Disease frequency, patient characteristics, comorbidity outcomes and immunosuppressive therapy in systemic sclerosis and systemic sclerosis-associated interstitial lung disease: a US cohort study

Abstract

Objectives To investigate prevalence estimates and incidence rates (IRs) for SSc and SSc-associated interstitial lung disease (SSc-ILD) cohorts and describe patient characteristics, immunosuppressive therapy (IST) and comorbid outcomes among incident SSc and SSc-ILD cohorts. Methods Data were obtained from the US IBM MarketScan (2008–2017) claims database using algorithms developed with expert consultation. For the SSc cohort, newly diagnosed patients (aged ≥18 years) had one or more diagnostic claim for SSc. For the SSc-ILD cohort, patients had an additional ILD claim. Sensitivity analyses using two or more claims or alternative ILD diagnostic codes were also conducted. Results When requiring one or more diagnostic claim, the prevalence of SSc and SSc-ILD per 100 000 persons was 72.1 and 19.0. The IR for SSc and SSc-ILD per 100 000 person-years was 18.3 and 4.3. Sensitivity analyses requiring two or more claims yielded much lower prevalence (SSc: 41.5; SSc-ILD: 13.3) and IR (SSc: 8.8; SSc-ILD: 1.6) estimates. Patients with SSc-ILD were older, with increased comorbidities and diagnostic procedures at baseline. MTX and MMF were the most common ISTs; 12.7% of the SSc-ILD cohort received therapy at baseline vs 8.2% for SSc. A total of 42.5% and 45.0% of the SSc and SSc-ILD cohorts, respectively, started a stable IST regimen and 21.7% and 19.4% of these had an escalation. Skin disorders were the most common comorbid outcome in both cohorts during follow-up. Conclusions SSc, with or without associated ILD, is a rare disease in the US. Newly diagnosed patients with SSc-ILD had received more IST and had more comorbidities compared with newly diagnosed SSc.

Dyspnoea and cough in patients with systemic sclerosis–associated interstitial lung disease in the SENSCIS trial

“…Dyspnoea and cough are common in patients with SSc-ILD [3][4][5][6][7] and have a negative impact on patients' quality of life [4,[8][9][10]. Cough in patients with SSc-ILD may be due to the SSc-ILD itself [4][5][6], to gastroesophageal reflux disease (GERD) [11,12], to other comorbidities [13], or to medication use [14]. Similarly, dyspnoea is a common manifestation of ILD, but may also occur due to comorbidities such as pulmonary hypertension or cardiac involvement [15,16].…”

Detection and Monitoring of Interstitial Lung Disease in Patients with Systemic Sclerosis

“…In most patients, the first-line treatment for SSc-ILD is immunosuppression, and many patients with SSc-ILD will in any case be taking an immunomodulatory therapy to manage other manifestations of their SSc, such as skin fibrosis [ 31 ]. The most commonly used immunosuppressant therapy in patients with SSc-ILD is mycophenolate, which is better tolerated than cyclophosphamide and non-inferior to cyclophosphamide in its effects on lung function [ 32 ].…”

Systemic Sclerosis: Highlighting Respiratory Complications and Significance of Early Screening

“…They divided patients into limited and extensive disease categories using a combination of HRCT and PFTs [ 47 ]. Patients with SSc-ILD can also be divided into groups based on disease progression [ 48 ]. Patients with a slow, gradual reduction in forced vital capacity (FVC) or stability and those with a fast progressive clinical picture will require lung transplantation or progress to eventual death regardless of therapy [ 48 ].…”

“…Patients with SSc-ILD can also be divided into groups based on disease progression [ 48 ]. Patients with a slow, gradual reduction in forced vital capacity (FVC) or stability and those with a fast progressive clinical picture will require lung transplantation or progress to eventual death regardless of therapy [ 48 ]. The disease's clinical presentation is varied and requires a thorough clinical analysis [ 48 ].…”