Lung scarring and inflammation may be early signs of SSc: analysis | ILD First found in 4% to 6% of scleroderma patients in US database

Between 4% and 6% of people with scleroderma (SSc) were first determined to have interstitial lung disease (ILD), according to an analysis of a large US insurance claims database.

This finding supports the evidence for the early impact of scleroderma on lung disease and “highlights the importance of clinical examination and serological testing.” [blood] tests in patients with PID to rule out autoimmune disease [such as SSc] as the underlying cause,” the researchers wrote.

The study, “Understanding diagnostic pathways in systemic sclerosis and interstitial lung disease associated with systemic sclerosis: a retrospective cohort study,” was published in the journal Medicine.

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PID is a serious complication for patients with scleroderma

PID, a group of lung conditions characterized by inflammation and fibrosis (scarring) of tissue in and around the air sacs of the lungs, is a serious complication in people with SSc. PIDs are often detected after diagnosis of a connective tissue disease such as SSc, but they can also be an early manifestation of such diseases.

In some SSc patients, pulmonary manifestations precede non-lung related disease symptoms by months to, potentially, years, the study notes.

“Progression of SSc-ILD is associated with a significant increase in mortality,” noted the researchers, who included several investigators working for Boehringer Ingelheim Pharmaceuticals. “Early identification of SSc-ILD is important to allow patients to receive prompt treatment to slow progression and improve outcomes.”

To better understand the diagnostic pathway for ILDs in patients with scleroderma in the United States, the team analyzed data – spanning the period from January 2007 to June 2019 – from a large US drug claims database. insurance, Optum’s Clinformatics Data Mart database. It contains data, devoid of identifying information for analysis, from commercial claims and Medicare Advantage covering more than 60 million people in the United States.

Patients were divided into two groups, according to the version of the International Classification of Diseases, Clinical Modification, used as a diagnostic tool. Specifically, patients were diagnosed according to the ICD-9-CM (ninth revision, used until October 2015) or the ICD-10-CM group (the tenth and current revision).

In total, the ICD-9-CM group included 1779 adults and the ICD-10-CM group 1032 adults. In both groups, patients had two or more SSc-related medical claims at different dates within a year. In the ICD-9-CM group, patients had three or more years of continuous registration in the database prior to their first insurance claim due to SSc, called the index date. In the CIM-10-CM group, patients had at least two years of enrollment.

The existence of a PID diagnosis was defined as two or more medical claims associated with a PID on different dates. A pre-SSc ILD finding was determined through a second ILD request filed by a patient prior to a first, or index, SSc request.

Patients had a mean age of 60.8 years in the CIM-9-CM group and 65 years in the CIM-10-CM group at the time of their first SSc claim. In both groups, more than 80% of patients were female and approximately 65% ​​were Caucasian.

The researchers also studied the rate of ILD before a diagnosis of SSc using data from the anonymized US-based Cerner Health Facts database, collected from January 2000 to September 2018. It should be noted that data from the Cerner database are extracted from electronic medical records.

A total of 4,969 patients were identified in the Cerner database, of which 476 patients had three or more years of medical history prior to their first SSc-related encounter and were part of the Cerner ICD-9-CM group. They had an average age of 58.4 years when they were first diagnosed with SSc, 91.8% were female and 75% were Caucasian.

In the Optum database, a second ILD medical claim before the SSc index date was recorded for 136 patients in the ICD-9-CM group (7.6%) and 96 patients in the ICD-10-CM group (9.3 %). Additionally, 76 patients (4.3%) in the CIM-9-CM group and 58 patients (5.6%) in the CIM-10-CM group had their second medical claim for IPD more than a year before a first. SSc claim. In the Cerner ICD-9-CM group, this was observed in 16 patients (3.4%).

Based on the Optum database, no significant age differences were observed among patients who had ILD claims before or after SSc claims.

Lung disease specialists, general hospital providers, and radiologists were the most common healthcare professionals submitting a first or second claim for LTD. The most common procedures were X-rays (22.4%) and breathing capacity tests (15.8%).

Overall, “analyses of a large US health insurance database showed that 4% to 6% of patients with SSc had claims for ILD [over] 1 year prior to an SSc application,” the researchers wrote.

“These data provide further evidence that SSc can affect the lungs at an early stage of SSc and reinforce the importance of screening patients with SSc for PID and patients with PID for SSc,” they said. concluded.

Boehringer Ingelheim markets Ofev (nintedanib), an approved treatment for SSc-ILD, and the company funded this analysis.


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