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Medicare data indicated that most patients with rheumatologic conditions and living in areas where valley fever (aka coccidioidomycosis) is endemic were not screened for the disease before starting disease-modifying anti-rheumatic drugs (DMARDs).

Records from 2012-2016 for Medicare beneficiaries with rheumatologic diagnoses in Arizona, where valley fever is most common, showed that fewer than one quarter had a claim for Coccidioides serologic screening in the year prior to receiving a prescription for biologic or other targeted DMARDs, according to Wei-Hsuan Lo-Ciganic, PhD, of the University of Pittsburgh, and colleagues.

Screening rates were even lower in other states with high case rates, the researchers . In California, fewer than 3% of DMARD or corticosteroid users had been screened; the group didn't find a single patient screened in Texas.

The findings are important because valley fever is a fungal disease that could be substantially worsened among patients taking immunosuppressant drugs, including those with relatively selective activity such as biologic agents and Janus-associated kinase (JAK) inhibitors. These products come with boxed warnings about increased risk of serious fungal and other infections. While the drug labels don't specifically recommend prior screening except for tuberculosis, from the Infectious Diseases Society of America (IDSA) do suggest it.

On the other hand, guidelines from rheumatology groups make no mention of valley fever screening, and the IDSA guidelines neglects to provide recommendations for patients starting corticosteroids. Thus, Lo-Ciganic and colleagues wrote, it made sense to investigate how clinicians in endemic regions are approaching the issue.

First, some background on valley fever. In the U.S., ground zero is the southwest, with Arizona accounting for more than half of all cases in the country; most of the rest occur in California. It's also considered endemic in portions of Texas, but while valley fever is officially a reportable disease, Texas does not require notifications and hence include nothing from the state.

The most recent statistics, as of March 9, indicate that Arizona already has twice as many cases (3,308) as were seen at the same point in 2023 (1,579). If that rate continues, the full-year total could approach 18,000, which would be more than 60% above last year's count. Rates have been soaring nationally: in 2014, the first year for which the CDC has kept count, the national total was 8,109; at the end of 2023 it reached 19,845. Thus, at least in Arizona, the disease is something rheumatologists should take note of. Lo-Ciganic and colleagues cited a in Arizona indicating that screening 48 people before starting tumor necrosis factor inhibitors would prevent one case of valley fever -- and systemic coccidioidomycosis had severe consequences when it did develop.

For the current study, Lo-Ciganic and colleagues took Medicare's 5% samples for 2012-2016 and looked for people living in endemic areas with rheumatic autoimmune diseases, with claims indicating prescriptions for various DMARD classes (conventional, biologic, or targeted small-molecule) or corticosteroids. A total of 4,331 met these criteria. The researchers then searched for records of serologic screening for Coccidioides in the year prior to the first drug prescription.

About half of these patients had rheumatoid arthritis and another quarter had some form of spondyloarthritis. Diagnoses for the rest included lupus, inflammatory bowel disease, and psoriasis, among others. Endemic areas were selected on the basis of coccidioidomycosis rates of at least 25 per 10,000 beneficiaries in hospital referral regions.

Needless to say, screening rates were disappointingly low. In Arizona, about 20% given biologic or targeted therapies were screened; but only about 8% had screening prior to conventional DMARD treatment, and roughly the same proportion were screened before initiating steroid treatment. Corresponding rates in endemic areas of California were about 3%, 1%, and 1%. And in Texas, where the Odessa area was the only endemic region, the Medicare data showed not a single screen being conducted in the study population. Adjusting for comorbidities, disability, and demographic variables made little difference in the calculated rates.

Lo-Ciganic's group also examined Arizona's experience year by year, finding that screening prior to biologic/targeted therapy did increase over time: from about 16% in 2012 to 28% in 2016. The data also indicated that rheumatologists ordered screens at twice the rate of other providers. Both suggested that clinicians' awareness of the risk plays a role in screening.

What remains unclear is how often rheumatology patients should be screened, i.e., whether a single baseline screen is sufficient versus repeat screening on a regular schedule. Studies thus far haven't yielded enough data on outcomes to inform such decisions, the researchers noted. But at least, they concluded, "interventions intended to increase prescriber awareness of coccidioidomycosis risks and the utility of serologic screening [prior to starting immune-suppressing drugs] are warranted."

Limitations to the study included reliance on administrative data and the possibility that patients obtained screening without a Medicare claim (such as at local health departments). Also, the researchers counted intra-articular steroid injections in the analysis, which might carry less risk for promoting systemic fungal disease than oral versions.

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