Background/Purpose: Pemphigus vulgaris (PV) is a rare autoimmune disorder that affects the skin and mucous membranes. Many patients with PV suffer from serious infections, and though rare, mortality is associated with complications of treatment of PV, including corticosteroids. This study aimed to estimate the burden of illness in patients with PV in the US.

Methods: A retrospective cohort of patients with a new diagnosis of PV was identified from the Truven Health Marketscan US commercial claims database between January 1, 2009 and September 30, 2016. Newly diagnosed PV patients were defined by one inpatient diagnosis or two outpatient diagnoses of Pemphigus NOS/PV ((PV), ICD-9/10 694.4/L10.0)) during the study period and no PV diagnosis in prior 12 months. One-year healthcare resource use and costs were calculated, and the most prevalent comorbidities in the PV population are described.

Results: A cohort of 631 PV patients was identified. The mean age of the cohort was 50 (SD=11) years and 55% were female. During the baseline period, the most frequently reported comorbidities were opportunistic infections (62.8%), hypertension (23.3%), and type 2 diabetes (12.8%); 58.3% of patients were prescribed corticosteroids. During the first-year after diagnosis of PV, outpatient visits were the most frequently reported resource use (mean=19.5; SD=17.5); mean length of inpatient stays, for patients with hospitalization, was 12.0 (SD=21) and mean ER visits were 0.33 (SD=0.8). Mean total costs in the year prior to diagnosis were $16,420 (SD: $39,781), comprised of pharmacy ($2,500; SD=$4,993), inpatient ($5,423; SD=$27,618), and outpatient ($8,498; SD=$17,121). Mean total costs during first year after diagnosis were $30,742 (SD=$69,875). Total costs were comprised of pharmacy ($3,558; SD=8,858), inpatient ($5,623; SD=$18,108), and outpatient ($21,561; SD=$59,785). The mean cost [SD] of outpatient services during the first year after diagnosis was: durable medical equipment ($1,107 [$2,319]), evaluation and management ($2,105 [$2,386]), unclassified ($8,717 [$41,132]), imaging ($1,454 [$3,066]), other outpatient services ($14,207 [$48,573]), procedures ($4,713 [$17,383]), and tests ($1,590 [$2,604]).

Conclusion: Patients diagnosed with PV have high a comorbidity burden, particularly opportunistic infections. Healthcare costs almost doubled in the year following the diagnosis of PV compared to one year prior to diagnosis of PV.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/burden-of-illness-of-treating-patients-with-pemphigus-vulgaris/