Healthcare and economic burden of ANCA-associated vasculitis in Italy: an integrated analysis from clinical and administrative databases

Abstract

ANCA-associated vasculitides (AAV) comprise a group of systemic vasculitides characterized by inflammation of small-sized blood vessels leading to multi-organ involvement. The worldwide annual incidence of AAV ranges from 1.2 to 3.3 cases per 100 000 individuals with a prevalence of 4.6-42.1 cases per 100 000 individuals. The prevalence of AAV is geographically heterogeneous; therefore, regional epidemiological studies can be more informative to improve health care systems. Even though clinicians are aware that the healthcare burden and the risk of hospitalization of AAV appear high, data on hospitalization and cost of illness due to AAV are still scarce or even lacking. This study aims to characterize the economic burden of AAV in Friuli Venezia Giulia (FVG), Italy. Thus, a retrospective study was conducted through the integration of many administrative health databases of the FVG as the source of information. From data integration, we estimated that more than two-thirds of AAV patients showed at least one hospitalization in their medical history, most frequently caused by the disease itself or superimposed infections. Around 10% of patients developed end-stage renal disease. In an 8-year follow-up, the overall healthcare cost was € 1,215,078, corresponding to € 6,168 patient-year. ANCA-positive patients showed much higher costs than ANCA-negative patients did. Overall, AAV are rare diseases, but imply very high healthcare costs. Early diagnosis and optimal treatment probably still remain unmet needs for AAV.

Keywords: ANCA; Autoantibody; Cost; Granulomatosis; Healthcare; Hospitalization; Polyangiitis; Vasculitis.

Fig. 1

Distribution of the categories of main discharge diagnosis for 285 hospitalizations of patients with AAV in FVG (years 2013–2018). the following ICD-9-CM codes were considered “disease related”: 287.0 “Allergic purpura”, 288.3 “Eosinophilia”, 381.4 “Nonsuppurative otitis media, not specified as acute or chronic”, 423.0 “Hemopericardium “, 437.4 “Cerebral arteritis”, 446.4 “Wegener's granulomatosis”, 447.6 “Arteritis, unspecified”, 473.0 “Chronic maxillary sinusitis”, 473.2 “Chronic ethmoidal sinusitis”, 518.3 “Pulmonary eosinophilia”, 580.4 “Acute glomerulonephritis with lesion of rapidly progressive glomerulonephritis”, 582.4 “Chronic nephritis with lesion of necrotizing glomerulitis”, 582.9 “Chronic glomerulonephritis with unspecified pathological lesion in kidney”, 584.5 “Renal failure with (acute) tubular necrosis”, 375.53 “Stenosis of lacrimal canaliculi”, 375.56 “Stenosis of nasolacrimal duct, acquired”, 381.10 “Chronic serous otitis media, simple or unspecified”, 420.90 “Acute pericarditis, unspecified”, 446.29 “Other specified hypersensitivity angiitis”, 478.74 “Stenosis of larynx”, 519.19 “Unspecified disease of respiratory system”, 582.89 “Chronic glomerulonephritis with lesion of: exudative nephritis”

Fig. 2

The overall estimated healthcare costs (€) per person-year for 57 AAV patients, residents in the province of Udine from 2010 to 2018, divided into ANCA-positive and ANCA-negative

Fig. 3

The overall estimated healthcare costs (€) per person-year for 57 AAV patients, residents in the province of Udine from 2010 to 2018, also divided into each entity (GPA, MPA, and EGPA)

Fig. 4

Numbers of events in the 58 AAV patients followed by the Rheumatology Clinic of Udine (2010–2018). The patients have been divided into three categories: patients persistently ANCA-positive, patients becoming ANCA-negative, and patients ANCA-negative