gms | German Medical Science

Background: ANCA-associated vasculitis (AAV) is a severe systemic small vessel vasculitis and diagnosis can be difficult and prolonged. Referral pathways are complicated with potentially several different specialists involved and patients are concerned about their path to treatment. Patient comorbidity is important and therapy for AAV will have an important additional impact. This retrospective study examined referral, diagnosis, and therapy outcomes in AAV patients managed in routine German clinical practice.

Methods: A retrospective study was performed on 10 German AAV patients (19,0% relapsing) receiving care from 10 physicians (including 5 Rheumatologists). Patients commenced remission induction therapy between 2014-17 and data was collected from baseline and 1, 3, 6 and 12 months following induction therapy.

Results: Mean patient age was 53,84 years (21,0% of patients over 65 years) and 44,7% female and 55,3% male. 76% of patients were referred by other physicians. 54% were referred direct from primary care and only 22% were referred with the AAV diagnosis already made. Referral symptoms were general in most cases – fatigue (53%), fever (51,7%), musculoskeletal pain (51,7%), joint pain (46,7%), weight loss (44,7%), and nasal symptoms (35,3%) – 55,3% had renal disease. Physicians reported that 18.5% of patients had had AAV symptoms for over 3 months. Comorbidities were common (hypertension 47%, diabetes 17,7%, COPD/asthma 11,7%, coronary arterial disease 13,7%, arthritis 9,0%, osteoporosis 4,3% and peptic ulceration 3%) with only 29,3% having none. At diagnosis, the median eGFR was 43,1 ml/min, median urinary protein excretion 795 mg/24 hours and 64,7% had microscopic haematuria although it was not recorded in 13,7%. Granulomatosis with polyangiitis was diagnosed in 51,7% of patients. In only 14,7% of patients was the Birmingham Vasculitis Activity Scale (BVAS) used to assess activity. Clinically, 16% had mild localized disease, 54% had moderate/systemic disease and 30% had severe/rapidly progressive disease. Resource use at diagnosis and treatment was significant, although 38.7% were managed as out patients, those with in patient care had mean length of stay of 15.8 days including 2.2 intensive care unit days. Only a minority of patients were managed by individual physicians and 73,7% of patients were managed in collaboration with other clinical teams (Nephrologist 34%, Rheumatologist 23%, Internal Medicine 18%, Respiratory 10%, ENT 9,71%, Intensivist 8%).

Conclusion: AAV patients have complex pathways to diagnosis and have general symptoms sometimes of long duration. Most patients have comorbidities which need to be considered during AAV therapy. BVAS is used rarely in clinical practice and resource utilization at diagnosis and treatment is significant.