443. A path of trial and tribulation to diagnose full-blown ANCA-negative EGPA – a case report.
Creators: name: Gauckler1, Philipp
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Presentation of Case </strong>A 71-year old male was admitted with a 3 months history of dyspnea, general weakness and 5 kg weight loss. Adult-onset asthma with accompanying peripheral blood eosinophilia (PBE) was diagnosed and a treatment including montelukast and a short course of low-dose oral prednisolone for one week was started. Five months later, the patient was transferred from a peripheral hospital to our institution with persisting weakness, a further 10 kg of weight loss, bi-pulmonary nodular lesions and mucosal thickening of the maxillary sinuses on CT scan. Further laboratory analyses revealed an increase of PBE from 14.9% to 62%. Tissue specimen obtained from bronchoscopy revealed bronchitis with tissue eosinophilia. Further diagnostics for an underlying cause including (parasitic) infections and systemic autoimmune diseases (ANA, ANCA) were normal. Transthoracic echocardiography and cardiac MRI revealed, multiple intracardiac thrombi (bi-ventricular and pulmonary valve) and an endocardial late enhancement suggesting endocardial fibrosis. A haematologist was consulted and hypereosinophilic syndrome (HES) was suspected. Anticoagulation with s.c. enoxaparine (2 mg/kg weight daily dose) was started. Four days later, the patient presented with dizziness. A cranial CT revealed occipital mass bleeding and the patient was transferred to intensive care unit. No underlying bleeding-cause could be detected in a cranial MRI. Further neurologic assessment then revealed mononeuritis multiplex of the median nerve and lower limbs, suspicious for vasculitis. Biopsy of the sural nerve confirmed a perineural eosinophilic infiltration. A PET-CT scan showed no evidence of underlying malignancy and (large-vessel) vasculitis. In the following days, the patient developed acute kidney injury. Urinalysis revealed proteinuria (700 mg/g PCR) and haematuria. A kidney biopsy was performed without a definite diagnosis. With fulfilment of all 6 of the American College of Rheumatology 1990 classification criteria, diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) was established. An induction treatment with high-dose glucocorticoids and rituximab was started and montelukast was discontinued as a potential trigger of disease. <strong>Diagnostic Testing: </strong>Serial laboratory testing, Serial CT scans of trunk and cranium, TTE + cardiac MRI, Bronchoscopy + biopsy, PET-CT scan, Sural nerve biopsy, Kidney biopsy <strong>Differential &amp; Final Diagnosis: </strong>Asthma, HES, Montelukast-induced EGPA, Eosinophilic granulomatosis with polyangiitis (EGPA) <strong>Discussion of Management: </strong>There was a 9 months delay from onset of symptoms until the final diagnosis was established. The initial diagnosis of asthma is typical, but presence of systemic disease (weakness, weight loss, reduced physical fitness) could have aroused suspicion earlier. Starting anticoagulant treatment without treating the underlying pathology could not stop disease activity and resulted in severe treatment complication. Negative serologic testing for ANCA is frequent in EGPA(1) and significantly complicates diagnosis. Furthermore, EGPA and HES show significant clinical overlap, especially in ANCA-negative patients (2). <strong>Conclusions:</strong> Diagnosis of EGPA is difficult and often delayed, even if all criteria are fulfilled. Early diagnosis and prompt treatment is essential to prevent critical damage. An interdisciplinary patient management is mandatory. <strong>Disclosures</strong> PG has received consulting fees from UriSalt (not related with the present work). MR has nothing to disclose in relation with the present work. HN has nothing to disclose in relation with the present work. AK has received consulting fees from UriSalt, Alexion, Vifor Pharma, Otsuka, and Catalyst Biosciences