444. Large vessel involvement in antineutrophil cytoplasmic antibodies associated vasculitis-a rare presentation
Creators: name: Kayidhi1, Sunitha
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 50year older man presented with a history of fatigue, loss of appetite, loss of weight, on and off fever for the last six months. He also gave a history of central back pain that used to aggravate in a supine position and relieve bending forward. He had intermittent small joint pains without any swelling or early morning stiffness. He was diagnosed with new-onset diabetes and started on oral hypoglycemic agents prior to visiting us. He has had pain and blackish discoloration of the right fourth, fifth toes, and lateral aspect of sole for 10 days. He did not give any history of sinus pain, recurrent nasal or ear discharge, hearing loss, cough, or shortness of breath. There was neither any history of oral ulcers or excess hair loss or photosensitivity or dry mouth or dry eyes. He did not complain of claudication pain. On examination, he was moderately built and moderately nourished. All peripheral pulses felt with normal rate and rhythm. There was no carotidynia, or bruit felt. The examination also revealed gangrene Right fourth and fifth toe and tender erythematous patches over the lateral border of the sole. His Blood pressure was 140/90mm Hg. Musculoskeletal examination revealed tender PIP, MCP joints. Cardiovascular, respiratory, and abdominal examination was normal. <strong>Diagnostic Testing: </strong>Blood tests revealed anemia (Hemoglobin- 9.5gm/dl),leucocytosis(12,360)and high ESR(80mm) and CRP132 mg/dl).Liver and renal function tests were normal. Urine examination revealed proteinuria and active sediment (2+albumin,6-8 pus cells .6-8 RBC and granular casts).ANCA by immunofluorescence showed a perinuclear pattern(2+). Rheumatoid factor was positive(26 IU, normal&lt;12 IU). Antinuclear and anticardiolipin antibodies were negative. Serum complements were within the normal range. Serum Igg4 levels were normal. Blood and urine cultures were sterile.2D echocardiogram was normal. CT abdomen showed circumferential thickening of the right brachiocephalic artery, bilateral distal common carotid artery, abdominal aorta extending into common iliac artery and internal iliac artery, mild narrowing of left proximal renal and inferior mesenteric artery, right mild hydroureteronephrosis. PET CT also showed the above-mentioned vasculitic changes and avid uptake of the distal thoracic, whole of the abdominal aorta, and left proximal common iliac artery. Renal biopsy showed pauci immune glomerulonephritis(GN) with a focal fibro cellular crescent. <strong>Differential &amp; Final Diagnosis</strong>: 50-year-old male with the above clinical picture, the differentials considered are ANCA vasculitis, Takayasu arteritis, IGG4RD, cryoglobulinemic vasculitis, infection, or malignancy-associated vasculitis. There was no evidence of infection (cultures were sterile, procalcitonin normal) and malignancy (PET CT-normal) on evaluation. His C-ANCA was strongly positive, and renal biopsy showed pauci-immune crescentic GN, and therefore we diagnosed him with ANCA vasculitis with aortitis, crescentic GN, and gangrene. <strong>Discussion of Management: </strong>Because of severe /organ-threatening involvement, he was treated with pulse methylprednisolone (1000mgx 3 days), followed by oral prednisolone (1mg/kg bd wt)for one month, and then steroids were slowly tapered. Cyclophosphamide pulses were also prescribed as per the EUVAS protocol. His symptoms improved, and his urine examination showed a decrease in proteinuria and sediment over two months. <strong>Conclusions:</strong> ANCA vasculitis must be considered one of the differentials in aortitis cases though it is a rare presentation. <strong>Disclosures:</strong> None