Abstract Vasculidities involve complex interactions of immune dysregulation leading to inflammation of blood vessels resulting in tissue damage due to necrotizing granulomatous progression with multisystem involvement. A 24-year-old Mexican immigrant female without significant past medical history other than working at a pet store with intermittent exposure to birds, rodents, guinea pigs and ferrets presented to the ED with a 10 day history of progressive worsening of fever and productive cough despite antibiotic treatment. Recently referred to podiatry for painful purple nodules that progressed to ulceration, failed to improve with antibiotics. Denied night sweats, weight loss, hemoptysis, sick contacts, outside travel, IV drug misuse or TB exposure. Initial vital signs temperature 100.5 F, heart rate 144 bpm, respiratory rate 26 with 97% room air sat. Normal physical exam except for previously described nodules. ED workup with WBC 16.1 x 10*3/uL, CRP 250 mg/L, ESR 83 mm/hr. CT angiogram of the chest revealed thick walled cavitary masses with irregular inner margin, surrounded by ground-glass, involving the inferior aspect of the left upper lobe and the superior segment of the left lower lobe, multiple (largest being 7.5 cm x 5.6 cm). ID was consulted for concern of TB vs necrotizing pneumonia, broad spectrum antibiotics were started. ID work up for HIV, blood cultures, Influenza A, Influenza B, RSV, Aspergillus, AFB smear, Chlamydia psittaci, hepatitis B, C, Histoplasmosis, Blastomycosis and Cryptococcosis returned negative. Autoimmune work up Rheumatoid factor 162 U/mL, CCP IgG/IgA negative, ACE 32 U/L, Complement C3 88 mg/dL, C4 5 mg/dL, Anti- PR3 antibodies positive with 4.9 units, C-ANCA titers of 1:40 (normal 1:20 titer). WIth persistent symptoms and onset of hemoptysis, Pulmonology was consulted. Bronchoscopic evaluation was negative bacterial and fungal stain/culture PJP PCR, aspergillus antigen, Mycobacterium PCR and nocardia. Lung biopsy, brushing negative for granulomatous inflammation and malignancy. A right ankle ulcer biopsy revealed prominent vascular proliferation in the dermis, ulcer with fibrinoid necrosis and neutrophilic infiltrate suggestive of pyoderma gangrenosum. Given rapidly progressive thick walled cavitary nodules in multiple locations with surrounding ground glass opacities and tissue biopsy with pyoderma gangrenosum, elevated inflammatory markers, negative infectious disease work up and autoimmune workup revealing ACR/EULAR score 7, patient has been diagnosed with Granulomatosis with Polyangiitis. She was started on Prednisone 50 mg daily and Methotrexate 25 mg weekly achieving complete resolution of hemoptysis within 48 hrs. This abstract is funded by: None
Ganampet et al. (Fri,) studied this question.