A 55 year-old white male and his wife arrived in your office. He does not like to see doctors and the last time he went to a doctor’s office was when he was a baby. This time his wife insisted that he must see a doctor because his symptoms have only been getting worse for the past year. He reports he has had slight fever, fatigue, loss of appetite, weight loss, nose bleedings, sinus pains, painful gums, hoarseness of voice, repeated ‘ear infections’, ‘all my joints hurt’ joint pains, cough, bloody sputum, chest pains, ‘urine looks foamy and bloody’. He remembered having had a few tick bites 4 months ago while taking a hike in the nearby mountain. His wife insists on getting a Lyme test and prescribing doxycycline immediately. On physical examination, you noticed perforation of the nasal septum, collapse of the nasal bridge, swollen gums, right-sided facial palsy, tender, warm, swollen joints and palpable purpura on both upper and lower extremities. Suspecting a serious systemic disorder involving joints, you consulted a rheumatologist and together you ordered different laboratory and imaging tests for this patient. A chest computed tomography (CT) scan showed a cavitary lesion measuring 40 mm x 80 mm in the left lower lung lobe, subglottic tracheal stenosis and diffuse alveolar hemorrhage. Rheumatoid Factor……..Positive White blood cell count…..12,600/mm3 Erythrocyte sedimentation rate…..60 mm/hr Sputum cultures for Mycobacterium tuberculosis…..negative Anti-neutrophil cytoplasmic antibody (c-ANCA), Myeloperoxidase antibody……18 AU/ml and PR-3 antibody 28 AU/ml Lung biopsy showed non-caseating granulomatous inflammation and vasculitis Borrelia burgdorferi antibody enzyme immunoassay………0.7 Kidney biopsy: Necrotizing granuloma, vasculitis, multinucleated giant cells, palisading histiocytes, clusters of neutrophils within the blood vessel wall, coalescence of neutrophilic microabscesses, relatively sparse immunoglobulin and complement depositions, segmental necrotizing glomerulonephritis with multiple crescents Urinalysis…….Glucose – negative Blood – positive Protein – positive ECG…. showed diffuse ST-segment elevation in most leads On treatment, his symptoms responded well to two infusions of rituximab. What is the most likely diagnosis?
