Multiple Pulmonary Nodules: A Complex Case of Wegener’s Granulomatosis
A 78-year old male with past medical history significant for chronic shortness of breath (for the last two years), chronic systolic heart failure secondary to non-ischemic dilated cardiomyopathy, pulmonary hypertension, essential hypertension, coal miner’s pneumoconiosis, and chronic obstructive lung disease was found to have bilateral pulmonary nodules on pre-operative chest X-ray. The work-up for the pulmonary nodules was initially planned on an outpatient basis. However, two weeks later the patient was admitted to the hospital with progressive shortness of breath. Upon admission, vital signs included temperature 35.8°C, pulse 56 beats/min, respiratory rate 22 breaths/min, and blood pressure 110/54 mmHg. Oxygen saturation on room air was 96%. On review of systems he reported decreased appetite for the past three months and a 20 pound weight loss. He denied hematuria, rhinorrhea, rashes, sinus pain, muscle aches, ear ache, hearing loss or hemoptysis. Physical exam was significant for bilateral rhonchi on auscultation of the lungs. Fecal occult blood was negative. Electrolytes were within normal limits. Blood urea nitrogen was 18 mg/dL, creatinine 1.2 mg/dL, glomerular filtration rate 59 mL/min. The patient’s urine microscopy was initially negative. Chest X-ray revealed bilateral hilar and right upper lobe areas of infiltrate increased from the prior imaging examination. Computed tomography (CT) scan revealed multiple cavitary lesions, the largest on the right, with a large air fluid level suspicious for infection. The day after admission the patient underwent CT guided fine needle aspiration of the right lung. A catheter was placed and a 10 cc of frankly purulent material was aspirated. Pathological examination showed acute inflammation. Tissue cultures showed no growth. No acid fast bacteria were seen on smear. Potassium hydroxyde preparation was negative and no fungus was isolated. All blood cultures were the negative and so were cultures of the pleural fluid and the surgical specimen. One of the expectorant sputum samples grew Rhodococcus equi. The patient was then started empirically on ciprofloxacin and rifampin for Rhodococcus equi. However, the antibiotics were soon discontinued secondary to low suspicion for the presence of active infection. At that point the diagnosis of vasculitis was entertained and a vasculitis panel was significant for elevated sedimentation rate at 84 mm/h. Anti-glomerular basement membrane antibody level was negative, but proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA) level was elevated at 3.8 units. Liver function tests were normal.\nLung biopsy revealed chronic and acute inflammation and granuloma formation. Two weeks after hospital admission the patient complained of diarrhea. Laboratory results indicated that he was in renal failure. Stool studies were positive for Clostridium difficile for which the patient was started on flagyl. Repeat urinalysis showed brown colored urine with pH 6, specific gravity 1.023, moderate occult blood, bile negative, small leukocyte esterase, nitrate negative, 9-10 red blood cells (RBC)/high power field (HPF), 9-10 white blood cells/HPF, rare squamous epithelial cells, rare hyaline casts, 30A protein. Urine microscopy was positive for RBC casts. At that time the patient also developed purpuric rash over his extremities. The patient was empirically started on oral cyclophosphamide and IV methylprednisolone. Kidney biopsy was inadequate for diagnosis. During the course of his treatment he also completed 5 cycles of plasmapheresis. The patient remained dialysis dependent at 2 months follow-up.