A destructive nasal lesion and glomerulonephritis

Abstract

CASE PRESENTATION A 19-year-old gravid Hispanic female (G2P1) at an unknown gestational age presented to the Emergency Room (ER) with new onset of gross hematuria and a 2-week history of swelling and tenderness of the nose and upper lip. She was prescribed a 10-day course of azithromycin for presumed cellulitis. After 10 days, she returned to the ER with worsening nasal swelling and complaints of lightheadedness. She denied fevers, chills, night sweats, hemoptysis, dysuria, shortness of breath, cough, oral ulcers, dysphagia, or rashes. The patient had moved to the US from Mexico 6 months prior to admission. Her only significant past medical history was chronic upper airway irritation for 2 years. There was no family history of renal disease. Her temperature was 100.11F, with a regular pulse of 110 beats/min, and respirations of 15/min. Blood pressure was 104/66 while seated. The nasal alae were edematous with erythema extending to the philtrum (Figure 1a). The nasal septum was necrotic with an anterior perforation and purulent discharge. The sclerae were anicteric and no oral erythema or ulceration was seen. There was no lymphadenopathy. Cardiovascular examination revealed a regular tachycardic rhythm with normal heart sounds and a grade III/VI systolic ejection murmur. Neurologic, pulmonary, and abdominal examinations were unremarkable. The extremities revealed normal pulses without edema or clubbing. Laboratory values on presentation were as follows: hemoglobin, 6.1 g/dl (61 g/l) (normal range, 13–18 g/dl (130–180 g/l)); white-cell count, 5.5 10/l (normal range 4.3–10.8 10/l) with a normal differential; platelet count, 348 10/l (normal range, 150–500 10/l); BUN, 18 mg/dl (6.4 mmol/l) (normal range, 10–30 mg/dl (3.6–10.7 mmol/l)); serum creatinine, 1.7 mg/dl (150mmol/l); serum protein, 5.8 g/dl (58 g/l) (normal range, 6.3–8.2 g/dl (63–82 g/l)); serum albumin, 2.7 mg/dl (normal range, 3.5–4.9 g/dl (35–49 g/l)). The creatinine clearance was 28 ml/min. Serum sodium, potassium, chloride, calcium, bicarbonate, glucose, transaminases, and alkaline phosphatase were normal. Urinalysis showed 3þ protein and 4þ blood. Microscopic examination of the urine showed many RBCs per high power field (too numerous to count), multiple red blood cell casts, waxy casts, epithelial casts, and hyaline casts. The 24-h urine protein was 3984 mg. The following serologies were negative or normal: anti-neutrophil cytoplasmic antibody, anti-nuclear antibody, anti-double stranded DNA antibody, hepatitis B surface antigen, hepatitis C antibody, human immunodeficiency virus antibody, anti-glomerular basement membrane antibody, antistreptolysin-O, and serum complements. The erythrocyte sedimentation rate was greater than 140 mm/h. Computer tomography of the sinuses disclosed extensive bilateral nasal soft tissue swelling extending to the medial premaxillary region associated with a small perforation of the nasal septum and local hemorrhage. Chest radiograph was unremarkable. The ultrasound of the abdomen was consistent with a pregnancy of 9 weeks and 5 days. Renal ultrasound showed normal-sized kidneys with normal echogenicity. The patient was hospitalized for blood transfusions and supportive care. She was treated empirically with nafcillin for nasal cellulitis. A renal biopsy and endoscopic nasal biopsy were performed. Although anti-neutrophil cytoplasmic antibody serologies were negative, the possibility of Wegener’s granulomatosis was strongly entertained based on the presentation with a destructive nasal lesion, hematuria, and acute renal failure. http://www.kidney-international.org t h e r e n a l c o n s u l t