The term hemoptysis comes from the Greek words haima meaning blood, and ptysis meaning spitting. Though this is a simple definition, discerning the source of the blood is not always simple. The differential is very broad and includes the categories of infection, vascular, pulmonary and, neoplastic etiologies. The work up involves many modalities such as history and physical, radiography, lab studies and bronchoscopy. This case is an example of an uncommon etiology with an interesting diagnostic work up.
Patient is a 32 year-old female with 2-3 weeks of blood-streaked sputum, which occurred daily. She had no fevers, chills, cough, epistaxis, night sweats, weight loss, chest pain, or gastro-intestinal complaints though she had chronic intermittent left sided chest pain. Her history is significant for Iron deficiency anemia and recurrent urinary tract infections (UTI’s), which she gets 3-4 times per year. She emigrated from Poland 10 years ago. She has a 12-pack year tobacco history and no sick contacts or recent travel. On physical exam she had left costo-phrenic tenderness, her lungs were clear to auscultation, she had no adenopathy and her abdominal exam was benign. Labs revealed a 20 millimeter PPD, a white blood cell count of eleven and a microcytic anemia with a hematocrit of thirty-five. Her Acid Fast Bacillus (AFB) smears and cultures were negative times nine. A chest x-ray revealed a left lower lung mass and a subsequent CT scan revealed a renal mass that extended through the diaphragm into the lower lobe of her left lung. Bronchoscopy showed normal airways without evidence of bleeding. Bronchoalveolar lavage, transbronchial biopsies and cytobrush were non-diagnostic including negative AFB smears and cultures. The differential included xanthogranulomatous pyelonephritis (XGP), tuberculosis, and malignancy. Based on history and CT findings the decision was made to proceed with surgery, which included a left radical nephrectomy, partial diaphragmatic resection, diaphragmatic fistula repair, partial gastric resection and partial left lower lobectomy. The pathology revealed XGP with lipid-laden macrophages (xanthoma cells), giant cells and plasma cells. The culture from the tissue grew out Proteus mirabilis. She recovered uneventfully without recurrence of hemoptysis.
XGP, first described in 1916, is the sequelae of chronic, severe obstructive parenchymal inflammation. There is destruction of the renal medulla and cortex, which is the result of normal cells being replaced by sheets of lipid-laden macrophages (xanthomas). XGP has three stages: Stage I (nephric) granuloma tissue is confined to the kidney; Stage II (perinephric) granuloma invades the kidney and Gerota’s fat; Stage III (paranephric) granuloma invades the kidney, fat and retroperitoneal structures which include gastro-intestinal structures, the diaphragm and rarely extension into the lungs. Patients present with fever, flank pain, recurrent UTI’s from gram-negative organisms and, anemia. The differential includes renal carcinoma, malakoplakia, megalocystic interstitial nephritis and inflammatory processes such as pyonephrosis, tuberculosis and abscess secondary to renal calculi. The preferred diagnostic tool is the CT scan, which can exclude renal cancer, detect renal stones and determine the extent of granuloma involvement. Macroscopic pathology reveals necrotic yellow material and layers of orange-colored tissue. Microscopic findings include necrosis with lymphocytes and macrophages, vascularized granulation tissue, giant cells and cholesterol clefts. The pathogenesis and etiology of XGP are unknown but it is an inflammatory process that may be due to a defect in the processing of bacteria by macrophages. Treatment consists of a total nephrectomy, removal of all involved tissue and closure of all fistulas. Prognosis is excellent following surgery. There are over 300 reported cases of XGP but only a few reported cases of it involving the lung.
We present an unusual case of hemoptysis due to xanthogranulomatous pyelonephritis involving the lung.
Michael Baydarian, None.