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A case study of a 39-year-old man with chest pain and other complications is discussed to better understand the growth of mediastinal mass which affects the pulmonary arteries and cardiac conduction system, onset of arthritis, etc. The only way to explain the disease from the symptoms is Wegener's granulomatosis.

A 55-year-old man presented to our emergency department with ankle pain and swelling. He had been in his usual state of health until 2 weeks before presentation, when he noticed [...]

With reference to a case study of three patients, the clinical representation of Wegener's granulomatosis (WG) in the oral cavity is examined. The oral expression of WG helps in identifying its causes, symptoms and in managing it. The role dentists play in identifying WG is discussed.

Byline: Ozlem Erdogan (1), AyAe Aner (1,5), Gulay Demircin (1), Mehmet Bulbul (1), Leyla MemiA (2), Cigdem Uner (3), Nural Kiper (4) Keywords: Overlap syndrome; Systemic lupus erythematosus; Wegener granulomatosis Abstract: An 11-year-old boy with consecutive development of systemic lupus erythematosus (SLE) and Wegener granulomatosis (WG) is presented. He was first admitted to the hospital with the findings of SLE, including crescentic glomerulonephritis, Coombs test-positive hemolytic anemia, hypocomplementemia, antinuclear antibody (ANA) positivity, and elevated levels of anti-double-stranded (ds) DNA antibodies. He was treated successfully with steroids, cyclophosphamide, and peritoneal dialysis. One month after his discharge he developed an apparent viral infection. Three weeks afterwards he was readmitted with the findings of lower respiratory tract involvement, maxillary sinusitis, nasal septum perforation, p- and c-antineutrophil cytoplasmic antibody (ANCA) positivity, but normal complement, ANA, and anti-ds DNA levels, suggesting the diagnosis of WG. He did not respond to anti-infectious and immunosuppressive treatment, and he died of Pseudomonas sepsis. Author Affiliation: (1) Department of Pediatric Nephrology, Dr. Sami Ulus Children's Hospital, Ankara, Turkey (2) Department of Pathology, University Hospital of Gazi, Ankara, Turkey (3) Department of Radiology, Dr. Sami Ulus Children's Hospital, Ankara, Turkey (4) Department of Pediatric Chest Disease, University Hospital of Hacettepe, Ankara, Turkey (5) Bilkent 1, CamlA+-k Sitesi, D7-Blok, no 21, 06530 Ankara, Turkey Article History: Registration Date: 23/10/2003 Received Date: 06/01/2003 Accepted Date: 08/10/2003 Online Date: 23/01/2004

A 48-year-old man admitted to a hospital with blood in his sputum and urine and an abnormal chest X-ray was diagnosed with Wegener's granulomatosis. This medical condition is caused by an inflammation inside arteries that usually affects the lungs, upper respiratory tract, and kidneys.

A young girl hospitalized with fever, rash, declining vision in one eye, weight loss, and drainage from the ear illustrates the presentation and diagnosis of systemic lupus erythematosus. The constellation of symptoms suggests an autoimmune disease or an infectious disease, such as herpes or HIV. Tests for antinuclear antibodies, HIV antibodies, antineutrophil cytoplasmic antibodies, and other serological values were performed. Following a diagnosis of lupus, with features of Wegener's granulomatosis, the patient received methylprednisolone and zidovudine, with considerable improvement in her symptoms.

Byline: Carmen Mejia-Hernandez (0), Antonio Alvarez-Mendoza (0), Beatriz DeLeon-Bojorge (0) Keywords: Key words: Takayasu's arteritis, Wegener's granulomatosis, overlap syndrome, crescentic glomerulonephritis Abstract: A case of Wegener's granulomatosis (WG) coexisting with Takayasu's arteritis (TA) in a 12-year-old girl is presented. She presented with fulminant and severe renal insufficiency due to crescentic glomerulonephritis. At autopsy, aortic lesions of Takayasu's arteritis coexisted with pulmonary and renal findings of WG, and the patient's serum at autopsy had an elevated level of antineutrophil cytoplasmic antibodies (ANCA). Both forms of vasculitis have been thought to be triggered by infectious agents and it is postulated that this occurrence accounts for the coexistence of the two forms of vasculitis in this case. Author Affiliation: (0) Department of Pathology, Instituto Nacional de Pediatria, Insurgentes Sur No. 3700-C, Mexico City, DF 04530, Mexico , MX Article note: Received February 20, 1998 accepted August 31, 1998.

A 74-year-old woman was hospitalized after she fell and was unable to get up due to generalized weakness. She had a history of headaches, weakness and falls, and colon and endometrial cancer. A brain biopsy revealed that she had meningitis and hydrocephalus, or water on the brain. Her doctors suspected a condition called Wegener's granulomatosis, which was confirmed by testing blood samples for the presence of antineutrophil cytoplasmic antibodies (ANCA). These antibodies are often found in patients with Wegener's granulomatosis, an inflammatory disease that causes little nodules to form in various organs.

A 69-year-old woman with a mass in the area of her right eye and conjunctivitis was diagnosed with Wegener's granulomatosis involving the soft tissue of the right eye and breast. Wegener's granulomatosis is a progressive disease causing changes in the tissues of the body known as granulomas. It commonly begins in the eye. Important features of the mass in the eye were ill-defined margins, anterior location, and independence of other structures of the eye. The patient had also recently experienced fatigue, anorexia, and weight loss, and an antineutrophil cytoplasmic antibodies (ANCA) test was positive for symptoms of Wegener's granulomatosis. The mass found in the breast was removed and did not recur. The patient was successfully treated with steroids and cyclophosphamide.

A 17-year-old girl was diagnosed with Goodpasture's syndrome and Wegener's granulomatosis involving the lungs, kidney and spleen. Goodpasture's syndrome is an inflammation of the kidney glomeruli associated with heavy bleeding in the lungs and antibodies against basement membrane antigens. Wegener's granulomatosis is characterized by an accumulation of inflammatory cells that may be a hypersensitivity reaction to an antigen. She was hospitalized because of kidney failure and coughing up blood-tinged sputum and blood. She developed symptoms of an upper respiratory tract infection, headache, loss of appetite, nausea, vomiting, shortness of breath and chest pain shortly before admission. Her arterial-blood gas levels, oxygenation and blood pressure readings fell continuously while in the hospital. CPR attempts failed and she died about 7 1/2 hours after admission. She tested positive for anti-glomerular basement membrane antibodies at the time of her death.

A 40-year-old woman was admitted to a hospital because of a cough that was occasionally accompanied by blood. A previous examination had found narrowing of her trachea. Examination of a biopsy specimen of her tracheal tissue showed ulceration and inflammation. A CT scan showed irregularities in both bronchi, the muscular tubes extending from the trachea to the lungs. A bronchial biopsy confirmed the diagnosis of Wegener's granulomatosis of the trachea. She was treated with prednisone and cyclophosphamide.

Byline: Toshiyuki Ohta (1,4), Takashi Sakano (1), Mami Shiotsu (1), Takeki Furue (1), Hideyuki Ohtani (1), Yoshihisa Kinoshita (1), Tatsuya Mizoue (2), Katsuzo Kiya (2), Issei Tanaka (3) Keywords: Hypertension; Chronic renal failure; Reversible posterior leukoencephalopathy syndrome; Wegener granulomatosis; Anti-neutrophil cytoplasmic antibody Abstract: A 14-year-old girl with rapidly progressive glomerulonephritis was transferred to our hospital because of acute renal failure. A diagnosis of Wegener granulomatosis was made according to the symptom triad of a renal biopsy demonstrating crescentic glomerulonephritis, severe sinusitis, and serological findings of raised proteinase 3 anti-neutrophil cytoplasmic antibody level. In spite of combination therapy with methylprednisolone, cyclophosphamide, and plasma exchange, her renal function gradually deteriorated. Thereafter, she suffered a severe headache and generalized seizures. Brain computed tomography (CT) scan revealed bilateral low-density areas in the parieto-occipital lobes. Magnetic resonance imaging (MRI) disclosed a high-intensity area on T2-weighted images and a low-signal intensity area on T1-weighted images in the same lesion. Follow-up brain CT scan 3 weeks and MRI 2 months after the first studies showed complete resolution of the abnormal lesions, which indicated reversible posterior leukoencephalopathy syndrome. In addition to renal failure, hypertension, and cyclophoshamide, the primary disease may have played a role in the development of this uncommon syndrome in our patient. Author Affiliation: (1) Department of Pediatrics, Hiroshima Prefectural Hospital, Hiroshima, Japan (2) Department of Neurosurgery, Hiroshima Prefectural Hospital, Hiroshima, Japan (3) Dialysis Center, Hiroshima Prefectural Hospital, Hiroshima, Japan (4) Department of Pediatrics, Hiroshima Prefectural Hospital, 1--5-54 Ujinakanda, Minami-ku, Hiroshima 734--8530, Japan Article History: Registration Date: 01/01/2003 Received Date: 28/02/2003 Accepted Date: 14/07/2003 Online Date: 16/12/2003

A 78 year old man presented with acute renal failure following a prolonged respiratory illness. A renal biopsy demonstrated severe suppurative interstitial nephritis with normal glomeruli. After nine weeks of [...]