Wegener' granulomatosis is an autoimmune diseases, often involving the lung and kidney, has a high mortality rate in nontreatment patients. The low incidence and nonspecific features, often lead to misdiagnosis and delayed treatment. This paper reported the diagnosis and treatment of a 55-year-old female patient with primary Wegener' granuloma of the lung diagnosed by percutaneous lung biopsy of pulmonary nodules, and reviews the relevant literature.
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Female ; Granulomatosis with Polyangiitis/drug therapy* ; Humans ; Lung Neoplasms ; Middle Aged ; Pneumonia

Adrenal Cortex Hormones ; therapeutic use ; Antiviral Agents ; therapeutic use ; Diagnosis, Differential ; Granulomatosis with Polyangiitis ; diagnosis ; Humans ; Lung Neoplasms ; diagnostic imaging ; drug therapy ; pathology ; Lymphomatoid Granulomatosis ; diagnostic imaging ; drug therapy ; pathology ; Prognosis ; Radiography ; Sarcoidosis ; diagnosis ; Tuberculosis, Pulmonary ; diagnosis

No abstract available.
Adult ; Biopsy ; Bone Marrow Examination ; Female ; Glucocorticoids/therapeutic use ; Granulomatosis with Polyangiitis/*diagnosis/drug therapy/immunology ; Humans ; Lymphohistiocytosis, Hemophagocytic/*diagnosis ; Predictive Value of Tests ; Recurrence ; Treatment Outcome

OBJECTIVETo compare the clinical and pathological manifestations of patients with antineutrophil cytoplasmic autoantibodies (ANCA) directed against proteinase 3 (anti-PR3) or myeloperoxidase (anti-MPO).

METHODSOne hundred and forty patients with ANCA were detected for anti-PR3 and anti-MPO by ELISA. The clinical features at presentation, histopathological characteristics and outcome of all patients who were tested positive for anti-PR3 or anti-MPO were analysed.

RESULTSIn anti-PR3 group (n = 21), 16 cases (76.2%) had systemic vasculitis, in which Wegener's granulomatosis prevailed (13 cases, 61.9%). In anti-MPO group (n = 31), 19 cases (61.3%) were diagnosed as systemic vasculitis and 12 cases (38.7%) as microscopic angiitis. For vasculitic patients with anti-PR3 and anti-MPO, the disease duration at diagnosis was 9.6 +/- 2.0 m and 4.4 +/- 0.9 m respectively, P < 0.05; vasculitis activity index (BVAS) and mean number of affected organ were 22.5 +/- 2.1, 5.0 +/- 0.4 and 25.1 +/- 1.7, 4.8 +/- 0.4 respectively, P > 0.05; upper respiratory tract, eye and joint involvements were 11(68.8%), 7(43.8%), 11(68.8%) and 7(36.8%), 2(10.5%), 5(26.3%) respectively, P < 0.05. Although there was no statistical difference in renal involvement between these two groups, patients with serum creatine > 500 micromol/L were more commonly seen in anti-MPO group than in anti-PR3 group, which were 8(42.1%) and 2(12.5%) respectively, P < 0.05. Ten relapses were seen in anti-PR3 group and only 2 in anti-MPO group, but the acute mortality rate in anti-MPO group (5/19, 27.4%) was much higher than that in anti-PR3 group (1/16, 6.3%).

CONCLUSIONSAnti-PR3 and anti-MPO occurred mainly in systemic vasculitis. A large divergence was seen in the disease spectrum between patients with anti-PR3 and those with anti-MPO. In particular, upper respiratory tract, eye and joint involvements, granuloma formation and relapse were more prominent in anti-PR3 patients. By contrast, the anti-MPO patients had a more acute disease onset, more rapid progressive renal involvement and a higher acute mortality rate.

Antibodies, Antineutrophil Cytoplasmic ; analysis ; Autoantibodies ; analysis ; Follow-Up Studies ; Granulomatosis with Polyangiitis ; drug therapy ; immunology ; pathology ; Humans ; Immunosuppressive Agents ; therapeutic use ; Kidney ; pathology ; Myeloblastin ; Peroxidase ; immunology ; Respiratory System ; pathology ; Serine Endopeptidases ; immunology ; Vasculitis ; drug therapy ; immunology ; pathology

Upper airway disease, especially nasal and paranasal sinus involvement, is the most common manifestation of Wegener's granulomatosis. Chronic otitis media and facial palsy are rare but well known presenting features of Wegener's granulomatosis. We report a 40-year-old woman who presented with complaints of ear discharge, deep-seated ear pain and loss of hearing in her right ear. Early diagnosis demands heightened suspicion in a patient with otological symptoms and facial paralysis.
Administration, Oral ; Adult ; Biopsy ; Chronic Disease ; Cyclophosphamide ; therapeutic use ; toxicity ; Diagnosis, Differential ; Drug Therapy, Combination ; Facial Paralysis ; etiology ; pathology ; Fatal Outcome ; Female ; Granulomatosis with Polyangiitis ; diagnosis ; pathology ; Humans ; Immunosuppressive Agents ; therapeutic use ; toxicity ; Nasal Mucosa ; pathology ; Neutropenia ; chemically induced ; Otitis Media with Effusion ; etiology ; pathology ; Plasma Exchange ; Prednisolone ; therapeutic use ; toxicity ; Renal Dialysis