Early diagnosis and stage-adapted treatment of Wegener’s granulomatosis

2003 ◽  
Vol 117 (3) ◽  
pp. 208-211 ◽  
Author(s):  
G. Cadoni ◽  
S. Agostino ◽  
E. Campobasso ◽  
P. Vulpiani ◽  
R. Manna ◽  
...  

A case of Wegener’s granulomatosis (WG) presenting with hearing loss and right facial nerve palsy is reported. The definitive diagnosis was based upon clinical data and serum cANCA and AECA detection. Early assessment of WG prevented surgical facial nerve decompression to treat a chronic otitis media complication. Immunosuppressive therapy with steroids, cyclophosphamide and methotrexate was required for relief of clinical symptoms and cANCA negativity as an expression of disease remission. The effectiveness of co-trimoxazole for preventing relapses of WG is discussed.

1998 ◽  
Vol 107 (7) ◽  
pp. 555-559 ◽  
Author(s):  
Paul Dagum ◽  
Joseph B. Roberson

Wegener's granulomatosis, characterized by necrotizing granulomas and vasculitis of the respiratory tract and kidney, frequently first presents with otologic symptoms. We report a case of primary otologic Wegener's granulomatosis in a patient who presented with symptoms of acute otomastoiditis and associated facial nerve palsy. The patient subsequently developed neuropathies of various cranial nerves. The patient underwent urgent mastoidectomy with facial nerve decompression. Nonspecific inflammatory disease of the mastoid mucosa delayed the correct diagnosis of Wegener's granulomatosis, which was confirmed by an elevated level of cytoplasmic-pattern antineutrophil cytoplasmic antibody (cANCA). We contrast the specificity of middle ear mucosal disease and cANCA serum levels in the diagnosis of Wegener's granulomatosis.


2008 ◽  
Vol 138 (6) ◽  
pp. 799-800 ◽  
Author(s):  
F. Preuss Simon ◽  
Markus Stenner ◽  
Dirk Beutner ◽  
Matthias Laudes ◽  
Jens Peter Klussmann

2016 ◽  
Vol 2016 ◽  
pp. 1-3 ◽  
Author(s):  
Roy Ujjawal ◽  
Pan Koushik ◽  
Panwar Ajay ◽  
Chakrabarti Subrata

Wegener’s granulomatosis or granulomatosis with polyangiitis is a necrotizing vasculitis affecting both arterioles and venules. The disease is characterized by the classical triad involving acute inflammation of the upper and lower respiratory tracts with renal involvement. However, the disease pathology can affect any organ system. This case presents Wegener’s granulomatosis presenting with facial nerve palsy as the first manifestation of the disease, which is rarely reported in medical literature.


1980 ◽  
Vol 94 (6) ◽  
pp. 649-657 ◽  
Author(s):  
I. H. Calonius ◽  
C. K. Christensen

SummaryIn two patients with Wegener's granulomatosis, treatment-resistant serous otitis media was followed by unilateral facial nerve palsy. Later both patients developed uraemia due to extracapillary glomerulonephritis. In one of them, who was treated with prednisone and cyclophosphamide, renal function improved, while in the other patient it remained impaired. Both patients suffered lasting hearing impairment in spite of general improvement during the course of the disease. The facial nerve palsy improved slightly in one, while no improvement was seen in the other.The possibility of Wegener's granulomatosis or other autoimmune collagen diseases should be considered in cases of treatment-resistant serous otitis media. Early diagnosis and treatment can possibly save the patient from the lifethreatening consequences of these diseases.


2001 ◽  
Vol 258 (4) ◽  
pp. 198-202 ◽  
Author(s):  
A. C. Nikolaou ◽  
Konstantinos C. Vlachtsis ◽  
Michalis A. Daniilidis ◽  
Dimitrios G. Petridis ◽  
Ioannis C. Daniilidis

1996 ◽  
Vol 110 (5) ◽  
pp. 459-461 ◽  
Author(s):  
J. D. Hern ◽  
L. J. Hollis ◽  
G. Mochloulis ◽  
P. Q. Montgomery ◽  
N. S. Tolley

AbstractWegener's granulomatosis is a multi-system disease characterized by granuloma formation and a necrotizing vasculitis. It classically presents with involvement of the upper and lower respiratory and renal systems. However locoregional disease is common and may include otological manifestations. Facial nerve palsy has been reported during the course of the disease process but it is extremely rare for it to be the presenting feature. Previously reported cases have involved a protracted diagnostic process including exploratory tympanotomy, mastoidectomy and facial nerve decompression. We report a case of Wegener's granulomatosis which presented with a facial nerve palsy. An early diagnosis was achieved by measurement of the erythrocyte sedimentation rate (ESR), followed by serological assay of cytoplasmic anti-neutrophil cytoplasmic antibody (cANCA). Histological examination of nasal mucosal biopsies confirmed granuloma formation consistent with a diagnosis of Wegener's granulomatosis. This allowed early treatment with immunosuppressive therapy and avoided unnecessary and potentially hazardous middle ear surgery.


1978 ◽  
Vol 87 (1) ◽  
pp. 60-69 ◽  
Author(s):  
Stephen J. Wetmore ◽  
Charles E. Platz

The term lethal midline granuloma has been used to describe a number of lesions which may present in the midface. A list of possible causes is presented and three entities, midline malignant reticulosis, Wegener's granulomatosis, and malignant lymphoma, are discussed in detail. The pathological descriptions, the clinical features, and the recommended forms of therapy are outlined. It is important to vigorously pursue the patient with a midline facial lesion until a definite tissue diagnosis is obtained. Multiple biopsies may be necessary because necrosis and nonspecific inflammatory changes may be present in various of these disease entities. The correct diagnosis is essential because the treatment of these diseases is different: Wegener's granulomatosis responds best to chemotherapy, whereas midline malignant reticulosis and lymphoma should be treated with radiation therapy if they are localized. Despite conceptual variations, most recent authors believe that these three diseases exist as separate entities, and furthermore, recommend that the term lethal midline granuloma be either dropped from the lexicon or relegated to a descriptive clinical term to be used only until a more definitive diagnosis can be made.


1998 ◽  
Vol 19 (1) ◽  
pp. 54-60 ◽  
Author(s):  
Dana M. Hartl ◽  
Patrick Aïdan ◽  
Olivier Brugière ◽  
Olivier Sterkers

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