Fulminant Wegener’s granulomatosis with multiorgan dysfunction

Open Medicine ◽  
2008 ◽  
Vol 3 (1) ◽  
pp. 119-122
Author(s):  
Andreja Sinkovic ◽  
Artur Pahor
Keyword(s):  
Differential Diagnosis ◽  
Wegener’S Granulomatosis ◽  
Correct Diagnosis ◽  
Serum Levels ◽  
Wegener's Granulomatosis ◽  
Proteinase 3 ◽  
Kidney Dysfunction ◽  
Multiorgan Dysfunction ◽  
Lung And Kidney ◽  
Severe Lung

AbstractFulminant cases of Wegener’s granulomatosis (WG) with severe lung and kidney dysfunction may resemble pneumonia with sepsis and multiorgan dysfunction. Increased serum levels of antineutrophyl citoplasmic autoantibodies (c-ANCA) and proteinase 3 antibodies (PR3) confirm the correct diagnosis and are essential to start early immunosupressive treatment to improve the outcome. With the emphasis on diagnostic dilemmas, we present a 61-year old patient with fulminant WG, resembling pneumonia with sepsis and multiorgan dysfunction. After antibiotics and supportive measures increased serum levels of c-ANCA and PR3 confirmed the suspicion of WG and adequate immunosupressive treatment improved the patient’s condition. We concluded that it is important to include vasculitis in the differential diagnosis of unexplained multiorgan dysfunction, because only early adequate immunosuppressive therapy in combination with other resuscitation measures improves survival.

Otologic Wegener's Granulomatosis with Facial Nerve Palsy

1998 ◽  
Vol 107 (7) ◽  
pp. 555-559 ◽  
Author(s):  
Paul Dagum ◽  
Joseph B. Roberson
Keyword(s):  
Facial Nerve ◽  
Wegener’S Granulomatosis ◽  
Nerve Palsy ◽  
Facial Nerve Palsy ◽  
Correct Diagnosis ◽  
Cranial Nerves ◽  
Serum Levels ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Wegener's Granulomatosis ◽  
Facial Nerve Decompression

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.

A substrate‐based approach to convert SerpinB1 into a specific inhibitor of proteinase 3, the Wegener's granulomatosis autoantigen

2011 ◽  
Vol 25 (9) ◽  
pp. 3019-3031 ◽  
Author(s):  
Gwenhael Jégot ◽  
Chrystelle Derache ◽  
Sandrine Castella ◽  
Hichem Lahouassa ◽  
Elodie Pitois ◽  
...  
Keyword(s):  
Wegener’S Granulomatosis ◽  
Specific Inhibitor ◽  
Wegener's Granulomatosis ◽  
Proteinase 3

Sinonasal T-cell Lymphoma and Wegener's Granulomatosis: Aspects in Early Differential Diagnosis

1996 ◽  
Vol 10 (4) ◽  
pp. 239-246
Author(s):  
Anders Cervin ◽  
Michael Dictor ◽  
Olof Kalm
Keyword(s):  
Differential Diagnosis ◽  
In Situ Hybridization ◽  
T Cell ◽  
Wegener’S Granulomatosis ◽  
Cell Lymphoma ◽  
Wegener's Granulomatosis ◽  
T Cell Lymphoma ◽  
Upper Airways ◽  
Gastrointestinal Complications

The clinical course of 12 patients with sinonasal T-cell lymphoma retrospectively diagnosed using in situ hybridization for Epstein-Barr virus RNA was compared with that of 10 recently treated patients with Wegener's granulomatosis (WG) in the upper airways. In particular, we studied the presenting signs and symptoms of both diseases, which commonly offer a problem in differential diagnosis at the clinical and pathological level. A bimodal age distribution was suggested in both T-cell lymphoma and WG; five patients with T-cell lymphoma developed disease prior to 40 years of age. Four of the 12 lymphoma patients had a history of “chronic rhinitis” for several years before developing mucosal ulcerations, which were initially unilateral, as opposed to the bilateral ulcerations in early sinonasal WG. Two lymphoma patients had swelling of the nasal dorsum and cheek. In contrast to the WG patients, cases of T-cell lymphoma did not exhibit associated clinical signs of arthritis, conjunctivitis, pulmonary lesions, or nephritis in the early stage of the disease. Nine of the patients with T-cell lymphoma presenting as a sinonasal lesion developed disseminated disease, variably including infiltrates in intestine, lung, CNS, and skin. Four of these patients died from gastrointestinal complications of their disease. We conclude that unilateral ulcerative or hemorrhagic polypoid mucosal lesions in the sinonasal area are suggestive of lymphoma rather than WG, and nonspecific symptoms, at least in Western patients, may be present as early as the second or third decade of life. A biopsy specimen containing T lymphocytes positive for the EBV ribonucleoprotein EBER1 on in situ hybridization offers reliable confirmation of T-cell lymphoma and is of differential diagnostic value against WG.

Wegener's Granulomatosis and Antineutrophil Cytoplasmic Autoantibodies

1993 ◽  
Vol 102 (11) ◽  
pp. 906-908 ◽  
Author(s):  
John G. Batsakis ◽  
Adel K. El-Naggar
Keyword(s):  
Laboratory Test ◽  
Wegener’S Granulomatosis ◽  
Wegener's Granulomatosis ◽  
Proteinase 3 ◽  
Inflammatory Disorders ◽  
Serologic Tests ◽  
Criteria For Diagnosis ◽  
Antineutrophil Cytoplasmic Autoantibodies

Serologic tests for antineutrophil cytoplasmic autoantibodies can serve as markers for a number of necrotizing vasculitides and other inflammatory disorders. In the case of Wegener's granulomatosis, an immunofluorescent cytoplasmic pattern and immunoassay for proteinase 3 are quite specific for the disease and are capable of serving as additional criteria for diagnosis. As with any laboratory test, however, results are to be interpreted in the whole clinical and pathologic framework of the disease.

Idiopathic Midface Lesions

1978 ◽  
Vol 87 (1) ◽  
pp. 60-69 ◽  
Author(s):  
Stephen J. Wetmore ◽  
Charles E. Platz
Keyword(s):  
Wegener’S Granulomatosis ◽  
Correct Diagnosis ◽  
Wegener's Granulomatosis ◽  
Definitive Diagnosis ◽  
Lethal Midline Granuloma ◽  
Multiple Biopsies ◽  
Malignant Reticulosis ◽  
Disease Entities ◽  
Inflammatory Changes ◽  
Midline Granuloma

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.

scholarly journals Wegener's Granulomatosis: Anti–proteinase 3 Antibodies Are Potent Inductors of Human Endothelial Cell Signaling and Leakage Response

1998 ◽  
Vol 187 (4) ◽  
pp. 497-503 ◽  
Author(s):  
Ulf Sibelius ◽  
Katja Hattar ◽  
Angelika Schenkel ◽  
Thomas Noll ◽  
Elena Csernok ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
Wegener’S Granulomatosis ◽  
Inositol Phosphates ◽  
Vascular Endothelial Cells ◽  
Platelet Activating Factor ◽  
Barrier Properties ◽  
Wegener's Granulomatosis ◽  
Phosphoinositide Hydrolysis ◽  
Proteinase 3 ◽  
Dose Dependent

Anti–neutrophil cytoplasmic antibodies (ANCAs) targeting proteinase 3 (PR3) have a high specifity for Wegener's granulomatosis (WG), and their role in activating leukocytes is well appreciated. In this study, we investigated the influence of PR3-ANCA and murine monoclonal antibodies on human umbilical vascular endothelial cells (HUVECs). Priming of HUVECs with tumor necrosis factor α induced endothelial upregulation of PR3 message and surface expression of this antigen, as measured by Cyto-ELISA, with a maximum occurrence after 2 h. Primed cells responded to low concentrations of both antibodies (25 ng–2.5 μg/ml), but not to control immunoglobulins, with pronounced, dose-dependent phosphoinositide hydrolysis, as assessed by accumulation of inositol phosphates. The signaling response peaked after 20 min, in parallel with the appearance of marked prostacyclin and platelet-activating factor synthesis. The F(ab)2 fragment of ANCA was equally potent as ANCA itself. Disrupture of the endothelial F-actin content by botulinum C2 toxin to avoid antigen–antibody internalization did not affect the response. In addition to the metabolic events, anti-PR3 challenge, in the absence of plasma components, provoked delayed, dose-dependent increase in transendothelial protein leakage. We conclude that anti-PR3 antibodies are potent inductors of the preformed phosphoinositide hydrolysis–related signal tranduction pathway in human endothelial cells. Associated metabolic events and the loss of endothelial barrier properties suggest that anti-PR3–induced activation of endothelial cells may contribute to the pathogenetic sequelae of autoimmune vasculitis characterizing WG.

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