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Presented by Fred Askin, M.D. and prepared by Carol Allan, M.D.
Case 5: The patient is a 67-year old male who developed fatigue and weight loss approximately 5 months prior to admission.
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1. Question
Week 54: Case 5
The patient is a 67-year old male who developed fatigue and weight loss approximately 5 months prior to admission. A chest radiograph revealed “mass lesions”./images/fa5a.jpg
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Answer: Wegener’s granulomatosis
Histology: The biopsy shows a vasocentric necrotizing process. Associated with vascular necrosis is a distinctive red-purple “necrobiotic” appearance to the lung tissue. No well formed granulomas could be identified. Special stains for acid-fast bacteria, including Auramine-Rhodamine fluorescent stains, were negative, as were GMS stains for fungi. Cultures of the biopsy have been reported negative to date.
Discussion: Classic granulomatous necrotizing vasculitis associated with Wegener’s is actually a relatively late phase of the disease. Early diagnosis depends on recognition of tissue necrobiosis and an acute vasculitis, which may involve medium size (muscular) arteries as seen here or maybe manifest as an alveolar hemorrhage syndrome with leukocytoclastic vasculitis (capalaritis). The differential diagnosis always includes infection and appropriately obtained and processed cultures are a key element in the proper diagnosis. The availability of serum ANCA testing has been a boon for the accurate diagnosis of Wegener’s granulomatosis. The majority of cases of pulmonary Wegener’s are associated with serum antibodies to c-ANCA with a proteinase-3 specificity. A minority of cases will show p-ANCA antibodies with a myoperoxidase specificity. It is important to recognize that non-specific p-ANCA positivity may occur in patients with inflammatory bowel disease and that these patients may also have necrobiotic and/or inflammatory lesions in the lung (Medicine 1993; 72:151). There are some histologic features that will tend to favor granulomatous infection over a diagnosis of Wegener’s granulomatosis. In infection, vasculitis tends to be secondary due to contiguity with necrotic foci and the necrotic zones are often rounded, often eosinophilic and have a relatively thin cuff of inflamed lung tissue. Frequently, sarcoid-type non-necrotizing granulomas are seen away from foci of granulomatous necrosis. In Wegener’s on the other hand, the geographic basophilic necrosis and numerous scattered giant cells are typical. Zones of necrosis are surrounded by extensive consolidated lung tissue that may show a variety of acute, subacute or chronic inflammatory features. As in most pulmonary disease, correlation of the clinical, laboratory and radiographic features with the histologic findings will provide the proper diagnosis.
Incorrect
Answer: Wegener’s granulomatosis
Histology: The biopsy shows a vasocentric necrotizing process. Associated with vascular necrosis is a distinctive red-purple “necrobiotic” appearance to the lung tissue. No well formed granulomas could be identified. Special stains for acid-fast bacteria, including Auramine-Rhodamine fluorescent stains, were negative, as were GMS stains for fungi. Cultures of the biopsy have been reported negative to date.
Discussion: Classic granulomatous necrotizing vasculitis associated with Wegener’s is actually a relatively late phase of the disease. Early diagnosis depends on recognition of tissue necrobiosis and an acute vasculitis, which may involve medium size (muscular) arteries as seen here or maybe manifest as an alveolar hemorrhage syndrome with leukocytoclastic vasculitis (capalaritis). The differential diagnosis always includes infection and appropriately obtained and processed cultures are a key element in the proper diagnosis. The availability of serum ANCA testing has been a boon for the accurate diagnosis of Wegener’s granulomatosis. The majority of cases of pulmonary Wegener’s are associated with serum antibodies to c-ANCA with a proteinase-3 specificity. A minority of cases will show p-ANCA antibodies with a myoperoxidase specificity. It is important to recognize that non-specific p-ANCA positivity may occur in patients with inflammatory bowel disease and that these patients may also have necrobiotic and/or inflammatory lesions in the lung (Medicine 1993; 72:151). There are some histologic features that will tend to favor granulomatous infection over a diagnosis of Wegener’s granulomatosis. In infection, vasculitis tends to be secondary due to contiguity with necrotic foci and the necrotic zones are often rounded, often eosinophilic and have a relatively thin cuff of inflamed lung tissue. Frequently, sarcoid-type non-necrotizing granulomas are seen away from foci of granulomatous necrosis. In Wegener’s on the other hand, the geographic basophilic necrosis and numerous scattered giant cells are typical. Zones of necrosis are surrounded by extensive consolidated lung tissue that may show a variety of acute, subacute or chronic inflammatory features. As in most pulmonary disease, correlation of the clinical, laboratory and radiographic features with the histologic findings will provide the proper diagnosis.
