Wegener Granulomatosis presented with epistaxis, hemoptysis and polyarthralgia: A case report
Journal
Medicus
Date Issued
2020
Author(s)
Abstract
Wegener granulomatosis (WG) is a rare, multisystem, autoimmune disease with necrotizing granulomatous
inflammation, tissue necrosis, vasculitis in small and medium-sized blood-vessels. The classic clinical pattern is
a triad involving upper airways, lungs and kidneys. A 33-year-old woman was admitted to hospital because of dry
cough, shortness of breath, polyarthralgia, intermittent fever, epistaxis, hemoptysis. Chest X-ray presented multiple,
small infiltrates bilaterally. Laboratory results: Hgb 90g/L, hematocrit 30%, erythrocytes 3,6×1012/L, leucocytes
13,8×109/L, platelet 4,45×109/L, CRP 110mg/L, sedimentation 70mm/h. Urine sediment: erythrocytes(16-18),
proteins(+), epithelial cells(++), 24hour-proteinuria 0,5g/L. ECG: sinus tachycardia 120 beats/min. Gas analyses:
partial respiratory failure, hypoxemia 7,5kPa, hypocapnia 3,6kPa, saturation 91%. Lung-CT revealed multiple
bilateral infiltrates. Chest-ultrasound with bilateral, subpleural, hypoechogenic changes with central necrosis.
Because of intermittent fever, polyarthralgia, chest X-ray changes, elevated sedimentation, rheumatologist was
consulted and tests for autoimmune disease were performed (positive c-ANCA 95U/ml, RF 158IU/ml, ASO 88U/ml).
Ophthalmologist revealed punctiform conjunctival bleeding. Bronchoscopy: intranasal coagulum without changes
of nasal mucosa, transoral intubation presented diffuse erythema and edema of the vulnerable tracheobronchial
mucosa without ulcerous lesions or infiltrative changes. Bronchial alveolar lavage detected small increase of
neutrophils (total cell counts 320/ L, neutrophils 19,2%, macrophages 85,0%, lymphocytes 7,4%, eosinophils 0,0%),
no growth of bacterial culture, negative Gene X-pert. Transbronchial biopsy was performed, histologic analysis
detected necrotic granulomas with multinucleated giant cells accompanied by inflammatory cells. According to
all investigations, the diagnosis was WG. The patient was successfully treated multidisciplinary with high-dose
steroids and cyclophosphamide. Recognition of multisystem disease involving joints, kidney, eye and lung is critical
for diagnosis.
inflammation, tissue necrosis, vasculitis in small and medium-sized blood-vessels. The classic clinical pattern is
a triad involving upper airways, lungs and kidneys. A 33-year-old woman was admitted to hospital because of dry
cough, shortness of breath, polyarthralgia, intermittent fever, epistaxis, hemoptysis. Chest X-ray presented multiple,
small infiltrates bilaterally. Laboratory results: Hgb 90g/L, hematocrit 30%, erythrocytes 3,6×1012/L, leucocytes
13,8×109/L, platelet 4,45×109/L, CRP 110mg/L, sedimentation 70mm/h. Urine sediment: erythrocytes(16-18),
proteins(+), epithelial cells(++), 24hour-proteinuria 0,5g/L. ECG: sinus tachycardia 120 beats/min. Gas analyses:
partial respiratory failure, hypoxemia 7,5kPa, hypocapnia 3,6kPa, saturation 91%. Lung-CT revealed multiple
bilateral infiltrates. Chest-ultrasound with bilateral, subpleural, hypoechogenic changes with central necrosis.
Because of intermittent fever, polyarthralgia, chest X-ray changes, elevated sedimentation, rheumatologist was
consulted and tests for autoimmune disease were performed (positive c-ANCA 95U/ml, RF 158IU/ml, ASO 88U/ml).
Ophthalmologist revealed punctiform conjunctival bleeding. Bronchoscopy: intranasal coagulum without changes
of nasal mucosa, transoral intubation presented diffuse erythema and edema of the vulnerable tracheobronchial
mucosa without ulcerous lesions or infiltrative changes. Bronchial alveolar lavage detected small increase of
neutrophils (total cell counts 320/ L, neutrophils 19,2%, macrophages 85,0%, lymphocytes 7,4%, eosinophils 0,0%),
no growth of bacterial culture, negative Gene X-pert. Transbronchial biopsy was performed, histologic analysis
detected necrotic granulomas with multinucleated giant cells accompanied by inflammatory cells. According to
all investigations, the diagnosis was WG. The patient was successfully treated multidisciplinary with high-dose
steroids and cyclophosphamide. Recognition of multisystem disease involving joints, kidney, eye and lung is critical
for diagnosis.
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