A case report of Wegener granulomatosis (WG) presenting epistaxis, hemoptysis and polyarthralgia
Date Issued
2020-12
Author(s)
Kuzmanovska Dimitrovska, Melina
Abstract
Background: Wegener granulomatosis (WG) is a rare multisystem autoimmune
disease characterized by necrotizing granulomatous inflammation, tissue necrosis,
and vasculitis in small and medium-sized blood vessels. The classic clinical pattern is a
triad involving the upper airways, lungs and kidneys.
Case presentation: A 33-year-old woman was admitted to our hospital with a history
of progressively worsening dry cough, shortness of breath, polyarthralgia, fever,
epistaxis and hemoptysis. Two months before admission, she had episodes of nasal
bleeding, dry cough, fever not more than 38.2°C. Her primary physician did not detect
any abnormal findings in the chest radiographs at that time. Two months later, she
consulted the doctor again due to the symptoms and because of the chest X-ray with
multiple small infiltrates in both lungs, high sedimentation rate she was admitted to
our hospital. Lungs were clear to auscultation bilaterally. Laboratory results revealed
anemia with Hgb 90g/L, hematocrit 30%, erythrocytes 3600/L, leucocytes 13800/L, CRP
110mg/L, sedimentation rate 70mm/h. Urine sediment – erythrocytes 16-18, proteins +,
epithelial cells ++. 24hour proteinuria 0,5g/L (upper limit 0,2). Rheumatoid antibodies: positive c-ANCA 95U/ml, RF 158IU/ml, ASO 88U/ml. ECG with sinus tachycardia of
120 beats/min. Gas analyses in partial respiratory failure with hypoxemia 7,5kPa and
hypocapnia 3,6kPa, oxygen saturation 91%. Chest radiography and lung CT showed
multiple infiltrates in the bilateral upper lobes. Bronchoscopy finding of intranasal
coagulum without changes of nasal mucosa, transoral intubation revealed diffuse
erythema and edema of the vulnerable tracheobronchial mucosa without any ulcerous
lesions or infiltrative changes. Chest ultrasound with many apical bilateral, subpleural,
hypoechogenic changes with zones of central necrosis with maximal diameter 20mm.
Ophthalmology examination - punctiform conjunctival bleeding. Transbronchial
biopsy was performed and revealed necrotic granulomas with multinucleated giant
cells in the bronchial/bronchiolar and parenchymal lesions. Bronchial alveolar lavage
(BAL) was performed and showed the small increase of neutrophils (total cell counts: 320/μL, neutrophils: 19.2%, macrophages: 85.0%, lymphocytes: 7.4%, eosinophils: 0.0%) and no growth of bacterial culture. According to the results the diagnosis
granulomatosis with polyangiitis, Wegener’s granulomatosis. She was successfully
treated by rheumatologist with high-dose steroids and cyclophosphamide.
Conclusion: The recognition of multisystem disease involving joints, kidney, eye and
lung is critical for diagnosing Wegener's vasculitis.
disease characterized by necrotizing granulomatous inflammation, tissue necrosis,
and vasculitis in small and medium-sized blood vessels. The classic clinical pattern is a
triad involving the upper airways, lungs and kidneys.
Case presentation: A 33-year-old woman was admitted to our hospital with a history
of progressively worsening dry cough, shortness of breath, polyarthralgia, fever,
epistaxis and hemoptysis. Two months before admission, she had episodes of nasal
bleeding, dry cough, fever not more than 38.2°C. Her primary physician did not detect
any abnormal findings in the chest radiographs at that time. Two months later, she
consulted the doctor again due to the symptoms and because of the chest X-ray with
multiple small infiltrates in both lungs, high sedimentation rate she was admitted to
our hospital. Lungs were clear to auscultation bilaterally. Laboratory results revealed
anemia with Hgb 90g/L, hematocrit 30%, erythrocytes 3600/L, leucocytes 13800/L, CRP
110mg/L, sedimentation rate 70mm/h. Urine sediment – erythrocytes 16-18, proteins +,
epithelial cells ++. 24hour proteinuria 0,5g/L (upper limit 0,2). Rheumatoid antibodies: positive c-ANCA 95U/ml, RF 158IU/ml, ASO 88U/ml. ECG with sinus tachycardia of
120 beats/min. Gas analyses in partial respiratory failure with hypoxemia 7,5kPa and
hypocapnia 3,6kPa, oxygen saturation 91%. Chest radiography and lung CT showed
multiple infiltrates in the bilateral upper lobes. Bronchoscopy finding of intranasal
coagulum without changes of nasal mucosa, transoral intubation revealed diffuse
erythema and edema of the vulnerable tracheobronchial mucosa without any ulcerous
lesions or infiltrative changes. Chest ultrasound with many apical bilateral, subpleural,
hypoechogenic changes with zones of central necrosis with maximal diameter 20mm.
Ophthalmology examination - punctiform conjunctival bleeding. Transbronchial
biopsy was performed and revealed necrotic granulomas with multinucleated giant
cells in the bronchial/bronchiolar and parenchymal lesions. Bronchial alveolar lavage
(BAL) was performed and showed the small increase of neutrophils (total cell counts: 320/μL, neutrophils: 19.2%, macrophages: 85.0%, lymphocytes: 7.4%, eosinophils: 0.0%) and no growth of bacterial culture. According to the results the diagnosis
granulomatosis with polyangiitis, Wegener’s granulomatosis. She was successfully
treated by rheumatologist with high-dose steroids and cyclophosphamide.
Conclusion: The recognition of multisystem disease involving joints, kidney, eye and
lung is critical for diagnosing Wegener's vasculitis.
Subjects
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