Tuberc Respir Dis.  2007 Dec;63(6):531-536.

A Case of Wegener's Granulomatosis with Delayed Diagnosis due to Clinical Features of Henoch-Schonlein Purpura

• Song SB
• Choi HS
• Kim YH
• Choi CW
• Park MJ
• Yoo JH
• Kang HM
• Kim YH
• Par JC

Abstract

Wegener's granulomatosis is a systemic vasculitis of the medium and small arteries, as well as of the venules, arterioles, and occasionally large arteries, and primarily involves the upper and lower respiratory tracts and the kidneys. Renal symptoms of Wegener's granulomatosis are indistinguishable from those of vasculitis such as Henoch-Schonlein purpura and microscopic polyangiitis. This case, though initially diagnosed as Henoch-Schonlein purpura, was confirmed as Wegener's granulomatosis from a lung biopsy fifteen years after the initial diagnosis. We report this case with a review of the literature.

Keyword

Wegener's granulomatosis; Henoch-Schonlein purpura

MeSH Terms

Arteries
Arterioles
Biopsy
Delayed Diagnosis*
Diagnosis
Kidney
Lung
Microscopic Polyangiitis
Purpura, Schoenlein-Henoch*
Respiratory System
Systemic Vasculitis
Vasculitis
Venules
Wegener Granulomatosis*

Figure

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  Figure 1 On patient's facial picture, a saddle nose is observed.

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  Figure 2 Chest radiography shows cavitary lung mass at the right upper lung field.

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  Figure 3 Chest CT scans show an irregular shaped mass consisted of various size of cavities with surrounding fibrosis and speckled margins (A; mediastinal setting view, B; lung setting view).

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  Figure 4 Facial CT shows small fluid density and thickening of bony wall in the right and left maxillary sinuses. Axial (A) and coronal (B) view.

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  Figure 5 Cut surface shows a rather ill-defined mass, which is consisted of varying sized multiple cyst with dense fibrous walls in the middle lobe, measuring 5×4 cm in size.

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  Figure 6 On microscopic examination, the granulomatous necrotizing vasculitis of large arteries and veins (A; H&E stain, ×200), surrounded by irregular shaped fibrinous necrosis and histiocytes and multinucleated giant cells (B; H&E stain, ×400).

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