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Year : 2003  |  Volume : 13  |  Issue : 2  |  Page : 151-153
Images : Wegener's granulomatosis


Department of radiology, Amrita Institute of Medical Sciences and Research Centre, Amrita Lane, Elamakkara, P.O. Kochi, Kerala-682026, India

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Keywords: Wegener′s Granulomatosis, Vasculitis

How to cite this article:
Kumar S N, Sreekumar K P, Prabhu N K, Moorthy S. Images : Wegener's granulomatosis. Indian J Radiol Imaging 2003;13:151-3

How to cite this URL:
Kumar S N, Sreekumar K P, Prabhu N K, Moorthy S. Images : Wegener's granulomatosis. Indian J Radiol Imaging [serial online] 2003 [cited 2014 Aug 21];13:151-3. Available from: http://www.ijri.org/text.asp?2003/13/2/151/28649
Wegener's Granulomatosis is an idiopathic necrotizing systemic vasculitis characterized by inflammation of the upper and lower respiratory tracts and often associated with glomerulonephritis. Estimated annual incidence is 3 to 8.5 cases per million population. Peak incidence is in the fourth and fifth decades. It also has been reported in children as young as three months. Men are slightly more commonly affected than women


   Case report Top


A twenty seven year old man presented with fever, rhinorrhoea, joint pain, mild abdominal pain of one month duration and one episode of epistaxis. There was no past history of tuberculosis or diabetes mellitus.

Physical examination showed hyper-pigmented rashes on the dorsum of the right foot with weak dorsalis pedis and posterial tibial pulses on right side.

Hematological investigations showed elevated ESR (48mm/Hr),and neutrophilia (84.6%).

Liver function test showed elevated AST and ALT. Serum ANA test was negative but test for C-ANCA was positive. Anti ds-DNA was negative. Urine examination showed moderate haematuria, occasional granular casts and elevated 24 hour protein.

Chest radiograph showed bilateral upper zone nodular opacities. In the left lower zone, para cardiac region, there was an area of consolidation with cavitation [Figure - 1]. Radiographs of para nasal sinuses were normal.

CT scan of chest showed multiple, predominantly peripheral, nodular opacities. The largest lesion in the lingula and posterior basal segment of lower lobe showed cavitation [Figure - 2].

Contrast enhanced CT scan of abdomen showed hypo density of spleen with contrast enhancement only in the parenchyma adjacent to hilar region, suggestive of infarction [Figure - 3]. Both kidneys also showed multiple wedge shaped hypo densities with no contrast enhancement suggestive of renal infarcts [Figure - 4].

FNAC from pulmonary lesions showed granulomatous inflammation.


   Discussion Top


Pathologically Wegener's Granulomatosis is characterized by three features:

  1. Necrotizing granulomatous inflammation of the upper and lower respiratory tracts.
  2. Disseminated small vessel necrotizing vasculitis that affects both arteries and veins.
  3. Focal necrotizing glomerulonephritis.


Onset is usually acute but it may be subacute or even indolent. At presentation, upper airway involvement with sinusitis, rhinitis and otitis are the most common clinical features.

Diagnostic criteria for Wegener's Granulomatosis are, 1) Abnormal urine sediments, like red blood cells or casts, 2) An abnormal chest radiograph. 3) Oral ulcers or nasal discharge and 4) granulomatous inflammation on biopsy.

Pathogenesis is believed to be a hypersensitivity reaction to infection with staphylococcus aureus Scientific Name Search  and parvovirus B-19 [1]

Radiographic changes in the lungs occur at presentation in 50%-75% of patients. Most characteristic pulmonary finding is discrete focal opacities that vary in character from nodular masses to ill-defined areas of consolidation, both of which may cavitate. The nodules may be single or multiple [2] and may range from 3 to 10 centimeters and may be confluent [3].

The nodules are usually less than ten in number but may be innumerable. Some times there is a mixed pattern of nodules and consolidation. The nodules may resolve with or without treatment. Cavitation of the nodules and consolidations are seen in approximately 40% of cases at presentation. The cavitation is related to the size of the lesions. The cavities may be unilocular or multilocular and the outer margins of their walls are more commonly irregular than smooth. Typically it is thick walled but in chronic cases there is a tendency for the walls to become thinner. Cavities may show air fluid levels indicating secondary infection. The focal consolidations are commonly pleural based and have a wedge shaped configuration with or without air bronchogram. They have a pleural based tag in most cases. Diffuse bilateral consolidation can occur due to pulmonary hemorrhages. Hilar and mediastinal adenopathy have been detected in up to 2% of cases [4]. Pleural effusion can occur and varies from mild to severe [5]. Pneumothorax and hydropneumothorax are occasionally seen.

Peribronchovascular thickening also has been reported. Tracheal narrowing is a relatively common manifestation of Wegener's Granulomatosis. Tracheal stenosis is usually subglottic. CT scan shows abnormal soft tissue within tracheal rings.

Involvement of kidneys is characterized by focal or segmental necrotising glomerulonephritis or rapidly progressive glomerulonephritis. Infarcts due to vasculitis also occur.

Spleen shows enlargement and splenic infarcts are seen in less than 10% of cases.

Nasal lesions include a saddle nose deformity, septal perforation, chondritis, nasal crusting and epistaxis. Para nasal sinuses show mucosal thickening, pan sinusitis and bone destruction. Serous otitis, media sensory neural deafness and mastoiditis can occur. Skin is also involved in some cases. Urticaria, papules, vesicles, erythema, petechiae, pyoderma gangrenosum and palpable purpura are the common manifestations. In the eye, conjunctivitis, episcleritis, sclerocorneal ulcerations, uveitis, retinal vasculitis, central retinal artery occlusion, opthalmoplegia, optic neuropathy, orbital pseudotumour, dacryocystis, and lacrimal duct stenosis are reported in upto 40-70% of cases. Neurological conditions like multiple mononeuropathy, peripheral neuropathy, cranial nerve palsies, cerebral infarcts, transverse myelitis and seizures have been reported in 33% cases. Arthralgia and arthritis of small and large joints are seen in 30% of cases.

Our case had a typical clinical presentation, laboratory features and radiological findings in the chest. The splenic infarcts and renal infarcts were unusual features.

 
   References Top

1.Leavitt RY, Fauci AS, Pulmonary vasculitis. Am Rev Respir Dis 1986;134: 149-166.  Back to cited text no. 1    
2.Landman S, Burgener F, Pulmonary manifestation in Wegener's Granulamatosis. AJR 1974;122: 750-757.  Back to cited text no. 2    
3.McGregor MBB, Sandler G, Wegener's Granulamatosis. A clinical and radiological survey. Br J Radiol 1964; 37: 430-439.  Back to cited text no. 3    
4.George TM, Cash JM, Farver C etal, Mediastinal mass and hilar adenopathy. Rare thoracic manifestation of Wegener's granulomatosis. Arthritis Rheum 1997; 40:1992-1997.  Back to cited text no. 4    
5.Gohel VK, Dalnka MK, Israel HL etal, The radiological manifestations of Wegeners granulomatosis. Br J Radiology 1973; l46: 427-432.  Back to cited text no. 5    

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Correspondence Address:
S N Kumar
Department of radiology, Amrita Institute of Medical Sciences and Research Centre, Amrita Lane, Elamakkara, P.O. Kochi, Kerala-682026
India
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    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]



 

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