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| Discussion |
Case Report:
A 36-years old male presented with recurrent sinusitis, nasal discharge, headache, pain in nose and occasional episodes of epistaxis. The patient had been treated conservatively for sinusitis by antihistamics; but, he did not responded.
Gradually, the patient developed left sided facial paresis which progressed to bilateral facial paralysis. The patient also started complaining of otalgia and decreased hearing bilaterally.
Axial and coronal CT scans of the paranasal sinuses was performed.
Axial CT scan in soft tissue window showed opacification of both maxillary sinuses and the right half of the sphenoid sinus
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Fig. 1 |
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Axial CT scan in bone window demonstrates sclerosis of the walls of the maxillary sinuses bilaterally, of the sphenoid sinus and the base of the skull and mastoid air cells.
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Coronal CT section in bone tissue window, in addition, demonstrates sclerosis of the of the alveolar ridge, sphenoid sinus and the base of the skull. The vertical limbs of facial canal are sclerosed bilaterally and there is mastioditis.
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A diagnosis of chronic inflammatory disease involving the maxillary and sphenoid sinuses and the mastoid was made.
A biopsy of the nasal mucosa performed at another center revealed nonspecific inflammation. The patient was given a trial of antituberculous drugs but with no benefit.
Five months latter, the patient developed diplopia and decreased vision in the right eye.
Laboratory investigations revealed normocytic normochromic anemia [Hb10g/l], normal levels of platelets and WBC, The erythrocyte sedimentation rate was elevated to 65mm at the end of the first hour. The c-reactive protein levels were elevated.. Blood urea, serum creatinine, serum electrolytes were normal.
A repeat CT was performed at this time and this showed a reteroconal soft tissue lesion in the left orbit displacing medial and inferior recti muscle.
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MRI
T2 axial image showed an abnormal T2 intermediate signal intensity soft tissue lesion in the reteroconal region of the left orbit encasing the optic nerve and not separate from the medial and the inferior recti muscle.
There is hyperintense signal in the bilateral mastoid air cells
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Fig. 12 |
T1 axial images
Mucosal hypertrophy in both maxillary sinuses and the right sphenoid sinus is seen. An abnormal T1 hyperintense signal intensity soft tissue mass is seen in the reteroconal region of the left orbit .
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On T1 trans post-contrast fat sat images, the lesion shows post contrast enhancement and maxillary sinusitis.
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T2 trans post-contrast fat sat images demonstrates abnormal hyperintense signal in both maxillary sinuses and in the mastoid region.
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A repeat biopsy was performed and biopsy showed leukocytoclastic vasculitis with necrotic changes and granulomatous inflammation .
In view of the above findings of CT and MRI and histopathological report, the patient was further worked up for vasculitis and the c- ANCA was positive.
There was no history of chest pain ,breathlessness, hemoptysis.
Chest x-ray of the patient was normal.
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Fig. 21 |
Urinary investigations where with in normal limits, no microhematuria or red cell casts. There was no involvent of skin or joints. Ad iagnosis of limited Wegerner's granulomatosis was made.
The patient was started on immunosuppressive drug cyclophosphamide and he responded well to the treatment.
DIAGNOSIS
LIMITED WEGENER’S GRANULOMATOSIS
Wegener granulomatosis (WG) is a rare multisystem disease characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tracts and kidneys and by necrotizing vasculitis affecting small- and medium-sized vessels. In the limited form of the disease there is no renal involvement , takes a milder course, and has a better prognosis. However, it could progress to the classic form.
Pathophysiology
Inflammation with granuloma formation against a nonspecific inflammatory background is the classical tissue abnormality in all organs affected by Wegener's granulomatosis. It is now widely presumed that the anti-neutrophil cytoplasmic antibodies (ANCAs) are responsible for the inflammation in Wegener's
Initial symptoms are nonspecific. Rhinitis is generally the first sign in most patients.
· Trachea: subglottal stenosis
· Lungs: pulmonary nodules (referred to as "coin lesions"), infiltrates (often interpreted as pneumonia), cavitary lesions, pulmonary hemorrhage causing hemoptysis, and rarely bronchial stenosis.
· Kidney: rapidly progressive segmental necrotising glomerulonephritis (75%), leading to chronic renal failure
· Arthritis: Pain or swelling (60%), often initially diagnosed as rheumatoid arthritis.
· Skin: nodules on the elbow, purpura, various others
It is a rare disease that typically occurs in patients in their fourth to sixth decades, although it has been reported in patients with age range from 7 to 75. 1 There is a slight male preponderance with a male to female ratio of up to 1.5 to 1.
Wegener's granulomatosis is usually suspected only when a patient has had unexplained symptoms for a long period of time. Determination of ANCAs can aid in the diagnosis, but positivity is not conclusive and negative ANCAs are not sufficient to reject the diagnosis.
Cytoplasmic staining ANCAs that react with the enzyme proteinase 3 (cANCA) in neutrophils (a type of white blood cell) are associated with Wegener's
If the patient has renal failure or cutaneous vasculitis, these are the most logical organs to obtain a biopsy from. Rarely, thoracoscopic lung biopsy is required.
On histopathological examination, a biopsy will show leukocytoclastic vasculitis with necrotic changes and granulomatous inflammation (clumps of typically arranged white blood cells) on microscopy.
In 1990, the American College of Rheumatology accepted classification criteria for Wegener's. Two or more positive criteria have a sensitivity of 88.2% and a specificity of 92.0% of describing Wegener's.
TREATMENT
Initial treatment is generally with Corticosteroid and oral cyclophosphamide(CYC). Once remission is attained (normally 3 to 6 months), treatment is frequently changed to azathioprine or methotrexate which are less toxic drugs. Total duration of therapy should be at least one year, or longer in high risk patients. Corticosteroids are tapered to a low maintenance dose, 5–10 mg/day. Plasmapheresis may be beneficial in severe disease or pulmonary hemorrhage. The limited form runs a milder course and responds well to treatment.
In severe disease not responsive to treatment mycophenolate mofetil , 15-deoxyspergualin ,anti-thymocyte globulin ,rituximab and infliximab may be tried.
Follow-up:
General well-being and laboratory organ markers are checked on a regular basis to ascertain the patient has remained in remiss