Teaching Case

A 51-year-old woman without past medical history referred diffuse arthralgias, myalgia for the past 6 weeks and dyspnoea, dry cough and retrosternal pain for the past 2 days.
Laboratory studies showed raised erythrocyte sedimentation rate (101 mm/h), elevated white blood cell count of 12200/μl, high levels of D-dimers and dysmorphic haematuria and proteinuria.

CT scan of the chest and the abdomen were performed. Also a negative pulmonary scintigraphy was used to exclude the presence of pulmonary embolism. CT scan of the chest demonstrated bilateral “ground glass” opacities and a semilunar hypodense rim around the lumen of the aortic arch and part of the descending aorta. The differential diagnosis of this formation included intramural haematoma and aortitis. Two days later a CT angiography was performed in order to clarify the findings. The diagnosis of aortitis became more probable as a mild enhancement of the semilunar rim was demonstrated. A deterioration of the pulmonary findings with presence of nodular opacities was also noticed (Fig. 1, 2, 3).
Further investigation with specific serologic tests demonstrated that cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCAs) with antiproteinase-3 (anti-PR-3) specificity were present. Renal biopsy was also conducted in order to conclude diagnosis of Wegener’s granulomatosis (WG).

Wegener’s granulomatosis (WG) was first described by Heinz Klinger in 1931. Friedrich Wegener described 3 more cases and discovered it to be a vasculitis. It is an autoimmune systemic necrotising vasculitis of medium-sized and small blood vessels with granulomatous inflammation involving primarily the upper and lower respiratory tracts and kidneys. The presence of antineutrophil cytoplasmic autoantibodies (c-ANCAs) is also common. Large vessel and so aortic involvement is very rare and only a few cases of WG involving major arteries have been reported. Non-specific systemic symptoms and signs like fever, anorexia, weight loss, fatigue, tremors, night sweats, myalgias and arthralgia are common. Sinusitis, nasal crusting, bleeding, obstruction and collapse of the nasal bridge can been seen. It can also cause otitis media and tracheal stenosis. When the lungs are affected, it may present with cough, haemoptysis and dyspnoea. Renal involvement may manifest as haematuria and proteinuria and can lead to renal failure. Aortic involvement is not expected in WG. Chronic inflammation can lead to arterial aneurysm formation, a characteristic of medium-size vessel vasculitis, but a very unusual feature of WG. Aortic involvement is most frequently seen in large vessel vasculitides such as giant cell arteritis and Takayasu’s arteritis. Although less common, (peri)aortitis can be seen occasionally in other vasculitides. According to the ACR classification criteria in 1990, a person is said to have WG if at least two of the following four criteria are present: nasal/oral inflammation; abnormal chest X-ray; urinary sediment (more than 5 erythrocytes per high-power field); granulomatous inflammation on biopsy. Afterwards a fifth criterion was added: haemoptysis. The differential diagnosis of vasculitis with large-vessel involvement includes other vasculitides that overlap with WG, most frequently Takayasu's disease. Glomerulonephritis and c-ANCA are not characteristic of this disorder, and pulmonary involvement is also rare.
Giant cell arteritis of the elderly (temporal arteritis) may involve also the aorta, the coronary arteries, lungs, and kidneys. The young age, the absence of cranial symptoms or polymyalgia rheumatica and the positive c-ANCA speak against this form of vasculitis.
Churg–Strauss vasculitis, another form of granulomatous vasculitis, is very improbable in the absence of marked eosinophilia or allergic manifestations.
Polyarteritis nodosa (PAN) does not involve small vessels or glomeruli and there are no granulomas. Neither saddle-nose deformity nor c-ANCA are features of PAN.
Untreated WG is fatal within 5 years. Immunosuppression and steroid therapy may slow its progression.
WG can be added to the list of systemic vasculitides that can cause (peri)aortitis.