Carotid dissection as a manifestation of granulomatosis with polyangiitis
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0028-3886.166541
Source of Support: None, Conflict of Interest: None
A 60-year-old man presented with transient right-sided hemiparesis 1-day prior to admission. He was admitted to the Neurology Ward of Namazi Hospital affiliated to Shiraz University of Medical Sciences. An informed consent was taken from the patient. There was a history of psoriasis in his brother. His general physical and neurological examination revealed mild hypertension (140/90 mmHg) at the time of admission with right mild hemiparesis (4+ out of 5 on the Medical Research Council scale for both upper and lower extremities) without hyperreflexia.
The laboratory results including hematological assessment, viral assays and lipid profile, brain computed tomographic (CT) scan, and transthoracic echocardiography were all normal. Color Doppler sonography revealed a significantly reduced flow (16 cm/s) in the left internal carotid artery (LICA) suggestive of LICA occlusion. His CT angiography showed a long string pattern followed by occlusion of the distal part of LICA [Figure 1]. The patient was discharged without any change in the grade of weakness detected at admission and was placed on clopidogrel, atorvastatin, acetylsalicylic acid, losartan, and ranitidine. The weakness improved completely after one month of physiotherapy.
Two years later, he presented with a one month history of hoarseness, left sided nasal congestion, hemoptysis, weight loss (3 kg in 1month), fever, and anorexia. The results of his laboratory tests showed an erythrocyte sedimentation rate of 110mm in the first hour, C-reactive protein two times the normal value, cytoplasmic antineutrophil cytoplasmic antibodies (C-ANCA) 123.1 (normal<5), lupus anticoagulant 46.5 s (normal<45 s.), anti-cyclic citrullinated protein (1.1 U/ml) (normal<1 U/ml), and microscopic hematuria (2+ blood).
His kidney sonography yielded normal results. The high resolution CT showed a mosaic pattern in the anterior aspect of both lungs suggestive of air trapping and small airway disease. CT scan of the paranasal sinuses revealed pan sinusitis and obstruction of bilateral osteomeatal complexes. The patients refused to undergo renal and sinus biopsy. The patient was given azithromycin and methyl prednisolone.
He was discharged with prednisolone, folic acid, alendronate, methotrexate, and cotrimoxazole.
After a month on the medications, the patient's symptoms were relieved. His laboratory tests and Doppler sonography of cervical arteries were all normal. The repeat C-ANCA value was 10.4. He has been in complete remission for 2.5 years with the medications that were prescribed at discharge, still being continued.
To the best of our knowledge, this is the first report of carotid dissection as the initial manifestation of granulomatosis with polyangiitis (GPA). The patient fulfilled two of the four American College of Rheumatology criteria for GPA. GPA is a vasculitis of small- and medium-sized vessels. Kidneys, upper respiratory tract, lungs, joints, and skin are the major sites of involvement.
Dissection of the extracranial portion of ICA is a well-known cause of stroke. Arterial dissection accounts for up to 2.5% of patients with stroke. Although trauma is often the triggering event, spontaneous dissection has also been reported in the patients with collagen and elastic fiber synthesis defects.
The association of extracranial carotid artery dissection with a vasculitides syndrome has been extensively reviewed in the available literature. The association of GPA and carotid dissection can be justified by the inflammatory granulomatous process occurring within the media and adventitia of the carotid vessels. The involvement of kidney may have resulted in systemic hypertension. Above all, the involvement of the carotid vessel vasa vasorum may be responsible for the carotid dissection.
The association of carotid dissection with other autoimmune diseases like Behcet's disease and Takayasu's arteritis have been previously reported. While Takayasu's disease mostly affects medium-to-large sized vessels, Behcet's disease mainly involves venules and rarely affects large arteries. Carotid dissection, being a large vessel involvement, does not solely occur in vasculitides with large arterial involvement. Several cases have been reported where the large vessel involvement also occured in diseases with an elevated anti-neutrophil cytoplasmic antibody (ANCA) [that is usually associated with small vessel vasculitis]. Furthermore, abdominal periaortitis and intramural dissection have already been reported with GPA. Therefore, the etiology of carotid artery involvement in this patient may also be possibly attributed to the preexisting GPA.
The main limitation of this report was the lack of tissue biopsy, which may have helped in confirming the association between GPA and carotid arteritis. However, as the patient had pre-existing GPA, it is proposed that the association of carotid dissection in our patient was more than just a coincidental finding and was, in fact, a manifestation of the preexisting vasculitides syndrome.
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