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Crowned dens syndrome is an under-recognized entity that can mimic neurological disease, in particular meningitis or giant-cell arteritis. We present a 48-year-old woman presenting with an inflammatory meningitis-like syndrome with headache and neck stiffness. Lumbar puncture was normal and computed tomography (CT) of the atlantoaxial joint showed abnormal calcifications around the odontoid process, leading to a tentative diagnosis of crowned dens syndrome. In addition, signs of active inflammation in and around the dens were present on cervical MR imaging. Since CDS can mimic meningitis or giant-cell arteritis, neurologists should be aware of this entity. If CDS is suspected, the bone window on the head CT scan can lead to the diagnosis. On the other hand, asymptomatic periodontoid calcifications are common and should not preclude further investigations.

Imaging procedures have become an important diagnostic tool in vasculitis. In large vessel vasculitides, such as giant-cell arteritis (GCA) and Takayasu arteritis, ultrasound, magnetic resonance imaging (MRI), computed tomography (CT) and 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) can depict specific abnormalities of the arterial wall. A clinically suspected diagnosis can be confirmed by imaging if performed by a trained specialist using appropriate equipment, without histological investigations. Ultrasound, MRI and CT show a homogeneous, concentric thickening of the arterial wall and PET can detect increased glucose metabolism of the arterial wall. Ultrasound is the method of choice, especially in predominantly cranial GCA. Imaging should be performed before or within the first few days of glucocorticoid treatment as the PET findings of all arteries as well as ultrasound and MRI findings in temporal arteries normalize quickly with treatment. A planned imaging examination must not delay initiation of glucocorticoid treatment.

Large-vessel vasculitis (LVV) is a group of diseases mainly comprised of giant-cell arteritis (GCA), Takayasu arteritis, and a series of rare diseases like Behçet's disease, IgG4-related disease, infectious aortitis, and other unfrequent entities. Besides clinical and laboratory features, Doppler sonography (DS) can assist in establishing the diagnosis. Its diagnostic sensitivity has been evaluated in various studies, most of them, however, in temporal arteritis (TA) respectively in LVV with involvement of the temporal artery. Little is known in extracranial LVV. We retrospectively evaluated the diagnostic accuracy of DS in 30 patients with extracranial, non-temporal LVV using the highly sensitive PET/CT as method of reference in comparison to 20 controls who were found to have no LVV. We investigated ten arterial sites and documented the presence of the sonographic halo sign. Sensitivities of DS for LVV were highest in the subclavian and axillary arteries (71.4%/72.2%) and low in the abdominal aorta (26.1%) and the common femoral artery (16.7%). DS detected 24 out of 30 cases of LVV (overall sensitivity 80.0%). The LVV cases where DS was completely negative did not significantly differ in leukocyte count, C-reactive protein, or erythrocyte sedimentation rate from LVV cases with positive DS. DS is a potent method in diagnosing extracranial LVV especially in the axillary and the subclavian arteries. Aortic, intraabdominal, and lower extremity artery manifestations, however, are often missed by DS. A second imaging modality (e.g., PET/CT) is therefore required.

Medium- and large-vessel vasculitides (MVV and LVV, respectively) comprise a heterogeneous group of disorders whose common denominator is the inflammatory involvement of vessels of medium and large size. This disease spectrum includes giant-cell arteritis and Takayasu's arteritis, which typically affect the aorta and its main branches, and Kawasaki's disease and polyarteritis nodosa, which involve medium-sized arteries. Chronic periaortitis, characterized by a perivascular fibro-inflammatory reaction affecting the abdominal aorta and the periaortic tissue, frequently has a systemic distribution, involving other segments of the aorta and its major branches, and could thus be included in this group. Unlike small-vessel vasculitides, MVV and LVV do not cause glomerulonephritis, although glomerular immune-mediated lesions and tubulo-interstitial nephritis occur with varying frequency. However, MVV and LVV can often involve the renal artery and its branches, causing a wide array of lesions that range from renal artery stenosis to intra-renal vasculitis causing renal ischaemia/infarction, microaneurysms and haemorrhage. This review focuses on renal involvement in MVV and LVV and underlines why renal abnormalities in these syndromes should not be overlooked.

Polymyalgia rheumatica (PMR) is an inflammatory rheumatic disorder in elderly people. It is frequently associated with giant-cell arteritis (GCA), although it is still debated if they are the same disease or two distinct pathologies. The etiology of PMR remains challenging, although current knowledge supports the role of both genetic and environmental factors. Among these, there is evidence that infectious agents could trigger the disease's onset in some cases. We describe the peculiar case where PMR occurred in two Italian married and cohabiting couples and provide a review of the literature for similar cases. This is the first reported occurrence of PMR in two conjugal pairs in Italy. Moreover, in the second case, the wife presented PMR associated with GCA. To date, seven cases of PMR and two of GCA in married couples are described in the literature. The occurrence of PMR in both a husband and a wife supports the pathogenetic role of an environmental factor.