Large vessel vasculitis is rather rare in everyday practice - but early diagnosis has a decisive influence on the prognosis of those affected. With the available diagnostics, the disease can now be detected quickly and reliably, but it is still often diagnosed late. Now, the German Society for Rheumatology (Deutsche Gesellschaft für Rheumatologie, DGRh), with the participation of numerous other specialist societies, has issued its recommendations for the first time.
Giant cell arteritis (GCA) from the group of large vessel vasculitides is one of the most common subtypes of these rheumatological vascular diseases in Europe. The aetiology of the autoimmune disease is still not fully understood. The disease typically involves large-cell, necrotising granulomatous inflammation of the vessel walls of large and medium-sized arteries. Vasculitis manifests itself particularly frequently in branches of the external carotid artery and the temporal artery. Overall, older people over 50 years of age are most affected - women more often than men.
The systemic inflammatory reaction increases the risk of vascular occlusion and aneurysms - possible consequences are serious complications up to blindness or cerebral ischaemia. Early diagnosis and the initiation of appropriate therapy are decisive for the success of treatment.
Whenever GCA is suspected, immediate presentation of the patient to a specialised centre for further diagnosis is recommended to reduce the risk of sudden blindness due to anterior ischaemic optic neuropathy (AION) or other sequelae. Persistent visual loss, including complete blindness, remains one of the most common complications in GCA patients today, with figures in the literature ranging from 6% to 37% of cases. Typical symptoms that should be considered as GCA include:
To date, no specific laboratory parameters are available for GCA diagnosis. As a rule, the non-specific inflammatory parameters (CRP, ESR) are elevated in affected persons. If this is the case, the suspected diagnosis should be confirmed by imaging procedures or histopathology. The standard procedure for GCA with predominant cranial involvement is temporal artery biopsy. Non-invasive methods using sonography or cross-sectional imaging are also becoming increasingly important. However, depending on whether glucocorticoid (GC) therapy has already been started in cases of reasonable suspicion, these methods lose their sensitivity within a few days and should therefore be performed as soon as possible.
The type and dosage of therapy depends on the severity and symptoms of GCA and the individual risk profile of the patient. In GCA without visual disturbances, initiation of GC therapy is recommended, initially with 40 to 60 mg of a prednisolone equivalent daily. Doses higher than 60 mg were not shown to be more effective and only increased the risk of GC-induced complications.
After remission is achieved, the dose is gradually reduced under strict clinical and laboratory control. The desired target dose is 10 to 15 mg daily after 3 months and <5 mg daily after one year. Adapted to the individual situation, the lowest effective GC dose should always be aimed for in order to minimise side effects and the risk of GC-induced complications as much as possible. After more than one year of sustained remission, complete discontinuation of CG can be considered.
This is not the case in GCA with acute visual loss or amaurosis fugax - in this case, higher-dose intravenous GC pulse therapy with 500 to 1000 mg methylprednisolone should be given immediately for three to five days. The same applies to recurrences, although here it may also be increased to the previous dose level depending on the severity.
If GC therapy is not effective due to various factors (previous illnesses, presence of or increased risk of GC-associated sequelae) or especially in the case of refractory or relapsing disease, GC-sparing therapy with the combined administration of tocilizumab (or methotrexate) can be considered. This enables a faster GC reduction compared to monotherapy. With this form of treatment, too, tapering is recommended after sustained remission.
Regular clinical and laboratory monitoring is recommended for all GCA patients, regardless of the type of therapy. If vascular complications occur, immediate referral to specialised centres or interdisciplinary vascular teams should be made. For all necessary endovascular and surgical interventions, the following applies: If possible, in remission phases of the disease and individually after interdisciplinary coordination.
The present S2k guideline contains for the first time collected specific recommendations of the German Society of Rheumatology with the participation of numerous other specialist societies from Germany, Austria and Switzerland on diagnosis, therapy and follow-up, or monitoring of patients with large-vessel vasculitis.
Reference:
Schirmer JH, Aries PM, Balzer K, Berlit P, Bley TA, Buttgereit F, u. a. S2k-Leitlinie: Management der Großgefäßvaskulitiden. Z Für Rheumatol. November 2020;79(S3):67–95.