**Author details**

N. Lukán

4th Internal Department Medical Faculty, Safarik University, Košice, Slovak Republic

### **References**


pathogenesis clearly indicates the need of an interdisciplinary team work incorporating intensive care specialists and immunologists as well. This integrative approach could pave the way toward the introduction of more efficient novel treatment regimes. Another challenge is the high risk of relapses in these condition occurring up to 50 %, of the patients. Early establishment of the exact diagnosis and effective etiology oriented treatment in such cases is rather difficult task requiring further experimental and clinical research and cooperation of

Perspective therapeutic approaches based on contemporary immunological knowledge (B-cell depletion, costimulatory molecule blockers, siRNAs controlling intracellular processes, cytokine treatment) supported by clinical experience will bring benefits for induction and maintenance of remission or also excluding the menacing catastrophic scenario of the disease.

ANCA – anti-neutrophil cytoplasmic antibody, APS – antiphospholipid syndrome, CSS – Churg-Strauss syndrome, DAH – diffuse alveolar hemorrhage, GBM – glomerular basement membrane, GN - glomerulonephritis, GPA – granulomatosis with polyangiitis, GPS – Goodpasture´s syndrome, ICU – intensive care unit, IVIG – intravenous immunoglobulin, MCTD – mixed connective tissue disease, MPA – microscopic polyangiitis, MPO myeloperoxidase, NAC – N-acetylcystein, NO –nitric oxide, eNOS – endothelial nitric oxide synthase, iNOS – inducible nitric oxide synthase, PRS – pulmonary-renal syndrome, RA – rheumatoid arthritis, siRNA - small interfering ribonucleic acids, ROS – reactive oxygen

species, SLE – systemic lupus erythematosus, WG – Wegener´s granulomatosis

4th Internal Department Medical Faculty, Safarik University, Košice, Slovak Republic

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**Abbreviations**

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N. Lukán

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**Chapter 4**

**Immunopathophysiology of Large Vessel Involvement**

Giant cell arteritis (GCA) or temporal arteritis or Horton's disease is classified amongst the primary large-vessel vasculitides, according to the 2012 revision of the Chapel-Hill classifica‐ tion criteria. The disease develops almost exclusively in patients older than 50 years (preva‐ lence of 1 in 500 individuals in this age spectrum) and represents the most common vasculitis in Western countries. [1] Incidence rates are progressively increased and estimated to range between 10-30 new cases per 100000 persons beyond the age of 50, while the highest frequency

The disease affects, mainly, the large- and medium-sized extracranial branches of the carotid artery and, classical clinical features, such as headache, jaw claudication, scalp tenderness and

On a histopathological basis, GCA involves all layers of the arterial wall, including the adventitia. Inflammatory lesions consist of activated T cells, dendritic cells (DCs) and macro‐ phages. These lesions are believed to be the histopathologic hallmark of GCA and are charac‐ terized by a predominance of mononuclear infiltrates or granulomas, usually with

Besides the inflammation of the carotid branches, involvement of the great arteries, such as the aorta and its main tributaries, was initially recognized in the late 1930s and reported sporadically thereafter in necropsy or histopathologic studies of surgically resected tissues. [5,

and reproduction in any medium, provided the original work is properly cited.

© 2013 Boura et al.; licensee InTech. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use,

© 2013 The Author(s). Licensee InTech. This chapter is distributed under the terms of the Creative Commons Attribution License http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution,

distribution, and reproduction in any medium, provided the original work is properly cited.

visual impairment, are closely related to this marked cranial tropism of GCA. [3]

**in Giant Cell Arteritis — Implications on Disease**

**Phenotype and Response to Treatment**

is reported in Scandinavian and North American populations. [2]

Panagiota Boura, Konstantinos Tselios,

Additional information is available at the end of the chapter

Ioannis Gkougkourelas and Alexandros Sarantopoulos

http://dx.doi.org/10.5772/55222

multinucleated giant cells. [4]

**1. Introduction**

