HPE Autoimmune disease: The fundamentals | Página 14

Age is a major risk factor and GCA should be considered in the differential diagnosis of a new-onset headache in those aged 50 or older , especially in presence of raised inflammatory markers ( i . e ., ESR or C-reactive protein ). The gold standard for diagnosis is temporal artery biopsy , with US increasingly used as an alternative . 25

Giant cell arteritis Giant cell arteritis ( also known as temporal arteritis ) is a systemic inflammatory large / medium vessel vasculitis . Headache , jaw claudication and scalp tenderness are important diagnostic symptoms . Blindness is the feared consequence of untreated disease . It can affect the aorta but especially involves the cranial branches of the carotid arteries . 24 Raised inflammatory markers and systemic symptoms are common ( including fever , anorexia , and fatigue ). It is the most common idiopathic systemic vasculitis . 24
It has been estimated that the lifetime risk of developing GCA is 1 % in women and 0.5 % in men . 24 The prevalence of this condition is variable and it has been reported highest in Scandinavia .
GCA has a complex pathophysiology . There is a predominance of CD4 + T lymphocytes and the inflammatory response involves activation of dendritic cells to strongly express MHC class II antigens and CD83 and 86 costimulatory molecules . 25
ANCA-associated vasculitis The anti-neutrophil cytoplasmic antibody ( ANCA ) -associated vasculitides represent a group of disorders involving severe , systemic , small-vessel vasculitis . These conditions are characterised by the development of autoantibodies to the neutrophil proteins , leukocyte proteinase 3 ( PR3-ANCA ) or myeloperoxidase ( MPO-ANCA ). 26
ANCA-associated vasculitis is estimated to affect 20 – 25 people per million per year . 26 The prevalence is estimated to be 0.0046 – 0.0184 %. 27
Diagnosis of ANCA-associated vasculitis can be difficult because there are no hallmark features of the disease . Usually biopsy of the skin , sural nerve or kidney is required for the diagnosis . The condition can present with non-specific symptoms suggestive of chronic inflammatory disease ( fatigue , weight loss , fever , night sweats , myalgia , or polyarthralgia ) or with specific features of end-organ involvement . It can affect multiple body systems with the respiratory system , ear nose and throat , and kidneys being among the most commonly affected . 26
Conclusions The index of suspicion for the presence of an auto-immune related rheumatological disorder should be raised particularly when patients present with symptoms of pain , swelling and joint stiffness and for which there is no obvious cause . Differentiating between the different conditions can be challenging and worsened by the absence , in some cases , of specific serum biomarkers . Nonetheless , given the hugely negative impact upon a patient ’ s quality-oflife and wellbeing , it is vital to undertake any relevant examination and testing to narrow the diagnostic field and ensure that patients are initiated on appropriate therapy .
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conditions / systemic-sclerosisscleroderma /#:~: text = Systemic % 20 sclerosis % 20can % 20cause % 20the % 20 tissues % 20around % 20joints % 20to % 20 stiffen , a % 20symptom % 20of % 20systemic % 20sclerosis ( accessed March 2023 ). 24 Salvarani C , Muratore F . Clinical manifestations of giant cell arteritis . UpToDate May 2022 . www . uptodate . com / contents / clinicalmanifestations-of-giant-cell-arteritis / print #:~: text = Epidemiology % 20Giant % 20 cell % 20arteritis % 20 ( GCA , percent % 20 in % 20men % 20 % 5B2 % 5D ( accessed March 2023 ). 25 Silva RC et al . Clinical manifestations of giant cell arteritis . 2021 . www . intechopen . com / chapters / 75468 ( accessed March 2023 ) 26 Hunter RW et al . ANCA associated vasculitis BMJ 2020 ; 369 : m1070 . 27 Berti A et al . The Epidemiology of ANCA Associated Vasculitis in the U . S .: A 20 Year Population Based Study [ abstract ]. Arthritis Rheumatol 2017 ; 69 ( suppl 10 ).
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