HPE Autoimmune disease: The fundamentals | Page 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 .
References 1 Moutsopoulos HM . Autoimmune rheumatic diseases : One or many diseases ? J Transl Autoimmun 2021 ; 4:100129 . 2 Goodnow CC . Multistep pathogenesis of autoimmune disease . Cell 2007 ; 130 ( 1 ): 25 – 35 . 3 McGonagle D , McDermott MF . A Proposed Classification of the Immunological Diseases . PLOS Medicine 2006 ; 3 ( 8 ): e297 4 National Institute for Health and Care Excellence ( NICE ). Rheumatoid arthritis ( Revised April 2020 ). https :// cks . nice . org . uk / topics / rheumatoid-arthritis / ( accessed March 2023 ) 5 Smith HR et al . Rheumatoid Arthritis ( RA ). Medscape Jan 2022 . https :// emedicine . medscape . com / article / 331715-overview ( accessed March 2023 ). 6 Smolen J et al . Rheumatoid arthritis . Nat Rev Dis Primers 2018 ; 4:18001 . 7 NICE . Ankylosing spondylitis . Revised May 2019 . https :// cks . nice . org . uk / topics / ankylosing-spondylitis / ( accessed March 2023 ). 8 Farouk HM , Abdel-Rahman MA , Hassan RM . Relationship between
smoking , clinical , inflammatory , and radiographic parameters in patients with ankylosing spondylitis . Egypt Rheumatol Rehabil 2021 ; 48:26 . 9 Al Hammadi A . Psoriatic arthritis . Medcape Jan 2022 . https :// emedicine . medscape . com / article / 2196539-overview ( accessed March 2023 ). 10 Moll JMH , Wright V . Psoriatic arthritis . Semin Arthritis Rheum 1973 ; 55 – 78 . 11 Web MD . August 2022 . www . webmd . com / arthritis / psoriatic-arthritis / psoriatic-arthritis-the-basics ( accessed March 2023 ). 12 Petty RE et al . International League of Associations for Rheumatology classification of juvenile idiopathic arthritis : Second revision , Edmonton , 2001 . J Rheumatol 2004 ; 31:390 – 2 . 13 Martini A et al . Toward new classification criteria for juvenile idiopathic arthritis : First steps , pediatric rheumatology international trials organization international consensus . J Rheumatol 2018 ; 46:190 – 7 . 14 Zaripova LN et al . Juvenile idiopathic arthritis : from aetiopathogenesis to therapeutic approaches . Pediatr Rheumatol 2021 ; 19:35 . 15 Jacobson JL , Pham JT . Juvenile
idiopathic arthritis : A focus on pharmacologic management . J . Pediatr Health Care 2018 ; 32:515 – 28 . 16 Gurion R , Lehman TJA , Moorthy LN . Systemic arthritis in children : A review of clinical presentation and treatment . Int J Inflamm 2011 ; 2012:1 – 16 . 17 Okamoto N et al . Clinical practice guidance for juvenile idiopathic arthritis ( JIA ) 2018 . Mod Rheumatol 2019 ; 29:41 – 59 . 18 Barut K et al . Juvenile Idiopathic Arthritis . Balk Med J 2017 ; 34:90 – 101 . 19 Malattia C , Martini A . Juvenile Idiopathic Arthritis , The Autoimmune Diseases , 5th ed .; Elsevier Inc .: Amsterdam , The Netherlands , 2013 ; 525 – 36 . 20 Kaul A et al . Systemic lupus erythematosus . Nat Rev Dis Primers 2016 ; 16039 . 21 Negrini S et al . Sjögren ’ s syndrome : a systemic autoimmune disease . Clin Exp Med 2011 ; 22:9 – 25 . 22 Jimenez SA . Scleroderma . Medscape Feb 2022 . https :// emedicine . medscape . com / article / 331864-overview # a2 ( accessed March 2023 ). 23 Versus Arthritis . www . versusarthritis . org / about-arthritis /
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 ).
SCIENCE PHOTO LIBRARY
14 | hospitalpharmacyeurope . com