HPE Autoimmune disease: The fundamentals | Page 18

TABLE 3

Summary of main autoimmune connective tissue disorders
Disease Prevalence Manifestation Antibodies / immunology
Lupus erythematosus ( LE ) 17
It is more frequent in women and African American patients ; annual incidence of 2.74 / 100,000 people for the cutaneous form
Multisystemic disorder that affects mainly the skin ( with specific and non-specific lesions ), with significant morbidity and mortality . It can be classified into systemic LE , drug-induced LE and cutaneous lupus erythematosus ( CLE )
Antinuclear antibodies , complement ( C3 and C4 ) levels and antiphospholipid antibodies . Subacute CLE often presents with anti-SSA / Ro antibodies 18
Dermatomyositis 19
Female predominance and a bimodal distribution of the age of onset ( with two peaks around 8 and 52 years ), with a prevalence ranging from 9 to 32 / 100,000 people
Periorbital oedema , Raynaud phenomenon , scalp erythema and scaling are cutaneous manifestations . Cutaneous lesions of dermatomyositis affect mainly sun-exposed areas and hands , elbows , and knees . Dermatomyositis can affect only the skin ; in up to 30 % of these cases there is interstitial lung disease
anti-Mi-2 , SRP , TIF1-γ , MDA5 , NXP-2 , SAE , Jo1 , PL-7 , anti-PL-12 , anti-EJ , and anti-OJ
Sjögren Prevalence of 60 / 100,000 , syndrome 20 affecting mainly middle aged
( fifth decade )
Affects mainly the salivary and lacrimal glands ( with dysfunction ) it can also associated with arthritis , peripheral neuropathy , internal organ involvement , and B-cell lymphoma . Skin manifestations include xerosis ( xerophthalmia , xerostomia and vaginal dryness ), annular erythema and Raynaud phenomenon anti-SSA / Ro , anti-SSB / La , rheumatoid factor
Antiphospholipid syndrome 21
Affects mainly young women , frequently in association with SLE
Vascular thrombosis ( sometimes with central nervous system involvement ) and complications of pregnancy . Other possible extracutaneous manifestations are thrombocytopenia , haemolytic anaemia , valvular heart disease and nephropathy
Antiphospholipid antibodies
Systemic sclerosis 22
17.6 / 100000 ; it is more frequent in women , and usually starts between the 3rd and 4th decade of life
Skin , vascular dysfunction ( Raynaud phenomenon is normally present ) and other organ involvement ( gastrointestinal tract , lungs , heart , and kidneys )
Anti-topoisomerase-I and anti-RNA polymerase III antibodies
Localised Incidence between 3.4 – scleroderma 23 27 / 100,000 and childhood onset in approximately 50 %
Normally limited to the skin and lesions are erythemato-violaceous patches and plaques that usually regress after several years of progression
Unknown
Lichen sclerosus et atrophicus 24
Unknown prevalence ( estimated at 0.1 % for children and 3 % for women over 80 years old )
Whitish , scar-like lesions with superficial wrinkling , which are often itchy and painful , and commonly affect the external genitalia ( less often in nongenital skin )
IgG autoantibodies against the extracellular matrix
Autoimmune bullous diseases There are several autoimmune bullous diseases caused by autoantibodies against skin elements . The main autoimmune bullous diseases are summarised in Table 2 .
Autoimmune connective tissue diseases The main autoimmune connective tissue diseases are summarised in Table 3 .
Conclusions Immune-mediated dermatological conditions encompass a wide range of conditions including inflammatory , bullous-like , and connective tissue disorders and they can have a major impact on the patient ’ s quality of life . Healthcare professionals are likely to be familiar with the presentation of common inflammatory dermatoses such as psoriasis , but they also need to remain vigilant to the development of any blistering and erosive changes reported by patients , particularly if present in conjunction with systemic upset , and arrange for further investigations . While such investigations may serve only to confirm or refute a more serious underlying disorder , more importantly , it provides an opportunity to develop a management plan that will hopefully help to alleviate patient suffering .
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