Teaching Clinic - Various Facets of the Clinical Manifestations of SLE Flashcards
PWM M/26 factory worker
- In 1992, he presented with sudden onset of malar rash and non-itchy, non painful erythematous plaque-like lesions over both upper limbs
- photosensitivity was also noted
- he was diagnosed to have discoid lupus
by the dermatologist
- initially treated with topical steroid only
What should dermatologist do?
- Check ANA (anti-nuclear) antibody [high sensitivity test, but non-specific (SCREENING), test for other antibodies if +ve]
Recorded in titres (1/120, 1/360, 1/1080 = still +ve after dilution, if 1/1080, it means that it is strongly +ve, underlying connective tissue disease)
- Anti-Ro, Anti-Smith (high specificity but low sensitive, may be a/w with neurological involvement), anti-La, anti-RNP (high levels in MCTD)
- anti-dsDNA (very specific, diagnostic)
CRITERIA FOR THE CLASSIFICATION OF SLE 1982
Need 4 out of 11 to be diagnosed with SLE
2012 SLICC* SLE Criteria
How is this different to SLE 1982 criteria?
Includes more immunologic criteria
Specific cutaneous manifestations in lupus erythematosus
- Discoid
- Subacute cutaneous (Papulosquamous & Annular/polycyclic)
- Malar rash
What is the burden of cutaneous manifestation of lupus?
Skin is second most commonly affected organ, prevalence is about 80%, cutnaeous lesions second most common presenting clinical features
Caries consideralbe morbidiities, psychosocial well-being and medical costs
Discoid lupus
- Characteristics
- Special indications
- Well-demarcated, palpable, indurated, hyper or hypopigmentation, heals with scarring, rash can be diffused
- Association with systemic involvement will be relatively less! = 5%
What are the two types of subacute cutaneous lupus erythematosus (SCLE)?
Two types of cutaneous lesions
- papulosquamous (psoraiform)
- annular/polycyclic
In contrast to discoid lesions, they are nonindurated and have little or no telangiectasia or follicular plugging [NO SCARRING]
[Photosensitivity is a hallmark for SCLE = AVOID SUN]
Psoriasis is more common in lupus patients
What are the associations of Subacute Cutaneous Lupus Erythematosus (SCLE)?
- 75% are female
- 30% to 50% of these patients may demonstrate systemic features
- systemic disease tends to be mild and nephritis rarely occurs
What is this a mimic of?
Erythema maginatum like lesions (acute rheumatic fever)
What is malar rash?
- typically occurs acutely in patients with SLE
- does not necessarily indicate the presence of active systemic disease
- Photosensitive dermatitis and discoid lesions may occur in a malar distribution in patients with both SCLE and DLE
Major DDx is dermatomyositis (doesn’t spare nasolabial folds)
Non-specific cutaneous manifestations in Lupus Erythematosus
Nonspecific Lesions
* Photosensitivity
* Alopecia
* Raynaud’s phenomenon (pallor [ischaemia], cyanosis [hypoxia], rubor [reactive vasodilation]) = think of systemic sclerosis
* Livedo reticularis (exposed to cold environment, excitement) = must screen for anti-phospholipid antibodies
* Bullous (pemphigoid / pemphigus / zoster)
* Lupus panniculitis (Erythema nodosum)
* Vasculitis (vasculitic lesions over hypothenar and thenar eminence, periungual telangiectasia, splinter haemorrhages = secondary vasculitic lesions)
* Urticaria-like vasculitis (hives usually last less than 24 hours, if it is more than 24 hours, there may be underlying vasculitis)
* Oral ulcerations
* Nail dystrophy
- Types:
◦ Diffuse
◦ Patchy
‣ Also describe the scalp:
‣ Inflammed: Scaring;
‣ Clean: NO Scarring
Livedo reticularis
Usually forearm and front of knee
- Panniculitis (Loss of subcutaneous fat: Depression)
- Lupus profundus
Vasculitis lesions
Periungual erythema
Periungual telangiectasia
Conventional Therapy for Cutaneous LE
- Education, lifestyle modification (i.e. photoprotection)
- Topical steroids
- Intralesional steroids
- Antimalarial drugs
How to counsel lupus patient for photoprotection?
- To avoid sun exposure especially between 10am to 3pm
- To wear photoresistant clothing and broad rimmed hats
- To apply sunscreen with a sun protective factor of 15 or higher at least 20 to 30 minutes before sun exposure
- Both ultraviolet A and ultraviolet B may induce lesions in photosensitive individuals
- Broad spectrum sunscreens may offer better protection
- Patients should take protective measures during prolonged car travel
Major S/E of topical steroids
- Commonly prescribed to treat cutaneous LE
- To minimise the risks of atrophy, the protency of the corticosteroid preparation is tailored to the location of the lesions being treated i.e.
– low potency for facial lesions
– medium potency for the trunk and extremities
– high potency for palms and soles
– adrenal crisis
Skin atrophy = underlying vessels will be more visible = reddish
- They are probably of limited value in the treatment of widespread DLE and in most patients with SCLE
- Application over large body surface may lead to significant specific absorption with its associated toxicity
Intralesional Steroids
- When is it used?
- When is it not recommended?
Side Effects of Antimalarial Drugs
- Bull’s eye maculopathy (refer to ophthalmologist)
- Blue black skin discoloration in sun exposed areas may occur
- Light coloured hair may also turn white
- Yellow discolouration of the skin, sclerae and bodily secretion occurs with the use of quinacrine
- Bone marrow toxcity, haemolytic anaemia and neuropathy may rarely occur
How are antimalarials used for SLE?
Renal manifesations in SLE
Blood is rich in protein, ask woman to skip urinalysis when she is on her period!!!
WHO classification of lupus nephritis 1982
No Man Faces Diffuse Menstrual Situation
What can be seen here?
- cytoid bodies were seen at his left macula region
- Dot blot haemorrages
- Exudates
DDx:
- Non-proliferative Diabetic retinopathy
- HTN-retinopathy (Type III)
- Cytoid bodies (SLE):
◦ ~Roth spot in infective endocarditis
◦ Cytoid in SLE: White spots
◦ Occuring in wide range of patients
In a patient with SLE and CNS involvement, what factors other than SLE can cause CNS involvement?
Could have psychological reaction: depressed due to Cushingnoid features, alopecia, self-steem issues
Pancytopenia in SLE
Patient w/ SLE on systemic steroids, presented with diffuse skin lesions. What could this be?
◦ Fungal infections
◦ Papular eczema
◦ Refractory lupus:
‣ DO NOT respond to conventional treatment
◦ Secondary syphilis: Generalized rash (especially: prefominantly palm region) = should have Hx of primary chancre + has more palmar involvement
‣ Trepenomal movilization syndrome
Treatment of Refratory Cutaneous Lupus
Systemic treatment for cutaneous lupus
Retinoids not too good
Methotrexate is useful for joint!
Mycophenolate (steroid staring)
Voclosporin (just approved 2 years ago)
Intravenous immunoglobulins
Belimumab (monoclonal antibody against B cell activating factors [BAFF])
Aniforlumab: anti-interferon alpha (just approved 3 years ago)
Which valve is most commonly effected in SLE? What antibodies is cardiac involvement associated with? What therapy may valvular heart disease in SLE respond to? What medications may we consider giving long-term?
CONDUCTION ABNORMALITIES IN SLE
CAD in SLE. What population may it affect the most?
