SLE
SLE An inflammatory, multi-system autoimmune disorder
with arthralgia and rashes as the most common clinical
features, and cerebral and renal disease as the most
serious problems
Common in women
Age 20-40 years old
Etiology 1. Hereditary
2. Genetics
3. Sex hormone status – pre-menopausal
4. Atopic disease – asthma a/w SLE
5. Smoking
6. Silica exposure
7. Hormonal (contraceptive, HRT)
8. Drugs – isoniazid, hydralazine, procainamide,
penicillamine
9. Ultraviolet light – skin
[Link] to EBV
Clinical General Fever
features Malaise
Tiredness
Joints & Symptoms resemble RA – symmetrical
muscles small joint arthralgia, slight soft tissue
swelling
Jaccoud’s arthropathy
Myalgia
Skin Erythema – butterfly distribution on cheeks
& across bridge of nose
Vasculitic lesions on fingertips and around
the nail folds, purpura and urticaria
Photosensitivity
Livedo reticularis
Palmar & plantar rashes
Pigmentation
Alopecia (scarring vs non-scarring)
Raynaud’s phenomenon
Lungs Recurrent pleurisy
Pleural effusions
Pneumonitis
Atelectasis
Pulmonary fibrosis
CVS Pericarditis with small pericardial effusions
Mild myocarditis
In a/w ALPS - Raynaud’s, vasculitis, and
⬆ frequency of IHD & stroke
arterial and venous thromboses can occur
Kidney Classification of lupus nephritis
Class I Minimal mesangial lupus nephritis
(LN),
with immunedeposits but normal on
light microscopy. Asymptomatic
Class II Mesangial proliferative LN
with mesangial hypercellularity and
matrix expansion. Clinically, there is
mild renal disease. – low dose pred,
immunosuppressant
Class Focal LN (involving <50% of
III glomeruli) with subdivisions for active
or chronic lesions. Subepithelial
deposits seen. Clinically, there is
hematuria and proteinuria; 10–20% of
all LN
Class Diffuse LN (involving >50% of
IV glomeruli) classified by the presence
of segmental and global lesions, as
well as active
and chronic lesions. Subendothelial
deposits are present. Clinically, there
is progression to the nephrotic
syndrome, HTN and renal insufficiency.
Most common and most severe form
of LN.
Class V Membranous LN affects 10–20% of
patients. Can occur in combination
with class III or IV. Good prognosis
Class Advanced sclerosing LN (≥90%
VI globally sclerosed glomeruli without
residual activity). This represents the
advanced stages of
the above, as well as healing.
Immunosuppressive therapy is unlikely
to
help, as it is ‘inactive’. Progressive
CKD.
CNS Mild depression
Epilepsy
Migraines
Cerebellar ataxia
Aseptic meningitis
CN lesions
Cerebrovascular disease
Polyneuropathy
Eyes Retinal vasculitis
Episcleritis
Conjunctivitis
Optic neuritis
GI Mouth ulcer
Mesenteric vasculitis
Lab findings
1. Hematological flares
haemolytic anaemia with reticulocytosis
leukopenia <4000/mm3 total on 2 occasions
lymphopenia <1500/2 mm3 on >2 occasions
thrombocytopenia <100000/ mm3
2. Indication of renal biopsy
persistent proteinuria >0.5 g/day or >3+ if
quantitation not performed
cellular casts: presence of red cell, haemoglobin,
granular, tubular or mixed
Investigation Blood 1. FBC
s Leucopenia
Lymphopenia
Thrombocytopenia
Anemia
⬆ ESR
AIHA
2. RP
persistent proteinuria >0.5 in UPCR or
>0.5 g/day in 24hUP or urine dipstick
≥3+
active urinary sediment (defined as
>5 red blood cells [RBCs] per high
power field [hpf]; >5 white blood cells
[WBCs]/hpf in the absence of infection
or cellular casts limited to RBC or WBC
casts)
3. Urinalysis with microscopy for sediment
4. UPCR
24-hour urine protein
5. LFT – transaminases , serum albumin
(hypoalbuminemia in LN)
6. ESR, CRP
7. Autoantibodies
ANA
Anti-dsDNA - ≥ 90% specificity
Anti-Ro
Anti-Sm
Anti-La
Antiphospholipid antibodies
ENA (anti-Sm, anti-SSA, anti-SSB, anti-
RNP)
8. Serum complement -C3 C4
Imaging 1. CT brain
2. MRI
Invasive Kidney biopsy
Diagnosis & 4 classification criteria
classification 1. ARA
criteria 2. ACR
3. SLICC
4. EULAR/ACR - +ve ANA titer ≥1:80 on Hep-2 cells)
Management 1. Advised to avoid excessive exposure to sunlight
2. NSAIDs – arthralgia, arthritis, fever, serositis
3. Corticosteroids
4. Antimalarial (hydroxychloroquine) (retinal toxicity)- anti-
inflammatory and immunomodulator effects
5. Immunosuppressive drugs
Cyclophosphamide
mycophenolate mofetil (MMF)
azathioprine
methotrexate
calcineurin inhibitors – ciclosporin, tacrolimus
rituximab (anti-CD20)
belimumab
6. Plasma exchange/ plasmapheresis
Removal of circulating immune complexes,
autoantibodies
7. IVIG
To add:
Types of lupus rash
SLE & ALPS