SLE Flashcards
What is the clinical presentation of SLE: joints (90%) and muscles ?
Joints
• Typical: symmetrical small joint polyarthritis (resembling RA), improves w trtmt (usually IPJ in the hands)
- Painful but no deformity (vs rarer Jaccoud’s Arthropathy)
• Rarer
- Joint deformity and bony erosions
- Jaccoud’s arthropathy (CORRECTABLE RA-like deformities 2’ to soft tissue abnormalities rather than joint destruction)
- AVN hip 2° to corticosteroids or disease itself
Muscles
• Generalized myalgia (50%)
• True myositis (<5%) - if prominent consider concurrent polymyositis
What are the mucocutaneous manifestations (85%) of SLE?
Alopecia (scarring/non-scarring): if scarring, can lead to irreversible bald patches
Butterfly/malar rash: characteristic
- Affects cheeks and nose bridge; Sparing nasolabial fold
Photosensitivity (40-50%; esp in anti-Ro +ve) – exposure to sunlight may ppt exacerbations -> causes scaly, raised erythematous rash on sun exposed skin
Painless aphthous mouth ulcers +/- 2° infection (will be painful)
Manifestations of Vasculitis
• Raynaud’s phenomenon: vasoconstriction (pale), cyanosis (blue) and hyperemia (red) due to cold or stress. May precede clinical probs by years
- Raynaud’s is more common in Systemic Sclerosis / Scleroderma
• Vasculitic infarcts: fingertips, nail folds, splinter haemorrhages, ulcerations. Splinter Haemorrhages: both SLE and IE is by immune complex
• Purpura, urticarial, livedo reticularis palmar & plantar rashes, pigmentation
• Palpable Pupura mainly on fingertips is very common (not specific for SLE)
Discoid lupus:
• Benign variant of lupus; only skin involved
• Well-defined erythematous plaques that progress to scarring & pigmentation (can cause scarring alopecia)
• Subacute cutaneous lupus erythematosus (rare variant)
What are the lung manifestations of SLE?
Bilat recurrent pleuritis + pleural effusion (often bilateral)
Acute lupus pneumonitis : fever, cough, SOB, hypoxemia, basal creps, pulmonary opacities on CXR
- Pneumonitis can then progress into ILD
- In fact, presents v similarly to pneumonia! Hence TRO infection!
ILD: chronic non-productive cough, SOB, ↓ ET, abnormal PFTs eg Spirometry (restrictive pattern, ↓ DLCO, desaturation on exertion), fine creps
Shrinking lung syndrome: SOB, repeated CP, progressive ↓ lung vol, no pleural/parenchymal disease on CT
Pulmonary haemorrhage 2’ vasculitis: SOB, cough, haemoptysis
*SLE treatment predisposes to infection, so must r/o infection!
What is the heart & CVS manifestations of SLE?
Heart
• Pericarditis w small pericardial effusions (detected by 2DE)
• Mild myocarditis: may cause arrhythmias
• Rarely
- Aortic valve lesions
- Cardiomyopathy
-Libman-Sacks Syndrome (non-infective endocarditis of MV)
CVS disease
• Raynaud’s, vasculitis, thromboses (arterial/venous) esp a/w APS
• ↑ risk of IHD and CVA in w SLE - due to alteration of common RFs (HTN, lipid levels) and chronic inflammation
What is the eye manifestations of SLE?
- Retinal vasculitis -> infarcts (cytoid bodies, appear as hard exudates) and haemorrhages
- Episcleritis, conjunctivitis, optic neuritis
- 2’ Sjogren’s syndrome (dry eyes, dry mouth) (15%)
What is the GIT manifestations of SLE?
Mesenteric / gut vasculitis (lupus gut) -> can cause SB infarction/perforation
- Causes absent bowel sounds, tender abdomen, N&V
- Loss of bowel sounds due to oedematous small bowels
Liver involvement and pancreatitis (uncommon)
What is the heme manifestations of SLE?
Symptomatic Anaemia? Jaundice? SLE is a/w AIHA, AoCKD
Easy bruising? SLE is a/w autoimmune thrombocytopenia
SLE can result in TTP
Look out for classical pentad of:
- Thrombocytopenia: Diagnose via assessing for ADAMST 13 deficiency
- MAHA
- Neuro Deficit
- AKI (renal deficit)
- Fever
What are the kidney manifestations of SLE?
All SLE patients – Screen regularly for haematuria, proteinuria
Range of presentation (asymptomatic haematuria / proteinuria; nephrotic syndrome / nephritic syndrome; RPGN; CKD)
- Nephritic syndrome most common! Hence haematuria!
- Class 4 (diffuse proliferative)is the most severe form!
Glomerulonephritis: proteinuria, (microscopic) haematuria, urinary casts, dysmorphic RBCs
- May even be cx by RPGN! And Acute renal failure!
- Any swelling in your feet, around your eyes?
Renal vein thrombosis: nephrotic syndrome or a/w APS
What are the classes of lupus nephritis? What are the clinical manifestations + histological features?
- Minimal mesangial
- Rarely diagnosed: pt have a normal urinalysis, no / minimal proteinuria, and a normal serum creatinine
- Histo: Presence of mesangial deposits on immunofluorescence - Mesangial proliferations lupus nephritis
- Presents w microscopic haematuria and/or proteinuria
- Histo: mesangial hypercellularity & mesangial matrix expansion (hence proliferative) on light microscopy - Focal proliferative nephritis
- Usually haematuria & proteinuria; Some have HTN,
decreased GFR, Nephrotic Syndrome (ie nephrotic-range proteinuria)
- <50% of glomeruli are affected on light microscopy (but we will see diffuse involvement on immunofluorescence)
- Subendothelial and Mesangial Deposition of immune complexes - Diffuse proliferative: Most Common and Most Severe form!
- All pts: Nephrotic Syndrome, HTN, decreased GFR
- >50% of glomeruli are affected on light microscopy (but we will see diffuse involvement on immunofluorescence)
- Subendothelial and Mesangial Deposition of immune complexes - Membranous: Proteinuria, nephrotic
- Presents similarly to Idiopathic Membranous mephropathy.
- Mainly presents with nephropathy, +/- haematuria, HTN
- Histo: diffuse thickening of the glomerular capillary wall on light microscopy + subepithelial immune deposit - Advanced sclerosing lupus nephritis / Glomerulosclerosis
- Presents w slowly progressive renal dysfunction a/w proteinuria and a relatively bland urine sediment
- Histo: global sclerosis of >90% of glomeruli; represents healing + scarring of prior inflamm injury + advanced stage of class III, IV, or V
- Immunosuppression will not help here! As there is no active GN
What are the CNS manifestations of SLE?
Psychiatric (depression, psychosis)
- Any hallucinations?
- Did family and friends notice anything different about you?
Neurologic (seizures, strokes, focal neuro deficits)
Others: migraines, cerebellar ataxia, aseptic meningitis, CN lesions, CVA or polyneuropathy
Cerebral lupus
What are the investigations required for SLE?
FBC
- Leukopenia, lymphopenia and/or thrombocytopenia
- Anaemia: NCNC anaemia due to ACD, Macrocytic anaemia 2’ thyroid probs (a/w SLE) or reticulocytosis 2’AIHA (DCT +ve)
Inflammatory markers
- ↑ ESR (in proportion to disease activity)
- CRP is usually normal, but may be ↑ in cases of serositis, lupus gut, pulmonary haemorrhage
RP
- Only ↑ when renal disease is advanced
- Hypoalbuminemia or ↑ UACR are early indicators of lupus nephritis
- Assess for pedal oedema and periorbital oedema (anasarca)
- Do urine dipstick, microscopy, Renal Panel
Autoab + complements
- ***anti-dsDNA, anti-Sm (spef for SLE eg when anti-dsDNA normal), ANA, anti-Ro anti-La
- ↓ C3, C4
Histology: IgG and complement deposition in kidneys and skin
Imaging
- CT brain: infarcts/ haemorrhage w evidence of cerebral atrophy
- MRI: detect lesions in white matter which are not seen on CT (hard to distinguish true vasculitis from small thrombi)
What is the screening required for SLE?
C3 C4, UFEME + dipstick if features of SLE/ lupus nephritis
What is the investigations required to monitor SLE?
↑ESR + ↑anti-dsDNA + ↓C3 C4 -> indicative of an acute flare
What is the work up for lupus nephritis?
Abnormal urinalysis + elevated Cr + SLE -> suspect Lupus Nephritis
- Usrinalysis: Look for Haematuria, Cellular Casts
- UPCR (urine protein to Cr ratio) OR 24hr urine protein
- UECr for azotemia
Followed up with Diagnosis via RENAL BIOPSY
- Only done if significant proteinuria (>500mg/day) OR +ve Urine Sediment
- Mild forms will rarely warrant immunosuppressive therapy!
- Treatment is guided by histological subtype!
- Clinical Presentation may not reflect the severity of the histologic findings
What is the diagnostic criteria of SLE?
EITHER ≥4/17 of the following, incl ≥1 clinical (in the absence of other causes) and ≥1 immunologic criteria
- B (blood): Haemolytic anaemia, Leukopenia OR lymphopenia, Thrombocytopenia
- R (renal): Spot UPCR (Urine Protein Cr Ratio) or 24h urine protein, representing 500mg/24h OR Red cell casts & dysmorphic RBC
- A (arthritis): ≥ 2 joints ie signs of inflamm Arthritis (Synovitis [effusion or swelling] OR Tenderness + ≥ 30 mins morning stiffness)
- I (Immunologic)(≥1/6): ANA , Anti-dsDNA, Anti-Sm, Low C3 C4 OR low CH50, Ant-phospholipid (lupus anticoag, anti-cardiolipin, anti-b2 glycoprot), Direct Coomb’s +ve in absence of haemolytic anaemia
- N (neurologic): Acute confusion state OR Seizures, psychosis, mononeuritis multiplex, peripheral or cranial neuropathy
S (serositis): Pleuritic pain ≥ 1 day or pleural effusion/rub OR Pericardial pain ≥ 1 day/effusion/rub or pericarditis by ECG
O: oral OR nasal ulcers
M – Malar rash (or other manifestations of acute cutaneous lupus etc photosensitivity)
A – alopecia (non-scarring)
D – discoid rash (or other manifestations of chronic cutaneous lupus)
OR Biopsy-proven lupus nephritis (w +ve ANA or anti-dsDNA)
What is the non pharm management of SLE?
Patient education and counselling: disease course, pregnancy
- Pregnancy should be avoided during active dz due to the high risk of miscarriage and exacerbation of SLE
- Only attempt pregnancy after 6 months of remission
Sun protection: UV light avoidance (esp 10am-4pm sun), high SPF (>50) sunscreen, avoid drugs that can cause photosensitivity
Smoking cessation (reduces disease activity)
Reduce CVS RF (including HTN control) -> pt will die of CV problems
Immunizations prior to immunosuppressant (NO LIVE VACC)
Encourage balanced diet/nutrition, exercise
What is the symptomatic management of SLE?
NSAIDs: for arthralgia, arthritis, fever, serositis
Topical corticosteroids: cutaneous lupus
Chloroquine/hydroxychloroquine: for mild skin disease, fatigue, unresponsive arthralgia
- Because HCQ is an immunomodulator rather than immunosuppresor
- Check eyes regularly to avoid bull’s eye maculopathy (1/2000)
High-dose oral corticosteroids: for severe AIHA or thrombocytopenia
IM (or short course of oral) steroids: severe arthritis, pleuritic or pericarditis
What is the pharmacological management of SLE?
For all: Hydroxychloroquine/ chloroquine: overall control of SLE activity + relieves constitutional, MSK, and mucocutaneous symptoms
Mild (eg skin, joint, mucosal)
- +/ - NSAIDS
- +/ - short course low dose steroids (eg pred ≤ 7.5mg daily)
- HCQ (+/- steroids) helps to both INDUCE and MAINTAIN remission
Moderate (sig but non-organ-threatening disease: constitutional, MSK, blood)
- Short course steroids (eg pred 5-15mg daily), taper once chloroquine effective: steroids helps to induce remission & as bridging therapy
- +/ - immunosuppressant (eg AZT MTX) for symptom control (often req): AZT can be used to maintain remission thereafter
Severe/life threatening (major organ involvement – lupus nephritis Class III-V, CNS, Mesenteric Vasculitis, TTP, Pulmonary Haemorrhage)
- Induction STAT: short course high dose GC (pred 1-2mg/kg) + IV immunosuppressant (eg mycophenolate / cyclophosphamide)
- Maintenance: Mycophenolate / Azathioprine
- Alternative therapy (if pt wants children) = Rituximab
What are poor prognostic features of SLE?
- Renal disease esp diffuse prolif GN
- HTN
- Male, young, or old at presentation
- Low socioeconomic status
- High overall disease activity
- APS/ presence of aPL
Can a patient get pregnant if that person has SLE?
Fertility is usually normal except in severe disease
Recurrent miscarriages may occur (esp. when aPL Abs are present)
Avoid pregnancy during active disease (esp w sig organ impairment) due to high risk of miscarriage and exacerbations (during and after preg)
Wait until disease has been quiescent for ≥6 months
Review medications
- Mycophenolate should be stopped
- Azathioprine, hydroxychloroquine and low-dose oral corticosteroids are generally safe
