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)
