systemic lupus erythematosus - case management and literature review
DESCRIPTION
Management of Systemic Lupus Erythematosus(SLE) and brief literature review.TRANSCRIPT
Systemic Lupus Erythematosus
DR. BERNARD FIIFI BRAKATU
OBJECTIVES
Case Presentation
Definition
Epidemiology and Etiology
Pathology
Management – Hx, PE, Work-up, DD, Rx
Prognosis
CASE PRESENTATION
HISTORY
PC: Generalized body weakness x3/7
Dizziness x 3/7
HPC: Sxx began 3days ago and have worsened prgressively. Visited the A. Polyclinic where she was referred here for further mngt.
ODQ: chills+, fevero, dysgeusiao, cougho, dysuriao, vaginal dischargeo, bleeding PRo, hematemesiso, arthralgia+, skin rash+, vomitingo,diarrheao, headacheo hx of jaundiceo, wt losso, dysphagia+, paraesthesia+
DH: Nil of Note
SH: Lives at Ashaiman, ROHo, Smokingo, NHISo
P.E. Middle-aged woman looking unwell
Afebrile, Anicteric, Pallor++, mildly dehydrated, bipedal edema, L+(submandibular)
CVS: S1S2Mo BP 110/70 Pules 120bpm RGV
Chest: Clinically clear SpO2- 98%
Abd: soft, MWR, non-tender, L(2cm, non-tender)So2Ko
CNS:GCS- 15/15, Power 5/5 in all limbs
Status Localis
Ulnar deviation of both digiti minimi
Flattening of both thenar and hypothenar eminences
Pt unable to open mouth, oral ulcers, but no microstomia
Hyperpigmented macules – trunk and Upper Limbs, Malar Rash on face
DIAGNOSIS/PLAN
Diagnosis = Symptomatic Anaemia 2o ?cause
PLAN
Urgent FBC, GXM x2units of PRBCs
IVFs – 1L N/S, 1L R/L
Blood film comment
Tb folic acid 5mg dly x 30
Tb Fersolate 200mg tds x 30/7
Labs Hb – 6.7g/dl
WBC – 1.8 x 109/l
Neu – 0.32 x 109(18.1%)
Lym(0.94x109(52.5%)
Plt – 71x109/l
HCT – 21.5%
RDW – 20.6%
MCV – 79/um3
MCH – 24.6pg
MCHC 31.3g/dl
Impression: Pancytopenia 20 ?cause
Differential – Autoimmune disorder r/o SLE and RVI
PLAN
ANA, ds-dNA, BUE & Cr, LFT
Hemotransfuse 2units of whole blood
IVF 3.5L – 2L N?S, 1L 5% Dex, 500ml R/L
IV Hydrocortisone 200mg 8hrly x 24hrs
Admit to GFW
Day 2-5
Same Mngt
Yet to do labs
Day 5 – Able to swallow
Stop Hydrocortisone
Tb Prednisolone 40mg dly x 14/7
Tb Omeprazole 20mg bd x 5/7
VCT – non-reactive
ESR – 55(0-20)
Urea (6.37(2.1-7.1), Cr- 136.7(53-123.8) eGFR -48(90-120)
Na – 135.9(136-150), K – 4.07(3.5- 5.5)
Total Protein – 50.4(60-80), Albumin13.6(30-55)
AST- 193(5-40), ALT -62(10-40), ALP -353.2(5-270)
Total bil 0- 6.41, Direct bil – 3.15, Indirect bil – 3.26
Day 6
c/o – diarrhea and vomiting
Tabs Cipro and Flagyl added
Awaiting ANA and ds- DNA(samples to be sent to S/A)
LITERATURE REVIEW
DEFINITION
SLE is a chronic inflammatory, multisystemic, autoimmune disease in which organs and cells undergo damage mediated by tissue-binding autoantibodies and immune complexes
EPIDEMIOLOGY/ETIOLOGY
M:F ratio – 1:9
Peak age of 20-40yrs
All ethnic groups affected but commoner in African Americans(>>Africans)
Accounts for 5.28% rheumatology cases in Nigeria(Adelowo, O.O. and Oguntona S.A.)
Etiology is from a complex interplay of genetic and environmental factors(drugs)
PATHOLOGY
Widespread vasculitis from fibrinoid and immune complex deposition on capillaries, arterioles and venules of vital organs and serous membranes.
MANAGEMENT
HISTORY Easy fatiguability, malaise, symptoms of anemia,
arthralgia(small jts in 90% of pts), Raynaud’s phenomenon, gangrene of the digits
Skin Rash – malar(butterfly) rash, livedo reticularis, alopecia, photosensitivity
Edema(renal involvement), fever, lymphadenopathy
Gum bleeding, menorrhagia, purpura (thrombocytopenia)
Cough, pleuritic chest pain
Neuropsychiatric Sxx, seizures
PMH – HPT, Prev. episodes(remissions and exacerbations)
DH: hydralazine, procainamide, minocycline,
Drug History Antiarrhythmics - procainamide, disopyramide, and propafenone;
Antihypertensives - hydralazine; methyldopa, several angiotensin-converting enzyme inhibitors and beta blockers
Antithyroid – Propylthiouracil
Antipsychotics - Chlorpromazine and lithium;
Anticonvulsants - carbamazepine and phenytoin;
Antibiotics - isoniazid, minocycline, and macrodantin;
Antirheumatic - sulfasalazine; penicillamine
Diuretic - hydrochlorothiazide;
Antihyperlipidemics - lovastatin and simvastatin;
Interferons and TNF inhibitors.
P.E. General Exam – Fever, Jaundice(rarely autoimmune
hepatitis), Pallor, edema, lymphadenopathy
Mouth ulcers, alopecia, livedo reticularis, discoid patches
Sun-exposed areas, elbows and knees - vasculitic rashes and subcutaneous nodules
Nails – splinter hemorrhages, nail-fold capillaries and periungual infarcts
Hands - Palmar erythema, Raynaud's phenomenon, arthritis
Malar rash
Discoid Rash
Livedo Reticularis
Subcutaneous Nodules
Systemic Exam CVS – HPT, Murmurs(Libman-Sacks endocarditis),
pericardial rub(pericarditis)
RS – decr AE, pleural rub, Creps – r/o Pleural Effusion, Atelectasis, pulmonary fibrosis
CNS – Decre orientation – r/o Seizures, cerebellar ataxia, aseptic meningitis, cranial nerve lesions, cerebrovascular disease or a polyneuropathy
Eyes - hard exudates, and haemorrhages, episcleritis, conjunctivitis or optic neuritis, but blindness is uncommon
Work-up
FBC – leucopenia, lymphopenia and/or thrombocytopenia. An autoimmune haemolytic anaemia occurs. The ESR is raised in proportion to the disease activity. CRP is normal.
ANA, anti-dsDNA, Anit-sm, antihistones
BUE & Cr
Urine R/E - proteinuria, cellular casts
Head CT scan/MRI – infarcts, hemorrhages and cerebral atrophy
Diagnostic criteria 1. Malar rash
2. Discoid rash
3. Photosensitivity
4. Oral ulcers
5. Arthritis - nonerosive arthritis
6. Serositis - pleuritis, pericarditis
7. Renal involvement
8. Neurological involvement - seizures, psychosis
9. Haematological involvement
10. Antinuclear antibody - Anti-dsDNA, anti-Sm, and/or anti-phospholipid
11. Immunological disorder - An abnormal titer of ANA
DifferentialsDrug-induced lupus
Antiphospholipid syndrome
Mixed Connective Tissue disease
CREST Syndrome
Scleroderma
Sjogren Syndrome
Polymyositis
Acute Pericarditis, Infective Endocarditis, Rheumatoid Arthritis
Lyme Disease
Treatment Corticosteroids
NSAIDS
Antimalarials
Immunomodulators –Cyclophosphamide, Methotrexate, Azathioprine, Mycophenolate
Anti-CD20 monoclonal antibodies eg. Rituximab
Avoid exposure to sunlight
Encourage patient to join a lupus society
Prognosis
An episodic course is characteristic, with exacerbations and complete remissions that may last for long periods
The arthritis is usually intermittent but arthralagia and fatigue are often more persistent
10-year survival rate is about 90%, although much lower if major organ-based complications are present
QUESTIONS???