systemic lupus erythematosus - case management and literature review

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???