Systemic Lupus Erythematosus L1:: Environmental: Genetic

L1: SYSTEMIC LUPUS ERYTHEMATOSUS
● Introduction:
• An autoimmune disease《Connective tissue disorder》
● Risk factors for developing SLE: (in Female)

Environmental: tobacco, viral infection (Epstein–Barr virus [EBV], cytomegalovirus [CMV],
Genetic:
• identical twins.
• (HLA) DR2 and DR3
● Autoantibodies in Systemic Lupus Erythematosus and Some of Their

Clinical Associations:
● European League Against Rheumatism (EULAR)/ACR SLE classification
criteria:
• A patient was classified as having SLE if they had a positive ANA ≥1:80 and had ≥10 points.
● Clinical Features of Neuropsychiatric Lupus and Their Prevalence:
Malar rash, also named a butterfly rash, is a common

…facial presentation of multiple disorders
:‫ دي‬rash malar ‫من ضمن األمراض اللي بيحصل فيها الـ‬
)‫ (و الحمل بردو‬mitral stenosis & SLE
)‫ )نسخت من داتا زون‬nasolabial fold ‫ مش بيأخد الـ‬lupus ‫أفرق بينهم ازاي؟ الـ‬
● Lung involvement in SLE:
• Pleuritis (common): bilateral. CRP usually elevated (sometimes very high). Consider
infection if unilateral presentation and a history of immunosuppression.
• Acute lupus pneumonitis with or without pulmonary hemorrhage (2%): usually seen in a
systemically ill patient with diffuse ground glass changes. Bronchoscopy to R/O diffuse
alveolar hemorrhage and or infection. Frequently associated with antiphospholipid
antibodies.
• Chronic interstitial lung disease/fibrosis: common in patients with mixed connective tissue
disease or prior acute lupus pneumonitis.
• Pulmonary hypertension (HTN).
• Alveolar disease that is highly responsive to steroids. Infection: Typical or atypical.
● In what ways can the heart be involved in SLE?

• Pericarditis: When symptomatic, can be associated with a left-sided pleural effusion.
Colchicine may be an effective treatment. An asymptomatic pericardial effusion can be seen
on echocardiogram.
• Myocarditis: Rare. Presents as heart failure or unexplained tachycardia. Troponin is elevated.

• Vasculitis: (coronary): Rare.
• Secondary atherosclerotic coronary artery disease and myocardial infarction: Very common
especially later in disease.
● gastrointestinal tract be involvement in SLE:

• Esophageal dysmotility: Usually involves upper third of esophagus.
• Pancreatitis: Usually due to gallstones, alcohol, or hypertriglyceridemia. Can be due to
medications (azathioprine [AZA]). If due to SLE, the patient will have diffusely active disease.
• Serositis: Only occurs in patients with active systemic disease.
• Mesenteric vasculitis: Likely associated with active disease.
• Hepatitis: usually as a result of medications.
● classification system for glomerulonephritis due to SLE:

Class I : Minimal change G.N.
Class II : Mesangial proliferative G.N.
Class III : Focal G.N.
Class IV: Diffuse IV-S = segmental
IV-G = global
Class V: Membranous G.N.
Class VI: Advanced sclerosing G.N.
● Pathologic Features of Chronicity and Activity in Lupus Nephritis.
● Which patients with severe lupus nephritis are more likely to progress to
end-stage renal disease (ESRD)?
• Lower socioeconomic status.
• Poor medication compliance.
• Comorbidities such as diabetes and HTN.
• Failure to normalize serum creatinine or serum creatinine of >2mg/dL on therapy.
• Failure to decrease proteinuria to <1 g/day within 6 months of starting treatment.
• Renal biopsy evidence of high disease activity (cellular crescents) and chronicity (interstitial
fibrosis).
● Therapy for patients with severe lupus nephritis
a) Induction therapy: Steroid
♤ Class III/IV lupus nephritis

Intravenous (IV) pulse methylprednisolone; plus Mycophenolate mofetil (MMF) or
cyclophosphamide [CYC])
•Note: For patients who fail to improve on MMF, consider switching to CYC. Patients who fail
to respond to CYC consider switching to MMF.
• Patients who fail to respond to both are candidates for rituximab, calcineurin inhibitors
(cyclosporine, tacrolimus), or a combination of calcineurin inhibitors and low-dose MMF.
♤ Class V lupus nephritis
• Oral prednisone
• MMF
• Calcineurin inhibitors (cyclosporine/ tacrolimus) can be added to MMF: use caution in
patients with renal insufficiency or hypertension. Voclosporin is currently under investigation.
• IV CYC if other therapies fail.
● What are the hematologic manifestations in SLE?

• Autoimmune hemolytic anemia (AIHA)
• Leukopenia (lymphopenia [common], neutropenia)
• Thrombocytopenia
• Antiphospholipid antibody syndrome (APLAS)
• Anemia of chronic disease
• Thrombotic thrombocytopenic purpura (TTP)
• Macrophage activation syndrome (MAS)
• Rare but can be fatal (fever, cytopenias, organ dysfunction, splenomegaly, low erythrocyte
sedimentation rate [ESR] due to inability of the liver to synthesize proteins such as fibrinogen,
high triglycerides, high ferritin, high IL-2 receptor [CD25]). R/O EBV or CMV infection as a
trigger.
● Mucocutaneous lesions associated with SLE

(A) Malar rash: erythematous plaques that spare nasolabial folds.
(B) Oral ulcers: often seen on the hard palate and usually painless.
(C) Discoid: hyperkeratotic rash with mucous plugging that can cause scarring alopecia.
(D) Subacute: resembles tinea corporis with erythematous, irregular edges and central
clearing.
● Identify manifestations of lupus that warrant high-dose (prednisone ≥1

mg/kg/day) corticosteroid therapy.
• Severe lupus nephritis
• CNS lupus with severe manifestations (including transverse myelitis)
• Autoimmune thrombocytopenia with extremely low platelet counts .
• AIHA
• Acute pneumonitis
• Diffuse alveolar hemorrhage
• Severe vasculitis with visceral organ involvement
• Serious complications from pleuritis, pericarditis, or peritonitis
• MAS.
● DRUG-INDUCED LUPUS ERYTHEMATOSUS
HIGH (>5%)
- Procainamide 15%-20%
- Hydralazine (5%-10%)
MODERATE
- Quinidine
- D-penicillamine
LOW (<1%)
- lsoniazid
- Methyldopa
- Chlorpromazine
- Minocycline (5/10,000 patients)
- Ant-tumor necrosis factor a agents (2/1000 patients.)
Sources: By
Dr. /Aya Nabil
lecturer of internal medicine
Sherif Hadi

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L1: SYSTEMIC LUPUS ERYTHEMATOSUS

● Risk factors for developing SLE: (in Female)

● Autoantibodies in Systemic Lupus Erythematosus and Some of Their

● Clinical Features of Neuropsychiatric Lupus and Their Prevalence:

Malar rash, also named a butterfly rash, is a common

:‫ دي‬rash malar ‫من ضمن األمراض اللي بيحصل فيها الـ‬

)‫ (و الحمل بردو‬mitral stenosis & SLE

• Pulmonary hypertension (HTN).

• Alveolar disease that is highly responsive to steroids. Infection: Typical or atypical.

● In what ways can the heart be involved in SLE?

• Myocarditis: Rare. Presents as heart failure or unexplained tachycardia. Troponin is elevated.

● gastrointestinal tract be involvement in SLE:

● classification system for glomerulonephritis due to SLE:

● Pathologic Features of Chronicity and Activity in Lupus Nephritis.

● Therapy for patients with severe lupus nephritis

a) Induction therapy: Steroid

♤ Class III/IV lupus nephritis

● What are the hematologic manifestations in SLE?

● Mucocutaneous lesions associated with SLE

● Identify manifestations of lupus that warrant high-dose (prednisone ≥1

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