Autoimmune Diseases and SLE Lecture Notes

Flashcards about Autoimmune Diseases and Systemic Lupus Erythematosus (SLE)

Autoimmune Disease

A disease caused by a breakdown of self-tolerance where the adaptive immune system responds to a self-antigen, causing cell and tissue damage.

Systemic Lupus Erythematosus (SLE)

A systemic autoimmune disease characterized by a vast array of autoantibodies, particularly antinuclear antibodies (ANAs), where injury is caused mainly by deposition of immune complexes and binding of antibodies to various cells and tissues.

Antinuclear Antibodies (ANAs)

Autoantibodies present in SLE that target nuclear antigens.

Type III Immune Complex-Mediated Disorders

Disorders where antigen-antibody (immune) complexes formed in the circulation deposit in blood vessels, leading to complement activation and acute inflammation.

Familial Association in SLE

Up to 20% of unaffected first-degree relatives have autoantibodies.

HLA Association in SLE

HLA-DR2 or HLA-DR3

Environmental Factors in SLE

Exposure to UV light, sex hormones, certain drugs like hydralazine, procainamide, and D-penicillamine.

Anti-phospholipid antibodies

Directed against epitopes of various plasma proteins that are revealed when the proteins are in complex with phospholipids.

EULAR/ACR SLE classification criteria

Includes ANA, Fever, leucopenia, thrombocytopenia, autoimmune haemolysis, delirium, psychosis, seizure, Non-scarring alopecia, Oral ulcers , etc

Subacute cutaneous lupus erythematosus

Annular or papulosquamous (psoriasiform) cutaneous eruption, usually photodistributed

Discoid lupus erythematosus

Erythematous-violaceous cutaneous lesions with secondary changes of atrophic scarring, dyspigmentation, often follicular hyperkeratosis/haematological (scalp), leading to scarring alopecia on the scalp

Clinical Manifestations of SLE

Arthritis, arthralgia, or myalgia, skin involvement, fever, fatigue, weight loss, renal issues, neuropsychiatric symptoms, pleuritis, pericarditis, gastrointestinal problems, Raynaud phenomenon, ocular issues, and peripheral neuropathy.

Acute Necrotizing Vasculitis

Involving capillaries, small arteries, and arterioles; seen in SLE.

Butterfly Rash

Erythema affects the face along the bridge of the nose and cheeks in SLE.

Libman-Sacks Endocarditis

Valvular endocarditis associated with SLE, characterized by single or multiple 1- to 3-mm verrucous deposits.

Onion-Skin Lesions

Concentric intimal and smooth muscle cell hyperplasia of central penicilliary arteries in the spleen, seen in SLE.

Neurolupus Pathology

Microinfarcts, microthrombosis, vasculopathy, and (rare) vasculitis in the central nervous system.

SLE Renal Involvement

Associated with the deposition of immune complexes within the glomeruli, leading to various patterns of glomerular disease (class I to class VI).

Wire-Loop Structures

Circumferential thickening of the capillary wall due to subendothelial immune complex deposits in diffuse lupus nephritis (class IV).

Advanced Sclerosing Lupus Nephritis (class VI)

Sclerosis of more than 90% of the glomeruli, representing end-stage renal disease.