Connective Tissue Disorders Flashcards
Define systemic lupus erythematosus (SLE)
an autoimmune disease in which the body’s immune system mistakenly attacks healthy tissue. It can affect the skin, joints, kidneys, brain, and other organs.
Define Limited scleroderma (=CREST)
one subtype of scleroderma — a condition that literally means “hardened skin.” The skin changes associated with limited scleroderma typically occur only in the lower arms and legs and sometimes the face and throat. Limited scleroderma can also affect your digestive tract.
Define Diffuse scleroderma
a type of systemic sclerosis (scleroderma). Systemic scleroderma can affect any part of the body. It often affects the skin, blood vessels, and internal organs, especially the lungs, heart, kidneys, and gastrointestinal tract.
Define polymyositis
(“inflammation of many muscles”) is a type of chronic inflammation of the muscles (inflammatory myopathy) related to dermatomyositis and inclusion body myositis.
Define dermatomyositis
an uncommon inflammatory disease marked by muscle weakness and a distinctive skin rash.
What is the HLA associated with SLE?
HLA-DR3
What is the HLA associated with scleroderma (limited & diffuse)?
HLA-DR5 & DRw8
What is the main clinical feature of scleroderma?
Skin fibrosis/hard skin
Polymyositis & dermatomyositis is associated with?
Underlying malignancy
What hypersensitivity reaction is SLE?
Type III -immune complex (tissue damage caused by the activation of complement in response to antigen-antibody (immune) complexes that are deposited in tissues.)
What are the 4 key features of connective tissue disease?
Inflammation Fibrosis/scarrign Vasospasm (including Reynaud's) Vascular thrombosisInflammation Fibrosis/scarrign Vasospasm (including Reynaud's) Vascular thrombosis
Risk factors for SLE?
Drug-induced
Increased in classical complement deficiencies
In afrocarribean
FemalesDrug-induced
Increased in classical complement deficiencies
In afrocarribean
Females
Autobodies associated with SLE:
ANA (95%) Anti dsDNA Anti-Sm Drug-induced Anti-histoneANA (95%) Anti dsDNA Anti-Sm Drug-induced Anti-histone
Autobodies associated with Limited scleroderma CREST:
Anti-centromere
Autobodies associated with diffuse scleroderma:
Anti Scl-70 Fibrillarin RNA pol I, II, III PM-SclAnti Scl-70 Fibrillarin RNA pol I, II, III PM-Scl
Autobodies associated with Polymyositis & dermatomyositis:
Anti Jo-1 (=tRNA synthetase)
Histolgy of Polymyositis:
Endomysial inflammation infiltrate
Histolgy of dermatomyositis:
Drop out’ of capillaries and myofibre damage
Histolgy of SLE:
LE bodies
CNS - small vessel angiopathy
Spleen - onion skin lesions
Heart - Libman-Sack Endocarditis
Histolgy of limited scleroderma:
Increased collagen in skin and organs
Onion skin thickening of arterioles
Histolgy of diffuse scleroderma:
Inflammation within or around muscle fibres
Signs & Symptoms of SLE:
Need 4/11 ACR criteria
SOAP BRAIN MD
Serositis
Oral ulcers
Arthritis
Photosensitivity
Blood disorders (AIHA, ITP, leucopenia) Renal involvement ANA +ve Immune phenomena (dsDNA, anti-Sm, Antiphospholipid Ab) Neuro symptoms
Malar rash
Discoid rash
Signs & Symptoms of Limited scleroderma:
Skin changes on face and distal to elbows and knees
CREST
Calcinosis Raynaud's Esophageal dysmotility Sclerodactyly Telangiectasia
Associated with pulmonary HTN
Signs & Symptoms of diffuse scleroderma:
Skin changes can occur anywhere
Widespread organ involvement
Associated with pulmonary fibrosis
Signs & Symptoms of Polymyositis & dermatomyositis:
Proximal muscle weakness Increased CK & abnormal EMG DM has cutaneous features: 1) Heliotrope rash 2) Gottron papules
Associated with pulmonary fibrosis
