Connective Tissue Diseases Flashcards
underlying pathology behind systemic sclerosis
excessive collagen deposition causing skin and internal organ changes
Condition most associated with systemic sclerosis
Raynauds
3 phases of cutaneous involvedment in systemic sclerosis
oedematous, indurative, atrophic –> skin thickened and tight
Organs affected in systemic sclerosis
lungs, kidneys, gut
Affect of systemic sclerosis on the lungs
pulmonary fibrosis and hypertension
Effect of systemic sclerosis on the kidneys
increased hypertension –> renal crisis
Effect of systemic sclerosis on the gut
dysphagia, malabsorption, reflux, dysmotility
Systemic sclerosis can be split into…
limited and diffuse
Limited cutaneous sclerosis can be described by CREST, which stands for…
Calcinosis of the subcutaneous tissues, Raynauds, oEsophageal and gut dysmotility, Sclerodactyl, Telangiectasia
Where is skin involvement limited to in limited systemic sclerosis?
face, hands and feet
Autoantibody associated with limited systemic sclerosis?
Anti-centromere (70-80%), ANA
Diffuse systemic sclerosis is…
rapid and widespread skin changes with early and significant organ involvement
Autoantibody associated with diffuse systemic sclerosis?
Anti-scl70 (topoisermerase), ANA
Investigations for systemic sclerosis
auto-antibodies, organ screening (lungs, kidneys, heart)
Management of Raynauds in systemic sclerosis
Calcium channel blockers, iloprost (PGI2 analogue), bosentan (endothelin R antagonist)
Management of renal involvement in systemic sclerosis
ACE-Is (ramipril), ARBs (losartan)
Management of GI involvement in systemic sclerosis
PPIs - against reflux
Management of ILD in systemic sclerosis
immunosuppression - cyclophosphamide
main organs affected by SLE
skin, joints, kidneys, blood cells and nervous system
What is the pathogenic mechanism behind SLE?
due to defect in apoptosis, increased cell death but also a delay in clearance of dead cells leads to persistence of antigens i.e. dsDNA and thus immune complex production. antibody-antigen complexes are deposited in the basement membranes of skin and kidneys
The majority of SLE is in which group?
Women (11:1), 20-30 years
General presentation of SLE (get 5/6)
fatigue, weightloss, fever, arthralgia, myalgia, malar rash, discoid lupus, ulceration, lupus nephritis, pleurisy/pleural effusion, leukopaenia, anaemia, pericarditis, autoimmune hepatitis
Immunology for SLE diagnosis
ANA (95%, non-specific), anti-dsDNA (specific and varies with activity), anti-sm (specific but low sensitivity), C3/C4 (low when disease is active)
What investigations should be done for SLE (3)?
Bloods (anaemia, leucopaenia, thrombocytopaenia), urinalysis (nephritis), immunology
Management of SLE with skin and arthralgia
hydroxychlorquine, topic steroids and NSAIDs
Mechanism of action of hydroxychloroquine
blocks APCs
Management of SLE with organ involvement
immunosuppression with azathioprine or mycofenolate mofetil
Management of SLE with severe organ involvement
IV steroids and cyclophosphamide
Monitoring carried out with SLE
anti-dsDNA, BP, cholesterol, urinalysis
What is the pathogenic mechanism in sjogrens syndrome?
lymphocitic infiltrates form in exocrine organs
Common symptoms associated with sjogrens syndrome?
dry eyes and mouth, arthralgia, fatigue, vaginal dryness, parotid gland swelling
Associated conditions with SLE
RA and SLE
Which cancer risk is increased with SLE?
lymphoma
Diagnosis of sjogrens syndrome is based on?
Anti-Ro and Anti-La antibodies and Schimers test
Management of sjogrens is by…
symptomatic relief, eye drops, pilocarpine to stimulate saliva production, hydroxychloroquine to aid arthralgia and fatigue
what is antiphospholipid syndrome?
a disorder that clinically manifests as recurrent venous or arterial thromboembolism
Antibodies associated with antiphospholipid syndrome
Anti-cardiolipin, anti- b2glycoprotein
Signs of Antiphospholipid syndrome
increased frequency of stroke or MI, recurrent pulmonary emboli, catastrophic APS, late spontaneous fetal loss, recurrent fetal loss (<10weeks), migraines, livedo reticularis (skin)
Management of symptomatic Antiphospholipid patients?
anti-coagulation
Management of symptomatic pregnant antiphospholipid patients
LMWH (dalteparin, enoxaparin) - warfarin is teratogenic
Management of asymptomatic antiphospholipid patients
no anticoagulation
what is catastrophic APS?
rare, serious and often fatal (50%) manifestation with multiple organ infarcts over days to weeks
