Connective Tissue Diseases: SLE Flashcards by Rosa Bock

what are connective tissue diseases characterised by

spontaneous over activity of immune system

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SLE

chronic autoimmune disease, the presentation and course can be highly varied

immune system attacks body’s cells and tissues, leading to inflammation and tissue damage

antiboidy immune complexes precipitate and cause a further immune response

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epidemiology

onset usually after puberty, 20s and 30s

females>males

prevalence high in black persons in UK and US, although rarely in blacks in Africa

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what happens when males get the disease

get a more severe form

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genetic factors

genetic predisposition:

concordance in monozygotic twins, inc incidence among relatives

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hormonal factors

higher oestrogen - early menarche and oestrogen containing contraceptives

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environmental factors

viruses eg EBV
UV light may stimulate skin cells to secrete cytokines stimulating B cells eg UVA/B therapy
silica dust may inc risk
toxins eg cigarette smoke

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development of autoantibodies

defect in apoptosis causes increased cell death and a disturbance in immune tolerance

defective clearance of apoptotic cell debris causes extended exposure to antigen and immune complex formation (autoantibodies)

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what type of hypersensitivity reaction

III

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what are many of the clinical manifestations of SLE due to

deposition of immune complexes that form with antigens in various tissues

form in the small blood vessels causing an inflammatory reaction

are desposited on BM of skin and kidneys

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renal disease

asymptomatic and very severe - RENAL BIOPSY if urinalysis suggests appropriate

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what may mild disese involve

only joint pain,alopecia and skin rash

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name some constitutional symptoms

fever, malaise, poor appetite, weight loss and fatigue

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muco cutaenous symptoms

malar rash

photosensitivity

discoid lupus

subacute cutaenous lupus

alopecia

oral/nasal ulceration

Rayndaus phenomemon

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discoid lupus

Benign variant of lupus in which only the skin is involved. The rash is characteristic and appears on the scalp, face and ears as well-defined erythematous plaques that progress to scarring and pigmentation
There is often hair loss
Most patients will just have skin involvement, up to 10% of people will eventually develop SLE

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photosensitivity

prolonged exposure to sunlight can lead to exacerbations of the disease

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what can non scarring alopecia lead to

irreversible bald patches

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describe the rash that is seen

butterfly pattern

spares the naso-labial folds

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what mucocutaneous feature can precede the development of other clinical signs by years

Raynaud’s phenomenon

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MSK symptoms

polyarthralgia and poyarthritis

myopathy

inflammatory arthrits

arthropathy - rarely deforming or erosive

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what deforming arthropathy can occur

Jaccoud’s arthritis is a chronic non-reversible joint disorder that may occur after repeated bouts of arthritis
Causes ulnar deviation through MCP subluxation
Can occur in lots of rheumatology things

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what is there an increased prevalence of in the femoral head

avascular nerosis - may relate to steroids use

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what is the most common respiratory manifestation

pleurisy (2/3 patients) with/out effusion - effusions are small and bilateral
also PE, pumonary hypertension and ILD

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what are some other respiratory manifestations

Basal pneumonitis is often present, perhaps as a result of poor movement of the diaphragm, or restriction of chest movements because of pleural pain.

Also, PE, pulmonary hypertension and interstitial lung disease.

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renal symptoms

lupus nephritis - severe and often asymptomatic * 1/3 patients will have evidence of renal damage * the most frequently observed abnormality is proteinuria, causes nephrotic syndrome * renal biopsy is required * early treatment needed * The most common and severe form is the diffuse proliferative GN (class IV), ‘wire-loop’ appearance

management of proteinuria

* Proteinuria \>3mg treated with steroids * Step up to steroids and cyclophosphamide ± immunosuppression

neurological symptoms

* Occurs in up to 60% cases and symptoms often fluctuate. There may be a mild depression, but occasionally more severe psychiatric disturbances. * Depression/psychosis, migrainous headache, seizures, cranial or peripheral neuropathy, mononeuritis multiplex (individual peripheral nerves affected – foot or wrist drop).

what happens if mononeuritis multiplex onyl affects one nerve

foot or wrist drop

haematological symptoms

lymphadenopathy leukopaenia anaemia - haemolytic thrombocytopaenia

what haematological symptom occurs in a quarter of patients

lymphadenopathy

cardiac symptoms

pericarditis, pericardial effusion, hypertension

which drugs can cause SLE, and what are the associated antibodies

associated with isoniazid, anti-histone antibodies

anti-phospholipid syndrome symptoms

thrombocytopaenia (low platelet count) causes venous AND arterial thrombosis livido reticularis (skin) late spontaenous, or reccurent early foetal loss migraines common

what is the classic presentation of someone with APS

child bearing age, with recurrent miscarriage and eg DVT recurrent PE and thrombosis can also lead to pulmonary hypertension

is APS primary or secondary

both

why are patinets with APS more susceptible to infection

intrinsic: low complement, imparied cell mediated immunity, defective phagocytosis etc extrinsic: steroids, IS drugs, nephrotic syndrome

what are the anti-phospholipid antibodies

* anti-cardiolipin * anti-beta 2 glycoprotein * lupus anticoagulant must be positive on 2 occasions less than 12 weeks apart

APS treatment

lifelong coagulation hydroxychloroquine helps with systemic symptoms

anticoagulation for APS

* lifelong warfarin - however this is teratogenic * in pregnancy - aspirin/heparin

diagnosis of APS

what are the aims of investigation

confirm/establish diagnosis and determine organ involvement wide range of investigations that depend on symptoms

what investigations would always be done

immunology FBC - may show thrombocytopaenia urinalysis/renal biopsy

immunology

ANA anti-dsDNA antibody anti-Sm C3/4 levels

ANA

* sensitive but not remotely specific * good screening test * can be positive in all autoimmune diseases, RA, HIV etc * ≥1:160 in 90% of patients withbSLE

what ANA level is seen in the general population

1:160

anti-dsDNA antibody

* the most specific and varies with disease activity * levels often relfect lupus nephritis activity

anti-SM

the most specific but low sensitivity

other immunology

**Anti Ro, anti-La and anti-RNP** – may be seen in SLE but also may be seen in other conditions.

C3/4 levels

negatively correlate with disease activity

what must be done once the diagnosis is established

* urinalysis to look fo evidence of GN * screen for organ involvement - CT, MRI, ECHO

management - non pharmacological

counselling, regular monitoring and advice on pregnancy issues is essential avoid sun exposure (photosensitivity)

what is given to all patients with SLE

hydroxychloroquine (anti-malarial) may reduce systemic complications

side effects of hydroxychloroquine

* irreversible retinopathy - blindness * corneal deposits

what drugs are commonly used

NSAIDs and analgesia

what are steroids used for

* Used in small doses for skin rashes, arthritis and serositis * Moderate does for haematological abnormalities and persistent serosits * High doses for severe haematological changes and major organ involvement

when is IS used

* used when there is inflammatory arthritis or some types or organ involvement * (e.g. azathioprine, cyclophosphamide, methotrexate, mycophenolate mofetil)

what are the negative effects of IS drugs

possibly teratogenic bone marrow suppression and inc suscpetibility to infection

treatment of severe organ disease eg lupus nephritis

IV steroids and cyclophosphamide

treatment of unresponsive cases

other therapies such as IV immunoglobulin and biologics may be necessary. * Rituximab (anti-CD20) * Belimumab (anti-byls)

monitoring

anti dsDNA antibodies are checked regularly, as these vary wtih disease activity check urinalysis for blood or protein

subactue cutaneous lupus

raynaud's phenomenon

Connective Tissue Diseases: SLE Flashcards

(62 cards)