Multisystem autoummine diease Flashcards
(32 cards)
what may it mimic?
drugs, infection, malignancy, cardiac myxoma, cholestrol emboli, scurvey
What is SLE (systemic Lupus erythemetosus)?
SLE is an inflammatory multisystem disease characterized by the presence of serum antibodies against nuclear components.
epidemiology sle
It is a disease mostly of young women, with a peak age of onset between 20 and 40 years. More common in african-american women. Multisystem inflammatory disorder. Wide spread vasculitis effecting all blood vessels and deposits of fibrinoid are found in both the blood vessels and tissues.
aetiology sle
the cause of the disease is unknown but probably multifactorial:hereditary, genetics, sex hormone status/ kleinfelters syndrome, drugs, uv light, eb virus exposure.
clincial features of SLE
MSK (symmetrical small joint arthralgia, Myopathy),
Skin (Butterfly rash (cheeks and bridge of nose), Discoid rash (raised, scarring, alopecia), photosensitivity, alopecia, mucosal ulceration of the nose, mouth and vagina, oral ulcers.),
vascular (Raynauds, vasculitic rash, VT),
Renal (glomneph, CKD),
Neurological (psychoses, epilepsy, migraine),
Haemotological (anaemia, thrombocytopenia, leucopenia), serositis (pleurisy or pericarditis or arrythmias),
Immunological. Wide spread vasculitis. Respiratory, GI and Cardiovascular effects.
Ix sle
Raised anti-dsDNA antibody,
Low serum complement C3 and C4,
Raised anti-SM antibody, anti-Ro, anti-La, histology, ANA, bloods,
ESR raised.
Tx sle
NSAIDs, corticosteroids,
Chloroquine and hydroxychloroquine,
immunosupressives, cyclophosphamide, steroid-sparring drugs such as azathioprine and methotrexate.
what is Scleroderma (SYSTEMIC SCLEROSIS)?
Systemic sclerosis (scleroderma) is a multisystem disease with involvement of the skin and Raynaud’s phenomenon.
RF’s of scleroderma
exposure to Silica dust or Vinyl chloride.
epidemiology of scleroderma
It is three times more common in women than in men and usually presents between the ages of 30 and 50 years.
Clinical features of Limited Cutaneous Scleroderma?
CREST - Calcinosis, Raynaud's (spasm of the arteries supplying the fingers and toes, leading to cold extremities), Eosophageal involvement (dysphagia) Sclerodactyly, Telaniectasia
Clinical features of Diffuse Scleroderma?
small bowel bacterial overgrowth, acute chronic kidney disease, pulmonary fibrosis, myocardial fibrosis. (RENAL, CARDIO, RESP)
how is the onset of diffuse cutaneous scleroderma different to the onset of limited cutaneous scleroderma?
limited starts with Raynaud’s, whereas diffuse begins with oedema before skin sclerosis follows.
Mx of scleroderma
tx raynauds, educate, skin care, immunosupression, nutritional support, tx of complications.
how is Raynaud’s managed?
oral vasodilators - CCB’s, ACEi, sympathectomy, hand warmers, battery powered gloves.
Sjogren’s Syndrome
characterized by immunologically mediated destruction of epithelial exocrine glands, especially the lacrimal and salivary glands. It predominantly affects middle-aged women.
clincial features Sjogren’s Syndrome
The main features are dry eyes (keratoconjunctivitis sicca, itchy, red, recurrent conjunctivitis) and dry mouth (xerostomia), parotid gland enlargement. Other features of Sjögren’s syndrome are arthritis, Raynaud’s phenomenon, renal tubular defects causing diabetes insipidus and renal tubular acidosis, pulmonary fibrosis, vasculitis and an increased incidence of non-Hodgkin’s B-cell lymphoma.
Ix Sjogren’s Syndrome
serum antibodies, labial gland biopsy, Positive anti-Ro, and anti-La. tear formation (schurmers test, rose bengal staining), salivary function (salivary gland scintigraphy, labial gland biopsy)
Tx Sjogren’s Syndrome
artifical tears, eye ointment, canaliculi occlusion, artifical saliva spray, tx secondary infections, hydroxychloroquinine may help associated arthritis, Corticosteroids if there are systemic sy present.
What is Polymyositis?
Polymyositis (PM) is a rare muscle disorder of unknown aetiology in which there is inflammation and necrosis of skeletal muscle fibres. When the skin is involved it is called dermatomyositis (DM)
what are the clinical features of Polymyositis?
symmetrical progressive muscle weakness effecting proximal muscles, Involvement of pharyngeal, laryngeal and respiratory muscles can lead to dysphagia, dysphonia and respiratory failure. heliotrope (purple) discolouration of the eyelids and scaly erythematous plaques over the knuckles (Gottron’s papules)
Tx Polymyositis
Oral prednisolone is the treatment of choice
what is the classification criteria for Giant cell Arteritis?
over 50, new headache, temporal artery tenderness, reduced pulsation, esr over 50, abnormal temporal biopsy.
GCA (GIANT CELL ARTERITIS)
most common form of vasculitis that occurs in adults. Almost all patients who develop giant cell arteritis are over the age of 50. GCA commonly causes headaches, joint pain, facial pain, fever, and difficulties with vision, and sometimes permanent visual loss in one or both eyes.
