Multisystem autoummine diease

Question 1

what may it mimic?

Answer 1

drugs, infection, malignancy, cardiac myxoma, cholestrol emboli, scurvey

Question 2

What is SLE (systemic Lupus erythemetosus)?

Answer 2

SLE is an inflammatory multisystem disease characterized by the presence of serum antibodies against nuclear components.

Question 3

epidemiology sle

Answer 3

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.

Question 4

aetiology sle

Answer 4

the cause of the disease is unknown but probably multifactorial:hereditary, genetics, sex hormone status/ kleinfelters syndrome, drugs, uv light, eb virus exposure.

Question 5

clincial features of SLE

Answer 5

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.

Question 6

Ix sle

Answer 6

Raised anti-dsDNA antibody,

Low serum complement C3 and C4,

Raised anti-SM antibody, anti-Ro, anti-La, histology, ANA, bloods,

ESR raised.

Question 7

Tx sle

Answer 7

NSAIDs, corticosteroids,

Chloroquine and hydroxychloroquine,

immunosupressives, cyclophosphamide, steroid-sparring drugs such as azathioprine and methotrexate.

Question 8

what is Scleroderma (SYSTEMIC SCLEROSIS)?

Answer 8

Systemic sclerosis (scleroderma) is a multisystem disease with involvement of the skin and Raynaud’s phenomenon.

Question 9

RF’s of scleroderma

Answer 9

exposure to Silica dust or Vinyl chloride.

Question 10

epidemiology of scleroderma

Answer 10

It is three times more common in women than in men and usually presents between the ages of 30 and 50 years.

Question 11

Clinical features of Limited Cutaneous Scleroderma?

Answer 11

CREST - Calcinosis, 
Raynaud's (spasm of the arteries supplying the fingers and toes, leading to cold extremities), 
Eosophageal involvement (dysphagia)
Sclerodactyly, 
Telaniectasia

Question 12

Clinical features of Diffuse Scleroderma?

Answer 12

small bowel bacterial overgrowth, acute chronic kidney disease, pulmonary fibrosis, myocardial fibrosis. (RENAL, CARDIO, RESP)

Question 13

how is the onset of diffuse cutaneous scleroderma different to the onset of limited cutaneous scleroderma?

Answer 13

limited starts with Raynaud’s, whereas diffuse begins with oedema before skin sclerosis follows.

Question 14

Mx of scleroderma

Answer 14

tx raynauds, educate, skin care, immunosupression, nutritional support, tx of complications.

Question 15

how is Raynaud’s managed?

Answer 15

oral vasodilators - CCB’s, ACEi, sympathectomy, hand warmers, battery powered gloves.

Question 16

Sjogren’s Syndrome

Answer 16

characterized by immunologically mediated destruction of epithelial exocrine glands, especially the lacrimal and salivary glands. It predominantly affects middle-aged women.

Question 17

clincial features Sjogren’s Syndrome

Answer 17

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.

Question 18

Ix Sjogren’s Syndrome

Answer 18

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)

Question 19

Tx Sjogren’s Syndrome

Answer 19

artifical tears, eye ointment, canaliculi occlusion, artifical saliva spray, tx secondary infections, hydroxychloroquinine may help associated arthritis, Corticosteroids if there are systemic sy present.

Question 20

What is Polymyositis?

Answer 20

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)

Question 21

what are the clinical features of Polymyositis?

Answer 21

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)

Question 22

Tx Polymyositis

Answer 22

Oral prednisolone is the treatment of choice

Question 23

what is the classification criteria for Giant cell Arteritis?

Answer 23

over 50, new headache, temporal artery tenderness, reduced pulsation, esr over 50, abnormal temporal biopsy.

Question 24

GCA (GIANT CELL ARTERITIS)

Answer 24

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.

Question 25

Sy/Sx GCA

Answer 25

scalp tenderness, sudden loss of vision, jaw claudication, rubor over temporal a., loss of apatite, headaches, joint pain, facial pain, fever, and difficulties with vision, and sometimes permanent visual loss in one or both eyes.

Question 26

Ix GCA

Answer 26

Raised ESR, temporal a, biopsy, raised crp,

Question 27

Tx GCA

Answer 27

Prednisolone.

Question 28

outline ANCA associated vasculitis

Answer 28

wegeners - involves urt and lrt, necrotising glomerulonephritis
microscopic polyangiitis, eosinophilic granulomatosis.

Question 29

lupus nephritis classes?

Answer 29

1- minimal mesangial
2 - mesangial proliferative
3 - focal/ 4- diffuse
5 - membranous
6 - advanced sclerosing

Question 30

Multisystem autoimmune diseases Tx

Answer 30

Mild - hydrochloroquine
moderate - azathioprine, methotrexate, mycophenolate
severe - cyclophosphamide, rituximab

Question 31

Case 1

Answer 31

45 Y female presents with 3/7 Hx difficulty breathing and right sided chest pain worse with deep inspiration. Her CXR confirms right pleural effusion. Treatment with antibiotics makes no difference. Her FBC showed persistently low WCC of 3.0 then 3.2 and low platelets of 100. In the last year she has been experiencing intermittent pain and swelling in her joints and recurrent facial rash after sun exposure .
diagnosis? scleroderma/ sle/ gca/ none of the above
bedside assessment prioritised? bp/ fundoscopy/ auscultation for bruits/ urine dipstick
which immunological test would support clinical suspicion? +ve dsDNA/ reduced complement levels/ positive smith antibodies/ positive anti-nuclear antibodies.
tx? NSAIDs, corticosteroids, Chloroquine and hydroxychloroquine, immunosupressives, cyclophosphamide.

Question 32

what Disorder is GCA associated with?

Answer 32

Polymyalgia Reumatica - disorder of connective tissue