Connective Tissue Disease and Vasculitis Flashcards Preview

Orthopedics & Rheumatology > Connective Tissue Disease and Vasculitis > Flashcards

Flashcards in Connective Tissue Disease and Vasculitis Deck (15):
1

What are the features of scleroderma?

- Types include localised, systemic and CREST

- Systemic invovles lung, renal, GIT and joints

- CREAT includes Calcinosis, Raynaud's, oEsophagus, Sclerodactyly and Telangectasia

- ANA and Scl 70 antibody present

2

What are the features of polymyositis/dermatomyositis?

- Chronic inflamamtion of striated muscle (with rash in case of dermatomyositis)

- Progressive muscle weakness +/- pain, fatigue, interstitial alveolitis or fibrosis of the lungs

3

What are the diagnostic features of polymyositis/dermatomyositis?

- Painful proximal myopathy with weakness

- Evidence of inflammation in the muscle (biopsy/MRI)

- Elevation of muscle enzymes (CK, ALT, LDH)

- Characteristic EMG pattern

- Characteristic JDM rash (in case of dermatomyositis)

4

What are the features of SLE?

- Butterfly rash on face that spaces nose

- Mucosal ulceration (painless)

- Alopecia

- Skin vasculitis

- Capillary dilatation

- Can have systemic involvement

5

How is SLE diagnosed?

- 4/11 of the classification criteria

- Malar rash

- Discoid rash

- Photosensitivity

- Oral ulcers

- Arthritis

- Serositis

- Renal disorder

- Neurological disorder

- Haematological disorder

- Immunological disorder

- ANA

6

How is SLE treated?

- High SPF sunblock

- Anti-malarial drugs

- Topical steroids

- Low dose prednisolone

7

What are the features of Sjorgren Syndrome?

- Dry eyes, dry mouth, salivary gland enlarged and tender, and dry mucosal membranes

- Arthralgia, Raynaud's, neurological neuropathy, kidneys and lungs

- Associated with lymphoma

8

What are the features of Giant Cell Arteritis (large vessel vasculitis)?

- Very common in elderly

- Features include fatigue, headaches, jaw claudication and scalp tenderness

- Test (very high ESR or biopsy)

- Related to polymyalgia rheumatica with presents with pain and stiffness in muscles of neck and shoulder girdle

9

What are the features of Takayasu arteritis (large vessel vasculitis)?

- Younger women (15-25yrs)

- Vascular insufficiency in aorta and large tributaries

- Diagnoses involves imaging antiography and CT

10

What are the features of polyarteritis nodosa (medium vessel vasculitis)?

- Fever and weight loss typical but not specific

- Organ infarction or ischaemia typically include gut, brain, heart, liver, skin, PNS, testis, limbs and kidneys

11

What are the features of Kawasaki disease (medium vessel vasculitis)?

- Children only

- Aneurysm formation in medium to large sized arteries

- Early features include high fever, miserable, mucositis and conjunctivitis

12

What are the features of HSP deposit IgA (immune complex mediated, small vessel vasculitis)?

- Commonest vasculitis in childhood

- Diagnostic triad of palpable purpura, abdominal rash and arthritis

13

What are the features of microscopic polyangitis (ANCA associated, small vessel vasculitis)?

- Perinuclear p-ANCA Myeloperoxidase (MPO) positive

- Five most common manifestations are kidney GN, weight loss, skin lesions, peripheral neuropathy/mononeuritis multiplex and fevers

14

What are the features of granulomatosis with polyangitis (ANCA associated, small vessel vasculitis)?

- Cytoplasmic c-ANCA Proteinase 3 (PR3) positive

- Classically involves upper/lower respiratory tract and kidneys

15

What are the features of eosinophilic granulomatosis with polyangitis (ANCA associated, small vessel vasculitis)?

- Perinuclear p-ANCA Myeloperoxidase (MPO) positive

- Asthma associated disease

- Eosinophils everywhere

- Peripheral neuropathy