Rheumatology (2) Flashcards
Simply, what’s myositis and dermatomyositis
Striated muscle inflammation
(striated muscle in cardiac and skeletal muscles)
General features of polymyositis and dermatomyositis
Progressive symmetrical proximal muscle weakness
- Wasting of the shoulder and pelvic girdle
- Dysphagia, dysphonia, respiratory weakness
Myalgia and arthralgia
Often a paraneoplastic phenomenon
-Lung, pancreas, ovarian, bowel cancers
Simply, what’s dermatomyositis
Dermatomyositis = myositis + skin signs
Presentaiton of dermatomyositis
Dermatomyositis = myositis + skin signs
- Heliotrope rash (redish/purple rash) on eyelids ± oedema
- Macular rash (shawl sign +ve: over back and shoulders)
- Nailfold erythema
- Gottron’s papules: knuckles, elbows, knees
- Mechanics hands: painful, rough skin cracking of fingertips
- Retinopathy: haemorrhages and cotton wool spots
- Subcutaneous calcifications
Extra-muscular features of dermatomyositis and myositis
- Fever
- Arthritis
- Bibasal pulmonary fibrosis
- Raynaud’s phenomenon
- Myocardial involvement: myocarditis, arrhythmias
Ix for myositis/dermatomyositis
- Muscle enzymes: ↑CK, ↑AST, ↑ALT, ↑LDH
- Antibodies: Anti-Jo1 (associated with extra-muscular features)
- EMG
- Muscle biopsy
- Screen for malignancy
- Tumour markers
- CXR
- Mammogram
- Pelvic/abdo US
- CT
Management of dermatomyositis/myositis
- Screen for malignancy
- Immunosuppression
- Steroids
- Cytotoxics: azathioprine, methotrexate
What’s another name for Sjorgen’s syndrome?
Sjogren’s Syndrome = Keratoconjunctivitis Sicca
Classification of Sjorgen’s syndrome (2)
- ↓ tear production → dry eyes
- ↓ salivation → xerostomia
- Bilateral parotid swelling
- Vaginal dryness → dyspareunia
- Systemic
- Polyarthritis
- Raynaud’s
- Bibasal pulmonary fibrosis
- Vasculitis
- Myositis
Diseases associated with Sjorgen’s syndrome
- Autoimmune disease: thyroid disease, autoimmune haemolytic anaemia, PBC
- Marginal zone MALT lymphoma
Ix of Sjorgen’s syndrome
- Schirmer tear test
- Antibodies: ANA – Ro and La, RF
- Hypergammaglobulinaemia
- Parotid biopsy
Management of Sjorgen’s syndrome
- Artificial tears
- Saliva replacement solutions
- NSAIDs and hydroxychloroquine for arthralgia
- Immunosuppression for severe systemic disease
Examples of large vessel vasculitis (2)
Large Vessel
- Giant Cell Arteritis
- Takayasu’s Arteritis
Examples of medium vessel vasculitis
Medium Vessel
- Polyarteritis Nodosa
- Kawasaki Disease
Examples of small vessel pANCA vasculitis (2)
pANCA
- Churg-Straus
- Microscopic Polyangiitis
Example of small vessel cANCA vasculitis (1)
cANCA: Wegener’s Granulomatosis
Examples of small vessel ANCA -ve vasculitis (4)
ANCA –ve
- Henoch-Schonlein Purpura
- Goodpasture’s Disease
- Cryoglobulinaemia
- Cutaneous Leukocytoclastic Vasculitis
