conditions Flashcards
myositis muscle pattern involvment?
symmetrical striated muscle involvment
proximal muscles
Extra-muscular signs of dermatomyositis?
- Raynaud’s
- interstitial lung fibrosis
- myocardial involvement (myocarditis, arrhythmias).
- respiratory failure—> diaphragmatic involvment
- upper esophageous–>swalling affected
- aspiration pneumonia—> upper eosphageous affected
Atrial Myxomas can also present with this syndrome
Atrial Myxomas can present with a vasculitis like syndrome:
ECHO can exclude this!
what is this? where do u see it in?
what other sign r assciated with this diseasE?
Myositis plus skin signs:
•Macular rash (shawl sign is +ve if over back and shoulders). •Lilac-purple (heliotrope) rash on eyelids + oedema .
•Nailfold erythema (dilated capillary loops).
•Gottron’s papules: roughened red papules over the knuckles, also seen on elbows and knees
where is Scleroderma renal crisis often seen in?
is a serious condition with features of accelerated hypertension, which can lead to renal failure if not treated promptly. It is seen early in the course of disease in patients with diffuse SSc.
-Often referred to as temporal arteritis (TA)
GCA
Drugs that trigger an SLE-like syndrome
HY PIM
- Hydralazine (antihypertensive)
- Procainamide (antiarryhtmatic)
- Isoniazid (antiTB)
- Miocycline (antiobiotic)
Median age of onset is 72
GCA
what other rheumatoid conditions can develop myositis?
SLE and scleroderma
treatment of Giant cell arteritis
Prednisolone 60–100 mg PO per day for at least 2 weeks before considering tapering down slowly
- For acute onset visual symptoms, consider 1g methylprednisolone IV pulse therapy for the 1–3 days
- Low-dose aspirin therapy to reduce thrombotic risks
SLE
Common symptoms and signs
5-10% life time risk of B cell lymphoma!
Sjogren’s syndrome
what is Morphoea?
Morphoea is a localized form of Scleroderma
What are the primary vasculitides?
Small-vessel vasculitis
- Microscopic polyangiitis (MPA)
- Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome)
- Granulomatosis with polyangiitis (GPA, Wegener’s granulomatosis)
- IgA vasculitis (Henoch-Schönlein purpura)
Medium-vessel vasculitis
- Polyarteritis nodosa (PAN)
- Kawasaki disease (KD)
Large-vessel vasculitis
- Takayasu arteritis (TAK)
- Giant cell arteritis (GCA)
What are spondyloarthropathies?
what r they?
These are a group of conditions that affect the spine and peripheral joints and are associated with the presence of HLA-B27.
SPEAR
- *- Ankylosing spondylitis (most common) - Enteropathic arthritis
- Psoriatic arthritis
- Reactive arthritis**
-enteric arthritis
More common in women and Asian people.
Hypermobility Spectrum Disorder
Extra-articular manifestations of Ankylosing spondylitis
- Anterior uveitis
- Aortic incompetence
- AV block
- Apical lung fibrosis
- Amyloidosis
Crest syndrome? associated with?
Systemic sclerosis
Raynauds syndrome is associated w/ what conditions?
scleroderma
SLE
Dermatomyositis
Polymyositis
Sjogrens syndrome
5 times more common in women
Systemic Sclerosis (SSc)
Ix for SLE
Most patients have raised ESR or plasma viscosity
Anaemia & leukopenia are common
Antibody test >>Anti-Ro & Anti-La are common
Anti- dsDNA titre rises with disease activity
Antiphospholipid antibodies increase the risk of pregnancy loss and thrombosis
C3 and C4 fall with disease activity
Urinalysis >> vital for detecting renal disease
- *Skin biopsy >>** can be diagnostic
- *Renal biopsy >>** can be diagnostic and can help prognosis
Vasculitis Tx?
General Measures: Rule out infection, stop offending drug in secondary causes
- *1st line:** Corticosteroids
- *2nd Line:** Cytotoxic medications, immunomodulatory, or biologic agents (e.g., cyclophosphamide methotrexate, azathioprine, leflunomide mycophenolate mofetil, rituximab, IVIG.
C3 and C4 fall with disease activity
SLE
Pseudogout crystals and type of ppl it effects
risk factor? features, xray finding? Mx
Pseudogout is a form of microcrystal synovitis caused by the deposition of calcium pyrophosphate dihydrate crystals in the synovium.
Risk factors
- haemochromatosis
- hyperparathyroidism
- acromegaly
- low magnesium, low phosphate
- Wilson’s disease
Features
knee, wrist and shoulders most commonly affected
joint aspiration: weakly-positively birefringent rhomboid-shaped crystals
x-ray: c
in the knee this can be seen as linear calcifications of the meniscus and articular cartilage
Management
- aspiration of joint fluid, to exclude septic arthritis
- NSAIDs or intra-articular, intra-muscular or oral steroids as for gout