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
name Large vessel Vasculitis & Medium Vessel
LARGE >>>> GCA & Takayasu arteritis
MEDIUM>>PIK
- Polyarteritis Nodosa
- Kawasaki Disease
- Isolated Central Nervous system vasculitis
Absence of Raynaud’s phenomenon makes the diagnosis very unlikely
systemic sclerosis
What is systemic sclerosis?
Multisystem autoimmune disease.
Previously known as scleroderma.
Increased fibroblast activity resulting in abnormal growth of connective tissue which leads to vascular damage and fibrosis.
Enteropathic arthritis
2 types of peripheral disease?
o Type 1 = oligoarticular, asymmetric & has a correlation w/ IBD flares
o Type 2 = polyarticular symmetrical & less correlation with IBD flares
Ractive arthritis symptoms
teleangiectasis
is a condition in which widened venules (tiny blood vessels) cause threadlike red lines or patterns on the skin.
telangiectases form gradually and often in clusters.
They’re sometimes known as “spider veins” because of their fine and weblike appearance
well demonstrated on the MRI
myositis
Antiphospholipid syndrome
Hypercoagulble state!!!!
CLOT
Coagulation defect (arterial/venous)
Livedo reticularis
Obstetric (recurrent miscarriage)
Thrombocytopenia.
Thrombotic tendency affects cerebral, renal, and other vessels
Dx: Persistent antiphosphlolipid antibodies with clinical features
Tx: Anticoagulation; seek advice in pregnancy
Headache is the most frequent symptom (70%)
GCA
Osteoporosis tx
Vitamin D ± calcium supplementation plus:
1st line: Oral bisphosphonates, or IV if oral not tolerated.
2nd line: Denosumab or teriparatide
many ptx have normal inflammatory markers?
dermatomyositis & polymyositis
ESR and plasma viscosity are raised in most patients but CRP is normal
SLE
most feared complication of GCA?
Permanent visual loss
what is Z-score?
is a comparison of the patient’s BMD with an age- & gender-matched population.
- A Z-score <−2 should prompt evaluation for causes of secondary osteoporosis.
Causes of positive ANA
- Auto-immune rheumatic diseases e.g. SLE, RA, Sjogren’s sydrome
- Other auto-immune diseases e.g. ITP
- Chronic liver disease
- Chronic infection
- Malignancy
- Drug-induced e.g. minocycline
Felty’s syndrome
RA
what genetic predisposition is GCA associated with?
HLA-DR24
Biopsy is characteristic
Sjogren’s syndrome
focal lymphocytic infiltration of exocrine glands.
types of Systemic Sclerosis (SSc)?
Diffuse Scleroderma & Limited Scleroderma
Giant cell arteritis –> inflammation of arteries supplying what areas mainly
supplying the
muscles of mastication results in jaw
claudication and tongue discomfort
what is this? which condition associated w/?
keratoderma blennorrhagica
REactive arthritis
plaques on soles/palms
Features of inflammatory back pain
IPAIN!
Insidious onset
Pain at night (with improvement on getting up)
Age at onset < 40 Improvement with exercise
No improvement with rest
Tx SLE
- Sun protection
- Advice on healthy lifestyle due to CVD
- Hydroxychloroquine is helpful for rash and arthralgia
- Mycophenolate mofetil, azathioprine & rituximab are commonly used too
Short courses of prednisolone for flares
Ptx have increased risk of malignancy escpecially in the 2-3 years before and after the diagnosis of it?
Dermatomyositis
Antibodies in Systemic sclerosis
- Positive ANA in 90% of patients
- Anti-centromere antibody strongly associated with limited SSc.
- Scl-70 (topisomerase) and anti RNA polymerase III antibodies strongly associated with diffuse SSc.
Sjogren’s syndrome
S & S
mnemonic –madfred!
- Myalgia
- Arthralgia
- Dry Mouth
- Fatigue
- Raynaud’s phenomenon
- Enlarged parotids
- Dry eyes
Associated with other autoimmune conditions
Sjogren’s syndrome
Coeliac disease, Primary Biliary Cirrhosis, Auto immune thyroid disease.
If there is not a dramatic and rapid response to steroids, reconsider the diagnosis.
Polymyalgia Rheumatica
Reactive arthritis
- definition
- presentation
- other features
- Ix
Sterile synovitis developing after a distant infection either post dysentery (Salmonella / Shigella / Campylobacter) or following urethritis/cervicitis (Chlamydia trachomatis)
- Presentation: few days – 2 weeks post infection, acute asymmetrical lower limb arthritis develops.
- Other features: skin (circinate balanitis, keratoderma blennorrhagica), eye (conjunctivitis, uveitis), enthesitis.
- Ix: serology/microbiology, inflammatory markers raised,
may need joint aspirate to rule out septic/crystal arthritis
Tx of Raynauds
complication?
All patients should be advised to keep warm & avoid smoking.
1st line: Ca+ channel blockers
PDE 5 inhibitors, and prostacyclins are usually effective.
There is a weak evidence base for angiotensin receptor antagonists, ACE inhibitors, SSRIs, systemic and topical nitrates.
Complications in RP include digital ulcers, severe digital ischaemia and infection.
what imaging is useful in DM and scleorderma? why?
Nail-fold capillaroscopy is non-invasive
This imaging tool enables evaluation of characteristic structural changes in the peripheral microcirculation
what r the risks for osteoporotic fractures
FRACTURES
FH, RA, Alcohol, cigarette, thin, UC (IBD), reduced mobility, endocrinopathies, steroids
what do we use to capture those at risk with osteoporosis?
FRAX TOOL
gold standard diagnosis for osteoporosis?
Dual eneregy X-ray absorptiometry (DEXA) of lumbar spine and hip
Fibromyalgia symp
Triad of
- poor sleep
- fatigue
- pain everywhere
Ix of Systemic sclerosis
Inflammatory markers normal
xray hands>> calcinosis hands
PFT, HRCT
ECO and Ecg
antobodies
Typical systemic sclerosis face
tapered nose
Pursed mouth
