What is systemic lupus erythematosus?
Systemic autoimmune disease caused by type 3 hypersentivity reaction.
i.e. soluble antigens & antibodies expressed in equal amounts, resulting
in extensive cross linking & formation of large immune complexes
What is sclerodactyly?
This is tightening and hardening of skin over the fingers and hands, seen in SLE.
What is telangiectasia?
Telangiectasias (also known as spider veins) are small dilated blood vessels near the surface of the skin or mucous membranes, measuring between 0.5 and 1 millimeter in diameter.
What is polymyositis?
T Cytotoxic cells invade and destroy healthy muscle fibres.
Only occurs in adults.
Autoimmune disease - invasion of muscle by white cells.
What is dermatomyositis?
This is an automimmune disease affecting muscle tissue. It occurs in children and adults. Produces characteristic skin lesions, heliotrope eyelids, and gottrons papules (plaques on knuckles). It affects both muscles and skin.
Look at this image. What condition is suspected?
Raised creatine kinase indicates that what type of tissue has been damaged?
What drug type is used in the treatment of conditions such as dermatomyositis and polymyositis? Give specific examples.
Immunosuppressants, I.E Azathioprine or Methotrexate
Can usually be withdrawn after 1-2 years.
What is Sjorgren syndrome?
Chronic autoimmune inflammatory disorder, with lympohcytic infiltration of exocrine organs. Mainly affects lacrimal glands. Salivary/parotid glands +/- nasal, vaginal, skin glands.
This is often secondary to RA/SLE, Mainly affects middle aged females.
What is Sicca syndrome? *CLUE*: It's often associated with Sjorgen syndrome.
Dry eyes, mouth, vagina, oral cavity (basically mucous membranes are all dry).
What is the Schirmer test?
Filter paper folded over lower eyelid. Should wet >15mm in 5 mins
+ve test = <5mm in 5mins
Which antibodies are often present in Sjorgen syndrome?
Anti - Ro/Anti LA antibodies
What is rheumatoid arthritis?
Chronic, Systemic Inflammatory Disorder caused by an Autoimmune aetiology.
In three words, which group is most likely to suffer from rheumatoid arthritis?
40's Female smoker.
What is rheumatoid vasculitis?
Inflammation of small vessels causing occlusion/ischaemia
What is mononeuritis multiplex?
Mononeuritis multiplex is a painful, asymmetrical, asynchronous sensory and motor peripheral neuropathy involving isolated damage to at least 2 separate nerve areas.
Multiple nerves in random areas of the body can be affected. As the condition worsens, it becomes less multifocal and more symmetrical. Mononeuropathy multiplex syndromes can be distributed bilaterally, distally, and proximally throughout the body.
What is Caplans syndrome?
RA + Pneumoconiosis (Occupational lung dis. from dust inhalation)
What is Felty's syndrome?
RA + Splenomegaly + Leukopenia
Anti CCP (Cyclic Citrillunated Peptide) is specific for which autoimmmune condition?
What are the treatments for R. Arthritis?
What must be given in conjunction with methotrexate in patients with RA?
Folate. Folate antagonism is not a desired effect in treatment of RA.
What is Sulfasalazine, and how does it work?
Sulfasalazine is an immunosuppressive medicine. It helps to suppress overactivity of the immune system in rheumatoid arthritis. It can help to reduce pain and swelling by limiting inflammation.
This suppresses IL-1, TNF and other immune cells.
Side effects include oligospermia, and rashes.
What is Leflunomide, and how does it work?
This is an immunosuppressive DMARD. It is a pyrimidine synthesis inhibitor.
Liver damage, lung damage and immunosuppression are all dose-limiting side effects.
Do NOT USE WITH METHOTREXATE - OR ELSE IT IS GOODBYE LIVER
Name two anti-TNF agents.
Infliximab, adalimumab, etanercept
What is the treatment ladder for rheumatoid arthritis?
Step 1 - Analgesia, NSAIDs, Steroids + 1 DMARD
Step 2 - Above + 2nd DMARD
Step 3 - Consider use of Biologics
What is a type 3 hypersensitivity reaction?
This is immune complex mediated hypersensitivity.
These complexes cause cytokine activity, causing massive infiltration by neutrophils in inflammatory response.
What is this rash called? What disease is it associated with?
This is a Malar/butterfly rash
What does this image show? Which condition is it associated with?
This is a Discoid rash. It is associated with SLE.
Hydroxychloroquine is a drug used to treat mild forms of which condition?
Which two immunosupressant drugs are most commonly used in the treatment of SLE?
Cylophosphamide and Mycophenolate (DMARDS)
What is Scleroderma?
This is a localised, cutaneous disease, causing only fibrosis and tightening of the skin.
Commonlhy affects the fingers and face.
Which antibody is linked to CREST syndrome (limited cutaneous systemic sclerosis)?
Which antibody is linked to diffuse cutaneous systemic sclerosis?
Anti scl-170 antibody
Which antibodies are usually associated with Sjorgren syndrome?
Anti-RO and Anti-La antibodies
What is Sjogren syndrome?
This is a chronic autoimmune condition. There is lymphocytic infiltration of exocrine organs, mediated by < T helper and B cells.
The antibodies damage epithelial cells, and AcH release by nerves.
Net result: They don't work!
Mainly affects lacrimal, salivary/parotid glands +/- nasal, vaginal etc
What deformity is shown here?
This is a swan neck deformity, seen is rheumatoid arthritis.
Hyperextension of PIP and fixed flexion of DIP.
What is this clinical sign?
This is Boutonniere deformity - it is seen in Rheumatoid arthritis. Fixed flexion of PIP and hyperextension of DIP.
What is mononeuritis multiplex?
This is a complex group of disorders - more of a syndrome than a specific diagnosis.
It is a painful, asymmetrical, asynchronous sensory and motor peripheral neuropathy involving isolated damage to at least 2 separate nerve areas. Multiple nerves in random areas of the body can be affected. As the condition worsens, it becomes more symmetrical.
Aetiology is related to the underlying condition.
Usually treated by treating the underlying condition.
What does Leflunomide do in the treatment of Rheumatoid Arthritis?
This drug inhibits lymphocyte proliferation. It is a DMARD.
It is hepato-toxic, so must be careful when using.
what is vasculitis?
This is inflammation and necrosis of a blood vessel, with impariment of the circulation.
What is Henoch-Schonlein purpura/what causes it?
Palpable purpura of the arms and legs and torso - most common vasculitis in chidren 3-15 YOA.
This is an IgA modulated auto-immune condition.
There is lower limb predominance, and it is usually without coagulopathy or thrombocytopaenia.
What clinical sign is this and which condition is it related to?
This is livedo reticularis. This is related to vasculitis.
Which clinical sign is this?
This is urticaria.
Notice itchy wheal and flare type skin lesions and/or angioedema (swelling of deeper skin layers).
Often called "hives".
What is the mainstay treatrment for large vessel vasculitis?
What is the mainstay treatment for medium veseel vasculitis?
Steroids and Immunosuppressants (like cyclophosphamide)
Except in Kawasaki's: Contra indicated due to high aneurysm rate.
What is Kawasaki's vasculitis?
This is an idiopathic self-limiting systemic vasculitis that most often affects children in the age range 6 months to 5 years. Deaths and significant morbidity occur through its major complication of coronary artery aneurysm formation. It has taken over from rheumatic fever as the most common cause of acquired childhood heart disease in the developed world.
Classical features of Kawasaki disease
Fever lasting ≥5 days.
Marked irritability of the child.
Erythema, swelling and desquamation affecting the skin of the extremities.
Inflammation of the lips, mouth and/or tongue.
What is Giant cell arteritis?
- This is the most common artertitis. Sometimes called temporal arteritis.
- Associated with polymyalgia rheumatica.
- Presents in Elderly women/men less often
- If untreated - can cause vision loss
- Often present with drastci change in vision
- Empirically treat with glucocorticoids (prednisolone 40mg Once daily usually)
What is Takayasu's arteritis?
- Presents in adolescent/young women
- Granulomatous inflammatin of aorta
- Causes stenosis, thrombosis, aneurysyms and fibrosis
- Diagnosed with Arteriography or echocardiography
- Two stages - initial inflammatory phase (generally unwell) and later pulseless phase (pulseless upper limbs, hypertension due to renal artery stenosis, and neuro symptoms due to > blood flow)
- Treatment is high dose steroids
What is the treatment for Kawasaki Disease?
IV Immunoglubulins - this is because steroids are contraindictaed - the risk an dincrease chance of coronary aneurysm.
What is Wegener's Granulomatosis?
- European middle aged men
- Triad of necrotising vasculitis, necrotising inflammation of the respiratory tract and necrotising glomerulonephritis.
- Symptoms: Fever, saddle nose deformity, otitis media and hearing loss, pulmonary symptoms, symptoms of glomerulonephritis
- < CRP and < PR-3-ANCA
- Treated with Steroids and immunosuppressants.
What deformity is shown on the right? Which autoimmune small vessel disease is this a clinical sign of?
This is a saddle nose deformity, seen is Wegener's granulomatosis.
Which small/medium vascular disease is strongly associated with Hepatitis B virus?
What is polyarteritis nodosa?
- Described as the "original vasculitis"
- Middle aged men
- Not an easy diagnosis
- Hep B strongly associated
- necrotising inflammatory lesions occur AT BIFURCATIONS of small arteries.
- Vague presentation, may have peripheral neuropathy and or renal disease
- Treatment is steroids and immunosuppressant
What is Churg-Strauss syndrome?
- This is a complicatino of asthma - may present on withdrawal or reduction of steroids
- Usually in middle age
- Three phases:
- Allergic rhinitis and asthma
- Peripheral neuropathy, renal problems, arthralgia, skin manifestations
- Blood will show high Eosinophils pANCA and IGe levels.
- Steroids usually adequate treatment.
What is Hypersensitivity (AKA Leukocytoclastic) vasculitis?