Rheumatology
Rheumatology
Rheumatology
صالح دعائكم
Please Grace us with your good prayers
يوسف معيوف
Youssef Maayouf
What are the Extractable Nuclear
Antigens?
• Specific nuclear antigens
• Usually associated with being ANA positive
What is Anti-Ro?
• Lead toxicity
What are the causes of increased uric acid
production?
• Myeloproliferative/lymphoproliferative disorder
• Cytotoxic drugs
• Severe psoriasis
If you have a patient with Gout, but taking Aspirin
75-150 mg for cardio protection, should you
continue the aspirin or discontinue?
• Continue
What is Lesch-Nyhan syndrome?
• Thiazides, furosemide
• Alcohol
• Cytotoxic agents
• Pyrazinamide
Lithium was actually used to treat gout in the
19th century, T/F?
• True
What should be your Acute management for
gout?
• NSAIDs
• Colchicine has a slower onset of action. (main side-effect is diarrhea and INR with warfarin)
• If the patient is already taking allopurinol (drug used to treat gout generic name Zyloric/No-Uric)it should be
continued
• Rasburicase: is a recombinant version of a urate oxidase enzyme given in acute setting, it allows allopurinol
to be commenced without worsening of symptoms. Only used when other Rx can not be given
When should you start Allupurinol for
treatment of gout?
• Gout: start allopurinol if >= 2 attacks in 12 month period
What are the guidelines for giving
Allupurinol for gout?
• Allopurinol should not be started until 2 weeks after an acute attack
has settled.
• Initial dose of 100 mg od, with the dose titrated every few weeks to
aim for a serum uric acid of < 300 μmol/l
• Tophi
• Renal disease
• Avoid food high in purines e.g. Liver, kidneys, seafood, oily fish
(mackerel, sardines) and yeast products
What is Pseudogout?
• Hyperparathyroidism
• Hypothyroidism
• Hemochromatosis
• Acromegaly
• Decreased magnesium, decreased phosphate
• Wilson's disease
Which investigations might be positive with a
case of Pseudogout?
• Transferrin saturation (may indicate hemochromatosis, a recognised
cause of pseudogout)
What should be your management for a
patient with Pseudogout?
• Aspiration of joint fluid, to exclude septic arthritis and show weakly-
positively birefringent brick shaped crystals
• Prevalence in UK = 1%
• Juxta-articular osteoporosis
• Soft-tissue swelling
What are the early x-ray findings for
Rheumatoid arthritis?
• Periarticular erosions (osteopenia and osteoporosis)
• Subluxation
What are the Poor prognostic features for
Rheumatoid arthritis?
• Rheumatoid factor positive
• Positive HLA DR4
• Anti-CCP antibodies
• female sex
• Poor functional status at presentation
• Insidious onset
• X-ray: early erosions (in < 2 years)
• Extra articular features e.g. Nodules
Rheumatoid arthritis: patients have an
increased risk of IHD, T/F?
• True
What are the Extra-articular complications
that occur in patients with rheumatoid
arthritis (RA)?
• Respiratory: pulmonary fibrosis, pleural effusion, pulmonary nodules,
bronchiolitis obliterans, methotrexate pneumonitis, pleurisy
• Ocular: keratoconjunctivitis sicca (most common), episcleritis, scleritis, corneal
ulceration, keratitis, steroid-induced cataracts, chloroquine retinopathy
• Osteoporosis
• ISCHEMIC heart disease: RA carries a similar risk to Type2 DM
• Increased risk of infections
• Depression
Proteus mirabilis is a (G-ve rod), causes UTI
predisposes susceptible patients to RA, T/F?
• True
Felty's syndrome (RA + splenomegaly + low
white cell count)and Amyloidosis are
complications of Rheimatoid arthritis, T/F?
• True
What is Felty’s syndrome?
• Felty’s syndrome usually occurs in patients with long-standing seropositive RA.
• Two-thirds of the patients are females. The clinical features include RA,
leucopenia, lymphadenopathy and splenomegaly. Leg ulcers, recurrent
infections and episcleritis may be present.
• Treatment includes splenectomy, disease-modifying anti-rheumatic drugs
(DMARDs) including in severe disease use of Pulsed corticosteroid and/or
cyclophosphamide, and colony stimulating factor to stimulate production of
granulocytes.
• ANA is positive in more than 90% of patients with Felty’s.
What is the first treatment you should give to
a patient with Rheumatoid arthritis?
• Patients with evidence of joint inflammation should start a
combination of disease-modifying drugs (DMARD) as soon as possible.
• Caplan's syndrome - massive fibrotic nodules with occupational coal dust exposure
• Infection (possibly atypical) secondary to immunosuppression
What is Adult Still's Disease?
• Arthralgia
• Fever (noticeable at afternoon and evening), Pyrexia
• Elevated serum ferritin
• Rash: salmon-pink, maculopapular, pruritic
• Lymphadenopathy
• RF and ANA negative (but ANA 25% positive). elevated ESR and CRP
• Steroids
• Methotrexate
Joint aspiration is mandatory in all
patients with a hot, swollen joint to rule
out septic arthritis, T/F?
• True
• Joint aspiration is mandatory in all patients with a hot, swollen joint
to rule out septic arthritis.
• If this was excluded then intra-articular or system steroid therapy
may be considered
What is the most common organism causing
septic arthritis?
• Most common organism overall is Staphylococcus aureus
• Antibiotic treatment is normally be given for several weeks (BNF states 6-12 weeks)
• "the patient can't see, can't pee, can't bend the knee"
What are the features of Reiter’s syndrome
(reactive arthritis)?
• Typically develops within 4 weeks of initial infection - symptoms generally last
around 4-6 months
• Arthritis is typically an asymmetrical oligoarthritis of lower limbs May present
as monoarthritis e.g. Knee
• Symptoms of urethritis
• Eye: conjunctivitis (seen in 50%), anterior uveitis
• Skin: circinate balanitis (painless vesicles on the coronal margin of the prepuce),
keratoderma blenorrhagica (waxy yellow/brown papules on palms and soles)
What is the prognosis of patients with
Reiter’s syndrome?
• Around 25% (15-50%) have recurrent episodes whilst 10% (15-30%)
develop chronic disease.
Ankylosing spondylitis develop in up to
50% of HLA B27 +ve patients, T/F?
• True
The American College of Rheumatology now define
reactive arthritis as an episode of peripheral
arthritis lasting for greater than 1 month associated
with urethritis/cervicitis or diarrhea, T/F?
• True
What is the Epidemiology of reactive
arthritis?
• Post-STI form much more common in men (e.g. 10:1)
• Shigella flexneri
• Salmonella typhimurium
• Salmonella enteritidis
• Yersinia enterocolitica
• Campylobacter
What are the Organisms that are often
responsible for post-STI
form of Reactive arthritis?
• Chlamydia trachomatis
What should be your management for a
patient with reactive arthritis?
• Symptomatic: analgesia, NSAIDs, intra-articular steroids
• It typically presents in s (sex ratio 5:1) aged 20-30 years old. It has
polygenic inheritance.
Can HLA-B27 used for the diagnosis of
Ankylosing Spondylitis?
• HLA-B27 is of little use in making the diagnosis as it is positive in: 90%
of patients with ankylosing spondylitis
• CNS- Seizures
• Skin- Butterfly Rash, Discoid Lupus
• Heart- Endocarditis, Myocarditis
• Glomerulonephritis
• Serositis pleuritic
• Haematologic- Haemolytic, Leukopenia, Thrombocytopenia
• Arthritis
• Lymphadenopathy
What is the Epidemiology of SLE?
• Autoimmune disease
• Associated with HLA B8, DR2, DR3
• Thought to be caused by immune system dysregulation leading to
immune complex formation
• Immune complex deposition can affect any organ; skin, joints, kidneys
and brain most commonly affected
What is the Immunological features of SLE?
• True
Which tests will you use to monitor the
disease activity for a patient with SLE?
• ESR: during active disease the CRP is characteristically normal - a
raised CRP may indicate underlying infection
• Complement levels (C3, C4) are low during active disease (formation
of complexes leads to consumption of complement) C4 is early
marker for disease activity
• Anti-DsDNA titers: used for disease monitoring disease activity (but
not present in all patients)
IN SLE, what is the early marker for disease
activity ?
• C4
In active SLE disease, CRP is usually raised,
T/F?
• False
• ESR: during active disease the CRP is characteristically normal - a
raised CRP may indicate underlying infection
SLE gets better with pregnancy, T/F?
• False
• Unlike many autoimmune diseases systemic lupus erythematous
(SLE) often becomes worse during pregnancy and the puerperium
What are the problems of SLE with
pregnancy?
• Risk of maternal autoantibodies crossing placenta
• It has a similar features to SLE but not all the typical features of
systemic lupus erythematosus are seen, with renal and nervous
system involvement being unusual.
• This condition results from ischemia of the optic nerve head which is
supplied by the posterior ciliary arteries and typically presents with a
chalky white oedematous optic disc.
Which investigations should you order for a
Temporal Arteritis patient?
• ESR > 50 mm/hr (note ESR < 30 in 10% of patients). CRP may also be
elevated
• Temporal artery biopsy: skip lesions may be present
• Note CK and EMG normal
• Decreased CD8+ T cells
How should you manage a patient with
Temporal Arteritis?
• Should be started immediately with high dose steroids (e.g. prednisolone
1mg/kg/day) to decrease the chance of visual loss.
• there should be a dramatic response, if not the diagnosis should be reconsidered
• Urgent ophthalmology review. Patients with visual symptoms should be seen the
same-day by an ophthalmologist. Visual damage is often irreversible
What is Takayasu disease (TD)?
• Churg-Strauss Syndrome
What are the features of Churg Strauss
syndrome?
• Asthma and sometimes pulmonary esinophilic infiltrate
• Blood eosinophilia (e.g. > 10%)
• Paranasal sinusitis
• Mononeuritis multiplex
• Polyarthritis
• Features of IgA nephropathy may occur e.g. Hematuria, renal failure
• Please notice that IgA nephropathy (Berger’s disease) is different
from Thrombangiitis Obliterans (B uerger’s disease)
What is Thrombangiitis Obliterans
(Buerger’s disease)?
• Thrombangiitis Obliterans (Buerger’s disease) is a disease of small
and mediumsized arteries and veins resulting in inflammation and
ulceration.
What are the features of Thrombangiitis
Obliterans (Buerger’s disease)?
• No excessive atheroma
• Does not involve the coronary arteries like atherosclerosis.
• Occurs mainly in cigarette smokers; it has not been documented in non-smokers
• Patients present with symptoms of arterial ischemia.
• Migratory phlebitis in the superficial vein is present in 40% of cases.
• The disease progresses proximally, resulting in gangrene of the digits.
What is the diagnosis and treatment of
Thrombangiitis Obliterans (Buerger’s
disease)?
• Diagnosis is usually clinical.
• Arteriogram is also of benefit and will show occlusion of distal arteries
of the hands and feet.
•Steroids
•Azathioprine
Antiphospholipid syndrome: (paradoxically)
prolonged APTT + low platelets, T/F?
• True
What is Antiphospholipid Syndrome?
• Lymphoproliferative disorders
• Phenothiazines (rare)
What is the management of
Antiphospholipid Syndrome?
• Initial venous thromboembolic events: evidence currently supports use of
warfarin with a target INR of 2-3 for 6 months
• Arterial thrombosis should be treated with lifelong warfarin with target INR 2-3
What are the complications of
Antiphospholipid Syndrome in pregnancy?
• Recurrent miscarriage
• IUGR
• Pre-eclampsia
• Placental abruption
• Pre-term delivery
• Venous thromboembolism
What is the management of
Antiphospholipid Syndrome in pregnancy?
• Low-dose aspirin should be commenced once the pregnancy is confirmed on
urine testing
• Low molecular weight heparin once a fetal heart is seen on ultrasound.
•
• This is usually discontinued at 34 weeks gestation
• High ESR
What is the treatment of Cryoglobulinemia?
• Immunosuppression
• Plasmapheresis
What is Raynaud's Phenomena?
• IV prostacyclin infusions
Some drugs may exacerbate Raynaud's
Phenomena give example?
• Methysergide
• Atenolol
What is Morphea?
• In circumscribed morphea, there may be just one or two lesions with no generalized spread.
• Changes often begin with small, violaceous, or erythematous skin lesions, which enlarge and
progress to firm hidebound skin with a variable degree of hypo- or hyperpigmentation.
• Lesions eventually settle into a waxy, white appearance with subsequent atrophy.
• Lesions vary in diameter between 1 and 10 cm.
• Condition generally resolves within 3-5 years, although sometimes a patch may persist for over
25 years.
What is the tests for Morphea (localized form
of scleroderma)?
• Auto-antibodies such as anti-nuclear antibody (ANA) are only rarely
positive in localized forms of scleroderma, as against systemic
subtypes where a positive ANA is one of the hallmarks of the disease.
What do you know about Psoriatic
Arthropathy?
• Psoriatic Arthropathy correlates poorly with cutaneous psoriasis and often
precedes the development of skin lesions.
• Around 10% of patients with skin lesions develop an arthropathy with males and
females being equally affected
What are the types of Psoriatic
Arthropathy?
• Rheumatoid-like polyarthritis: (30-40%, most common type)
• Asymmetrical oligoarthritis: typically affects hands and feet (20-30%)
• Sacroilitis
• DIP joint disease = arthropathy (10%)
• Sickle-cell disease
• Poor prognosis
What is Scleroderma (without internal
organ involvement) (one of the patterns of
Systemic Sclerosis)?
• Tightening and fibrosis of skin
• RF positive in 30%
• The skin tightness may be treated systemically with methotrexate and ciclosporin
How to treat Severe digital ulceration
resulting from Systemic Sclerosis?
• Severe digital ulceration in such patients can be treated with infusion
of a prostacyclin analogue such as iloprost.
• Prompt treatment is required to avoid gangrene and loss of digits.
• Drugs such as beta blockers and the oral contraceptive pill can
exacerbate Raynauds phenomenon by causing vascular spasm and
should therefore be avoided.
What is Dermatomyositis?
• Dysphagia, dysphonia
What are the investigations of
Dermatomyositis?
• Increased CK
• EMG
• Muscle biopsy
• Anti-jo-1 antibodies are not commonly seen in dermatomyositis - they are more
common in polymyositis where they are seen in a pattern of disease associated with lung
involvement, raynaud's and fever
• ANA positive in 60%
• Screen for malignancy by U/S abdomen + pelvis-(females)/+PSA(males) – CT chest might
be needed.
It is recommended that all
adult patients with
dermatomyositis should be screened for
underlying malignancy, T/F?
• True
What is the management of
Dermatomyositis?
• Prednisolone
How to differentiate between Polymyositis
and inclusion body myositis?
• Inclusion body myositis is the most common primary myopathy in elderly age group.
• It manifests as a slowly progressive weakness, usually affecting finger and wrist flexion initially.
• There is often fairly selective muscle involvement with wasting and weakness of the quadriceps
and the long finger flexors being the most common
• Lower limb weakness may also occur. Reflexes are usually diminished as in other myopathies.
• Creatine kinase levels are usually normal or only mildly raised, in contrast to polymyositis (where
creatine kinase levels are usually markedly elevated).
• Muscles are often tender in polymyositis, and the distal muscles are usually not affected until the
disease is advanced.
What is Sjogren's syndrome?
• Fibromyalgia is said to occur in between 1 and 2% of the general population and has
a female to male ratio of 9:1, presenting most often in the 30–50 year age group. It
presents with musculoskeletal pain that often has multiple trigger points and may be
poorly defined
• The presence of widespread pain for more than 3 months and (2) pain, not just
tenderness, that can be elicited by manual pressure of approximately 4 kg/cm2 at 11
or more defined tender points.
• Substantial overlap exists in major depressive disorder, various anxiety disorders,
and chronic fatigue syndrome and in multiple regional pain syndromes, such as
headache, atypical chest pain
What is the treatment of Fibromyalgia?