Rheumatology Flashcards
Churg-Strauss Syndrome (eosinophilic granulomatosis with polyangiitis) is associated with which antibodies?
MPO/P-ANCA
How does Churg-Strauss tend to present?
Nasal congestion, adult-onset asthma, peripheral neuropathy, and glomerulonephritis.
Which antibodies are associated with granulomatosis with polyangiitis (Wegner’s granulomatosis)?
cANCA
How does granulomatosis with polyangiitis present?
URT involvement (recurrent sinusitis, epistaxis, saddle nose deformity), LRT involvement (cough, haemoptysis, pleuritis), pauci-immune glomerulonephritis (haematuria, proteinuria).
Often presents with a decline in renal function alongside recurrent epistaxis.
How is granulomatosis with polyangiitis managed?
Steroids, biologics (rituximab)
What is the HLA association for Bechet’s Disease?
HLA-B51
What are the manifestations of Behcet’s disease?
Recurrent oral and genital ulcers, erythema nodosum, uveitis, acute pericarditis, acneiform lesions, superficial thrombophlebitis at venepuncture sites, arthritis, etc.
How do you investigate for and manage Behcet’s disease?
Inx: Pathergy test (intradermal skin prick: development of papule or pustule is a +ve result as it represents exaggerated inflammatory response)
Mx: steroids around flares, may require systemic therapy long term (methotrexate, biologics)
What is Polyarteritis nodosa?
Segmental transmural necrotising, small-medium muscular vessel vasculitis.
Systemic sx: skin (nodular and ulcerating lesions), heart, kidneys, GIT, and nervous system (mononeuritis multiplex).
Which hepatitis is polyarteritis associated with?
Hep B
Which vessels are NOT affected in polyarteritis nodosa?
Pulmonary vessels
Who does polyarteritis nodosa primarily affect?
Men 40-60 y/o
How is polyarteritis nodosa managed?
Mild: oral corticosteroids
Moderate: IV corticosteroids
Severe: Immunosuppressive therapy (e.g., cyclophosphamide)
What are the manifestations of Giant Cell Arteritis?
Headache, unilateral vision loss (curtain closing), scalp tenderness, jaw claudication, etc.
Patients with GCA are 17 times more likely to experience what aortic complication?
Aortic dissection.
Which other rheumatological condition is associated with GCA?
PMR (about 50% GCA pts have a background of PMR)
What type of optic neuropathy can develop in GCA? How does the optic disc look on examination?
Anterior ischaemic optic neuropathy: infarct of the short ciliary arteries which supply the head of the optic nerve.
Swollen, white, chalky optic disc on examination.
What is an enteropathic arthropathy? What are the skin manifestations?
Joint involvement before, during, or after IBD diagnosis.
Associated with skin manifestations (pyoderma gangrenosum, erythema nodosum).
What are the most common manifestations of psoriatic arthritis?
- Family/personal hx of psoriatic rash
- Occult rash (scalp, ears, umbilicus, natal cleft)
- Nail changes (pitting, oncolysis, ridges),
- Enthesopathy (Achilles tendonitis)
- Dactylitis
- Pencil-in-cup deformity on XR
- Asymmetrical oligoarthritis (large joint swelling)
What makes psoriatic arthritis seronegative?
No associated autoantibodies
What is the triad of reactive arthritis?
Conjunctivitis, urethritis, arthritis.
What most commonly triggers reactive arthritis?
Genitourinary or gastrointestinal illness, most commonly chlamydia trachomatis.
What is Caplan’s Syndrome?
Intrapulmonary nodules alongside rheumatoid arthritis.
‘Rheumatoid Pneumoconiosis’ associated with exposure to dust in industry (silica, asbestos, etc).
What are the features of polymyalgia Rheumetica?
Shoulder hip and girdle pain/stiffness, often in the morning for <1 hour.
Systemic features: weight loss, loss of appetite, malaise, low grade fever.
Who does PMR most commonly affect?
Women >50
How can you investigate for PMR?
Generic bloods, tend to find that ESR is markedly raised >60.
How is PMR managed?
Corticosteroids: sx relived within 72 hours.
What markers can be measured when diagnosing rheumatoid arthritis?
Rheumatoid factor and anti-CCP
What are the most commonly involved joints in rheumatoid arthritis?
PIPs and MCPs
What are the signs of rheumatoid arthritis in the hands on XR?
- Periarticular osteopenia
- Joint erosions
- Periarticular soft tissue swelling
- Joint space narrowing
What score can be used to measure disease severity of rheumatoid arthritis? At what point might you start a DMARD?
DAS28,
>5.0 = active disease - commence therapies.
Below what DAS28 score indicates well controlled rheumatoid arthritis disease?
<3.2
What medications are safer for patients with RA to be on if they wish to start family planning?
Sulfasalazine or hydroxychloroquine AND FOLIC ACID.
NOT methotrexate!!
What is Felty’s syndrome?
Longstanding (tends to be erosive) RA, splenomegaly, neutropenia.
What is meant by the term ‘Seronegative Spondyloarthropathy’?
Axial and peripheral joint pain, enthesitis, tend to be HLA-B27 +ve but are -ve for rheumatoid factor.
What signs do you see on XR with ankylosing spondylitis?
Lumbar spinal fusion (especially SI joints) know as bamboo spine, and syndesmophytes (bony outgrowths from spinal ligaments as they attach to adjacent vertebral bodies).
What type of respiratory failure can occur in AS?
T2 RF due to poor mechanical ventilation = hypoxia and hypercapnia.
Which HLA type is AS associated with?
HLA-B27
What are some extra-articular features of AS?
Anterior uveitis, aortic insufficiency, apical pulmonary fibrosis
How is AS managed?
NSAIDs, DMARDs, steroid injections, and if these are unsuccessful then try biologics (anti-TNF).
What are the manifestations of inflammatory arthritis?
Morning stiffness, loss of function, fatigue, generally unwell with flu-like sx, and swelling, erythema, and warmth around joints.
What is deposited in joints in pseudogout, how is this seen on microscopy?
Calcium pyrophosphate dihydrate crystals = positively birefringent rhomboids.
What might you see on XR of pseudogout?
Calcification of articular cartilage known as chondrocalcinosis.
True or False: Pts with hyperparathyroidism and haemochromatosis are more at risk of Pseudogout?
TRUE
What type of crystal is deposited in gout? How do these appear on microscopy?
Monosodium urate monohydrate crystals: negatively birefringent needles.
What is gout in the big toe known as?
Podagra
What are the signs/sx of Gout?
Sudden, Intense pain, hot joint, limited range of movement due to pain.
What are the RFs for gout?
- Male>55
- increased alcohol intake
- high protein diet
- episodes of dehydration
What is the acute and long term management of gout?
Acute: colchicine, NSAIDs
Long Term: allopurinol or febuxostat to reduce urate levels
How does Adult Onset Still’s Disease present?
Inflammatory disorder, presents with pyrexia, arthralgia, fine, non-purpuric salmon pink rash.
What markers are most specific in SLE?
- Anti-dsDNA
- Anti-smith
What happens to C3 and 4 in SLE?
Low
Which antibodies are found in drug-induced lupus? How does it present?
- Anti-histone abs.
- Rash and increased sensitivity to sunlight
What are the signs/sx of SLE?
- Butterfly malar rash
- Joint pain (Jaccoud arthropathy, where deformities are reducible in extension)
- Fatigues
- Lupus nephritis (declining kidney function)
- Cardiorespiratory issues
How do you manage hypertension in SLE?
ACEIs or ARBs to reduce cardiovascular risk and help to decrease proteinuria.
How do you manage the rash associated with Lupus if they have no other constitutional sx?
Hydroxychloroquine
How do you manage the rash associated with Lupus alongside other constitutional sx?
Hydroxychloroquine + prednisolone
How can we manage lupus nephritis?
Steroids and cytotoxic drugs such as cyclophosphamide.
Which hypersensitivity reaction is Sjogren’s? What is the pathophysiology?
Type IV - lymphocytic infiltration of glands causing decreased lacrimal and salivary secretion.
What are the manifestations of Sjogren’s?
- Dry eyes, mouth
- Joint pain
- Vaginal dryness = dyspareunia
Which antibodies develop in Sjogren’s?
Anti Ro and anti La
How is Sjogren’s managed?
Hypromellose: artificial tears, artificial saliva,
Sjogren’s increases the risk of which lymphoma? How does it present?
MALT low grade B-cell NHL: bilateral parotid swelling and cervical lymphadenopathy.
Cutaneous Sclerosis (Scleroderma) can be split into which two types?
Limited: CREST syndrome (calcinosis, Raynaud’s, oesophageal dysmotility, sclerodactyly, telangiectasia), anti-centromere antibodies
Diffuse: anti-scl 70 antibodies, spreads further and involves mulitple organ systems
What are the pulmonary complications of the two types of cutaneous sclerosis?
Limited: pulmonary HeTN
Diffuse: pulmonary fibrosis
What is Polymyositis? What antibodies are associated with it?
Inflammation and degeneration of skeletal muscle (inflammatory myopathy).
Anti-Jo-1 antibodies.
What is the main presentation of Polymyositis?
Bilaterally painless proximal muscle weakness due to chronic muscle inflammation.
This causes problems such as standing up from sitting.
Do you get muscle wasting in Polymyositis?
Much later on in the disease
What type of pts most commonly present with Polymyositis?
40-60 y/o women.
How can you investigate for Polymyositis?
- CK (will be elevated)
- EMG (to confirm myopathic disorder)
- Antibody testing
What is Dermatomyositis?
Polymyositis with added manifestations on the skin e.g., heliotrope rash and shawl sign.
Is Dermatomyositis associated with antibodies?
Yes, anti-Jo-1 antibodies like polymyositis BUT much less so than polymyositis.
Anti-Mi2 antibodies are more specific.
Which is more associated with malignancies of the lung: Polymyositis or Dermatomyositis?
Dermatomyositis
What is complex regional pain syndrome?
Severe pain, swelling, and increased sensitivity to touch which tends to be triggered by a minor injury.
May also cause skin colour changes.
What is 1st line for medical management of Raynaud’s?
Dihydropyridine CCB (nifedipine)
What is Pes Planus?
Flat footed, congenital or acquired loss of longitudinal foot arch.
What is acute Sarcoidosis called? How does it present? Mx?
Lofgren syndrome: fever, polyarthralgia, erythema nodosum, bilateral hilar lymphadenopathy.
Mx: NSAIDs and rest.
What are the systems affected my chronic sarcoidosis? Mx?
Pulmonary, neurological, ocular, cardiac, abdominal, and dermatological.
Mx: steroids oral or IV.
Which electrolyte abnormality is associated with Sarcoidosis?
Hypercalcaemia: granulomas contain macrophages which release excess 1,25-hydroxyvitamin D3 (active vitamin D aka calcitriol).
Which serum markers can be raised in dermatomyositis and polymyositis?
They turn your muscles into CLAAA:
- creatinine kinase
- lactate dehydrogenase
- aldolase
- AST
- ALT
Which serotype is associated with both Rheumatoid arthritis and Felty’s Syndrome?
HLA-DR4
What WCC abnormality is associated with SLE?
Lymphopenia
