Wegners (granulomoatosis polyangitis) CF

Rheum Cram Flashcards by Olivia Wells

Best test to screen for SLE?

ANA (almost 100% positive)

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Most sensitive test for SLE?

Anti-Smith (ENA)

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Most specific test for lupus nephritis?

Anti-dsDNA

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ENA associated w neonatal congenital heart block?

Anti-Ro

Begins crossing placenta at 16weeks

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cANCA assoc w

Wegners (granulomoatosis polyangitis)

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pANCA assoc w

Microscopic polyangitis
Polyarteritis nodosa
PSC/IBD
HSP
KD

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RF assoc with

Rheum arthritis
Sjogrens
SLE

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Histone assoc w

Drug induced lupus/SLE

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HLA-B27 assoc w

Enthesitis related arthritis
Ankylosing spondylitis 90% pos
Uveitis

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MTX mechanism and SEs

Blocks purine synthesis
Bone marrow suppression (not at rheum doses)
Teratogenic
Accumulates in 3rd spaces
AVOID bactrim/penicillins

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Leflunomide mechanism and SEs?

Blocks pyrimidine synthesis
Liver dysfunction
Parasthesia
Teratogenic

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Hydroxychloroquine mechanism and SEs?

Anti-inflammatory and immunosuppressive effects
Ototoxic - colour vision
Ophthal review 6-12mthly

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Mycophenalate mechanism and SEs?

Suppressive lymphoproliferation and antibody formation
Bone marrow suppression
Increased risk of neoplasia

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Infliximab mechanism and SEs?

Anti-TNFalpa
Cytopenia
Demyelination
Lupus like syndrome

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Rituximab mechanism and SEs?

AntiCD20 - depletes B lymphocytes
MSK pain/weakness
Infection
Progressive multifocal leukoencephalopathy

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Anakinra mechanism and SEs?

IL-1 receptor antagonist
Injection site reaction
Headache
neutropenia

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Tocilizumab mechanism and SEs?

IL-6 receptor antagonist
Deranged LFTs
Injection site reaction
GI perforations

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>2/52 hx of:
arthritis
daily fever 
lymphadenopathy
evanescent erythematous rash
serositis

Systemic JIA

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Most common form of JIA?

Oligoarthritis

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DIP arthritis and psoriasis with nail/finger changes and Fhx of psoriasis?

Psoriatic arthritis

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HLA-B27 positive and hip/low back pain

Enthesitis related arthritis

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First line mx for JIA?

NSAIDs
Steroids
2nd line DMARDs - MTX, antiTNF, anakinra

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When not to use etanercept?

Uveitis

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Complications of JIA?

Uveitis
Limb length discrepancy/growth retardation
Interstitial lung disease/Pulm HTN
TMJ (50%)
Decreased BMD from steroids

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JIA type most assoc w chronic uveitis?

Oligoarticular | Particularly ANA pos, RF neg

JIA type most assoc w acute uveitis?

Enthesitis JIA | Particularly if HLA-B27 pos

Unremitting fever, rash, arthritis Splenomegaly Cytopenia, High trigs, low ESR, very high ferritin Elevated soluble CD24

Macrophage Activation Syndrome | AKA HLH assoc w rheumatic disease

Mx of choice for MAS?

High dose steroids | Anakinra

Teenage girl, not-caucasian. Prolonged hx. Photosensitive rash, nasal ulcers, hard palate macules Fever, arthritis/arthralgia, hepatosplenomegaly, nephritis

SLE

Marker of disease activity in SLE? Role of C3/C4 in SLE?

Anti-dsDNA marker of disease activity | low C3 marker of disease activity but low C4 indicative of genetic pre-disposition for SLE

Mx of choice for SLE?

All pts should be on hydroxychloroquine - flare reduction, improves lipid profiles also Steroids for acute flares (topical to skin) Aspirin/warfarin if procoagulant

Most common inflammatory myopathy of childhood?

Dermatomyositis

Weakness, lipodystrophy/calcinosis, plaques over fingers (gottron papule) and heliotrope rash?

Dermatomyositis

Raynaud followed by skin changes distally, nail fold capillary change. ANA positive nucleolar staining?

Scleroderma

Anti-SCL-70 and anti-RNA polymerase positive?

Diffuse systemic sclerosis

Systems involvement in scleroderma?

GI - oesophagus (dysphagia, aspiration), rectal prolapse, constipation/fecal incontinence Lungs - interstitial fibrosis, pulmonary HTN Heart - dilated cardiomyopathy Renal - HTN

Centromere ANA staining pattern?

CREST variant scleroderma

Mx of scleroderma?

UV therapy/topical steroids Corticosteroids/MTX Cyclophos & MMF for resp involvement

``` Petechiae/purpura mostly of lower limbs Diffuse abdo pain (+/- diarrhoea) Arthritis/arthralgia Proliferative GN IgA deposition ```

Henoch Schonlein Purpura

Rate of HSP recurrence? Risk of long term ERSD post HSP?

30% recurrence rate usually in 6mths | Renal disease in 1-2%, 10% of these will have ESRD

Fever, fatigue, LOW Abdo pain Haematuria/proteinuria Purpura and livedo reticularis with painful nodules

Polyarteritis nodosa

Biopsy findings of polyarteritis nodes?

Necrotising vasculitis | Granulocytes and monocytes in small/medium sized arteries

Pulm haemorrhage/nasal ulceration/hearing loss Haematuria/proteinuria Palpable purpura cANCA positive

Gramulomatosis Polyangitis AKA Wegners

Pulm haemorrhage/hearing loss Haematuria/proteinuria Palpable purpura pANCA positive

Microscopic polyangitis

Refractory asthma Proteinuria/haematuria Eosinophilic infiltration

Churg Strauss

Genital and oral ulcers, scars from old ulcers Uveitis Erythema nodosum

Behcet's

Neonatal lupus syndrome most associated with?

Maternal Sjogrens

DIagnostic criteria for fibromyalgia?

Diffuse MSK pain in at least 3 areas of the body, persisting for 3 months in the absence of an underlying condition Must have at least 5 well defined tender points of examination

Periodic fevers lasting 1-3 days with arthritis, serositis and erysepiloid rash. Assoc w Behcets, HSP, polyarteritis nodosa

Familial Mediterranean fever | MEFV gene

Risk of developing amyloidosis in familial Mediterranean fever?

30-50% if untreated | Treat with colchicine

Baby with fever lasting up to 1 week rash and abdo pain and lymphadenitis, triggered by immunisation/surgery.

Hyper IgD Syndrome | MVK gene

Fever with painful rash, conjunctivitis, episodes last weeks at a time and occur multiple times/year.

TRAPS TNF receptor associated periodic syndrome Receptor 1A gene mutation TNFRSF1A

Risk of amyloidosis in TRAPS?

25% - kidneys and liver | Colchicine does not treat TRAPS - NSAID/pred

Infant in first week of life with fevers, meningitis, rash, arthropathy of knees.

NOMID | Mx anakinra

Febrile attacked 1-3hrs post cold exposure, resolve within 14hrs?

Familial cold auto inflammatory syndrome

Intermittent fevers with SNHL?

Muckle-Wells

Fever, adeninitis, pharyngitis, apthous stomatitis lasting 4-5 days every month.

PFAPA/Marshall Syndrome Periodic Fever, Apthous Stomatitis, Pharyngitis and Adenitis Mx - prev, colchicine and tonsillectomy

Biopsy showing eosinophilic material that stains with Congo red dye and shows apple green biofringence in polarised light?

Amyloidosis

Rash, uveitis and polyarthritis with large boggy effusions. Non-caeseating epithelia granulomas with hypercalcaemia and hypercalcuria

Sarcoidosis

Granulomas of eyes, skin and joints?

Blau syndrome