Multisystem Flashcards by Emmy Beever

What are the features of fibromyalgia (5 common / 3 variable / 6 associated conditions)

Common:
Widespread pain
Marked fatigue
Low affect/mood
Poor concentration
Non-restorative sleep

Variable locomotor sx:
Early morning stiffness
Finger swelling
Numbness/tingling fingers

Assoc conditions:
IBS
Allodynia (dyspareunia/hyperacusis / discomfort on touch)
Irritable bladder
Tension headache
Chemical sensitivity SEs to drugs
Depression

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What hand signs would be seen O/E in RA?

LOOK:
Wrist radial deviation
MCPJ ulnar deviation (at knuckles)
Swan neck (PIP ext / DIP flex) 
Boutonnieres (PIP flex / DIP ext)
Z thumb
Volar subluxation (late)

Palmar erythema
Small mm wasting

MOVE:
Reduced RoM
TEST:
Carpal tunnel signs

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What are some complications of severe RA disease in hand?

Fusion (ankylosis)
Rupture of tendons (4th/5th from swelling/sublux)
Carpal tunnel

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What may be seen o/XR in RA (4)

Loss joint space
Osteopenia (periarticular)
Swelling (soft tissues)
Erosions (marginal)

±Sublux/Dislocation

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What signs may be seen in the feet O/E in RA?

Hallux valgus
MTP lateral (fibular) deviation EXCEPT 5th
Hammer toe deformity

Callouses / ulcers

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What blood tests for RA / results expected (5)

FBC: WCC↓/Platelets↓ (acute) // Anaemic (chronic)
CRP-ESR:↑

RF: 70%
ANA: 30%
Anti-CCP: Most specific

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Outline the extra-articular manifestations in RA (8)

Eyes: kerato sicca / epi / scleritis
Atlanto-Axial Subluxation (50-80%)
Cardiac: IHD risk / Pericardial effusions
Pulm: Fibrosis / Exu effusions / Rh nodules
Abdo: Mesenteric ischaemia / Splenomeg (Felty’s)
Renal: amyloidosis
Skin: Rh nodules / Nail fold infarcts
Nervous: Entrapments / Sensory loss (nn vasculitis)
Haem: Felty’s / ACD

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What is Felty’s syndrome?

Anaemia
Leucopenia
Splenomegaly

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Outline the management of RA

MDT (ADL help / home adaptations)
Lifestyle: stop smoking (CV risk/↓DMARD effect)

DMARDs asap from Dx (MTX ± hydroxyC)
Short-term glucocorticoids (IM methylpred) for flares
NSAIDs (pain/stiffness)

Biologics (after 2+ DMARDs / DAS score)

Surgical:
Synovectomy / Arthroplasty (inc. excisional smalls)

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What is the DAS score based on in RA? (4)

No. swollen stires
No. tender sites
CRP/ESR
Pt perception of severity

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What are the extra-articular manifestations seen in Ank Spon (5)

Eyes: anterior uveitis
Pulm: Apical fibrosis
Cardiac: AVN block / Aortitis w. regurg
Renal: amyloidosis

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What are the main joints involved in Ank Spon

Sacroiliac**
Hip
Shoulder
Costochondrals

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What would be seen O/E in Ank Spon?

LOOK:
Question Mark posture (early)
Paraspinal mm wasting (late)

FEEL: SIJ tenderness
MOVE: ↓Lumbar RoM (lateral/forward)
TEST: Shober’s (↓length increase on flexion)

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What will be seen o/XR in Ank Spon?

Pelvic XR: 
Bilateral sacroilitis (narrowed joint space / fusion)

Spinal XR:
Squared vertebral bodies
Ossified discs
Ossified interspinous ligaments

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What is the 1st/2nd line management for Ank Spon?

1st line – full dose NSAIDs 6wks (try 2+ diffs)

2nd line – biologics e.g. eternacept (NB not inflix)

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List the 5 main subtypes of Psoriatic Arthritis

Symm Poly (40%)
Asymm Oligo (30%)

DIPJ predom (10%)
Mutilans (5%)
Spondylitis

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Outline the management of psoriatic arthritis

1 joint: Full-dose NSAID ± steroid

Multiple: treat same as RA (DMARDs) unless Spondylitis

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What are the radiological differences b/wn:
Psoriatic + RA
Psoriatic (spond) + AS

Vs RA:
Early DIPJ involvement
Minimal osteopenia
Central erosions (pencil in cup)

Vs AS: Unilateral SIJ

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What infections can reactive arthritis be caused by? (3)

Chlamydia
Campylobacter
Salmonella

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What is the classic triad of Reiter’s syndrome?

What other features may be seen (4)

‘Can’t pee, see or climb a tree’:
• Dysuria
• Conjunctivitis
• Lower limb oligoarthritis

Others:
• Spondylitis
• Enthesitis (Achilles/Plantar)
• Skin (balanitis / keratoderma blenorrhagica – plaques)

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How is Reactive Arthritis investigated and treated?

Joint Aspirate (exclude septic) – sterile

1st: full-dose NSAIDs ± steroid injections
2nd: Sulfasalazine (severe/extensive)

What skin involvement is seen in SLE (4)

Chronic discoid (plaques)
Malar (nasolabial fold sparing)
Photosensitivity
Alopecia

What other specific systems are involved in SLE + to what proportions (8)

MSK: arthritis (90%) / myalgia (50%)
Skin (75%)
CNS (60%): dep/anx** / seizures / vascular w. APL
Lung (50%): exu effusions / fibrosis
Renal (30%): interstitial nephritis
Cardiac (25%): pericarditis / higher artherosclerotic risk
Haem: ACD / Leucopenia / Thrombocytopenia / autoimmune haemolytic (rare)
Oral mucosal lesions (20%)

What autoimmune disease are assoc w. 2º Sjorgen’s (4)

RA
SLE
Scleroderma
Polymyositis

List features of Sjorgen's (3 specifics + 4 systemics)

Dry eyes (keratoconjunctivitis sicca) / Dry mouth Parotid enlargement Vaginal dryness Arthralgia Oesophageal motility probs Reynaud's NHL (1 in 6)

What monitoring bloods should be ordered in SLE (4)

FBC U+Es Urine dip C3/4 complement (reduced in active)

Outline the management of SLE

Avoid CV risks Avoid excess sunlight Meds – mild (skin/arthritis/fatigue): NSAIDs + hydroxyC Meds – severe (cardiac/renal): Pred + DMARDs

What are the features of APL syndrome (3+3)

Venous**/Arterial thrombosis Recurrent miscarriages Livedo reticularis (lace-like mottled rash) ± Thrombocytopenia ± Migraines / Epilepsy

How is APL Dx? | What is seen on coag studies ?

``` Anticardiolipin Abs (2 results; 12wks apart) APTT raised ```

How is APL managed: If no h/o thrombosis If h/o thrombosis If pregnant

No H/o thrombosis: low-dose aspirin + lifestyle H/o thrombosis: Warfarin INR 3–4 Pregnant: Low-dose aspirin + LMWH + early delivery

What are the main features of Systemic Sclerosis (scleroderma) (2)

Reynauds (long hx in limited cutaneous vs short in diffuse) | Skin tightening at extremities ± facial

Another name for limited cutaneous scleroderma? | + the features

CREST syndrome ``` Calcinosis Reynaud's Esophageal dysmotility Sclerodactyly Telangiectasia (skin/mucous membs e.g. →Pulm HTN ```

What are the main complications of diffuse cutaneous scleroderma?

Myocardial fibrosis Pulm fibrosis Renal fibrosis

What are some presentation features of Inclusion Body Myositis? What specific tests + their results

White males >50 Insidious Proximal/Distal mm weakness Myositis-specific Ags: –ve Mm biopsy: inflamm B-amyloid vacuole (inclusion body)

List the Dx criteria for Polymyalgia Rheumatica (4; PMR)

Pelvic girdle +/ bilateral shoulder ache Morning stiffness Response – acute phase (evidence of) >2wks

What is the main complication of GCA | How is it monitored

Aortic / large vessel involvement (aneurysm / dissection) Monitor with serials CXRs

How is PMR + GCA treated? | Monitoring + how this affects treatment

PMR: 15mg PREDNISOLONE o.d. GCA: No visual – 40mg od Visual sx – 60mg od SPLV – 80mg od Plus PPI + Bone protection Taper down steroids with CRP-ESR PMR ~2yrs GCA ~5yrs

``` What vasculitis disease involve in: Large vessels Medium vessels Medium/small vessels Small vessels ```

Large: GCA / Takayasu's Mediums: Kawasaki / Polyarteritis Nodosa Medium/smalls: Wegener's / Churg-Strauss / Microscopic Polyangiitis Smalls: HSP / Cryoglobulinaemia

What blood test can be done for medium/small vessel vasculitis diseases

ANCA (+ve) p-ANCA: Churg-Strauss / Polyangiitis c-ANCA: Wegener's (think more Complicated system involvement)

Outline the management options for vasculitis diseases (4)

Corticosteroids (w. prolonged reduction) Cyclophosphamide (ANCA+ve) IVIG (Kawasaki) Plasmaphoresis (life-threatening)

What is seen on FBC in most rheumatic diseases? | + list causes of these results

``` Anaemia: ACD Fe defc – assoc ulcers Megaloblastic – Pernicious/MTX) Haemolysis – SLE/Drugs ``` Lymphopenia: SLE Leucocytosis: acute SLE flare Thrombocytopenia: SLE/APL Felty's MTX

What REGULAR (non-rheum) blood tests are done for rheumatic diseases (3)

FBC U+Es (GN – but urine dip better) LFTs: raised ALP in most

List some causes of +ve/raised Rheumatoid Factor (4)

RA* (+ve in 70%) Sjorgen's* SLE (30%) Scleroderma (30%)

List some causes of +ve/raised ANA (5+3)

``` SLE** (100%/ –ve excludes) RA Sjorgen's Polymyositis Scleroderma ``` Thyroid (graves/hashimotos) GI (AIH/PBC/IBD) Pulmonary (idiopathic fibrosis)

When would anti-dsDNA be +ve?

SLE (almost 100% specific) – used for monitoring as s/o severe disease with e.g. renal involvement RA +ve/–ve Viruses (transiently): HIV/Parvovirus

What malignancies are poly/dermatomyositis associated with? (5 Bs)

``` Baby making (ovarian) Boobs (breast) Balls (prostate) Bum (colon) Bronchial ```

How do Polymyositis / Dermatomyositis present?

``` Poly: • Insidious or acute • Prox mm weakness / wasting (e.g. stairs) • Systemic: malaise/wt loss/fever • Untreated –> resp mm failure ``` ``` Dermato: same but skin • Heliotropic purple rash • Periorbital oedema • Gottron's papules • Shawl rash ```

What are the main differentiating bloods in inflammatory myopathies?

Myositis-specific Ag: • Poly/Dermato +ve • Inclusion body –ve Serum CK raised (both; v. high = unlikely malignancy)

What Ix can be done into inflammatory myopathies 1. in primary care setting? (3) 2. secondary care (3)

GP: Tumour markers / CXR / Abdo USS Hosp: EMG / MRI / mm biopsy

Outline the management of poly/dermatomyositis

``` Prednisolone (60-80mg) / DMARDs (start whilst awaiting results) • little response = cancer • good response = not • Titrate steroids down w. CK ``` If condition deteriorates: • Temporarily increase steroids • Whilst starting on azathio

How may a typical case of Inclusion Body Myositis present?

White male <50 Insidious prox + distal mm wasting Progressive / unresponsive to Pred + DMARDs

What are the main cautions/CIs in Biologics Tx? (5)

``` Malignancy (SCC risk) Active infection Latent TB Pulmonary fibrosis Severe HF ```