Rheumatology - Soft Tissue Flashcards by Claire Hannon

What is PMR?
Who gets it?
Features of PMR?

Post-inactivity stiffness and tenderness in proximal areas (pelvic and pectoral girdles)
Female, middle aged, FHx
Associated with temporal arteritis

Joint pain, stiffness, fever, malaise, weight loss, anorexia

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X Ray in PMR?

Coin Lesions in Lungs

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Complications

Glomerulonephritis

Coin Lesions in Lungs

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Investigation findings in PMR?

Bloods – all same, CRP, ESR, PV raised
CK normal
Muscle biopsy – normal

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How do you treat PMR?

Treat with Steroids

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What is Polymyositis?
Who gets it?
Features of Polymyositis?

Autoimmune Vasculitis which leads to ischaemia and necrosis of myofibrils and atrophy of fascicles.
In 40s-60s

Leads to symmetrical and diffuse muscle inflammation, pain and weakness of proximal muscles (rise from chair)

SOB (weak diaphragm)
Double vision (weak occular muscles)
Voice Change (vocal muscles)

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Investigation findings in Polymyositis?

ENA - anti-Jo1
ANA
CK and muscle biopsy
EMG Nerve conduction 
MRI

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How do you treat Polymyositis?

Steroids
Bisphosphnates
Immunosuppression

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What is Dermatomyositis?

Features?

Polymyositis + Skin Involvement]

Heliotrope rash

Gottrons papules

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Treatment of Dermatomyositis?

+ Hydroxychloroquine and sublock

Monitor (increase malignancy risk)

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What is SLE?

Who gets it?

Autoimmune inflammatory condition affecting multiple organs and skin involvement
More common in young, females
Thought to be induced by viral/UV

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Features of SLE?

ARA Criteria (4/11)
Serositis (pleuritis, pericarditis)
Oral Ulcers (painless)
Arthritis
Photosensitive

Blood (all low)
Renal (Proteinuria)
ANA
Immunologic (DS DNA)
Neuro

Malar
Discoid Rash

Raynauds
Hair Loss
Systemic Features

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Investigation findings in SLE?

ANA
DS DNA
ENA
All low bloods - FBC
Urine Dipstick
U and Es

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How do you treat SLE?

NSAIDS (careful of renal function)
Avoid UV
Skin/Joints - hydroxychloroquine
Other - steroids

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Complications of SLE?

Renal Failure
Avascular Necrosis
Infections

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What is Anti-phospholipid syndrome?

Autoimmune condition where there are Autoantibodies which interact with phosphopilids in cell membranes
Causes thrombus formation

Young, females, SLE

Features of aPL Syndrome?

Clotting Problems
Livedo Reticularis
Obstetric Complications
Thrombocytopenia

What is Raynauds?

Parasympathetic stimulation leading to an imbalance between vasodilators and constrictors, leading to vasospasm of vessels.
Affects young females (90%)
Can be 2ndary to SLE or RA

Features of Raynauds?

Painful ischaemia to the fingers/extremities (can also affect ears, nose, tongue and toes)

Linked to cold, stress

There is a colour change:
White – as there is ischaemia
Blue – as vessels dilate, pain
Red – reactive hyperaemia

Treating Raynauds?

Treat: cold avoidance, stop smoking
Can use vasodilators if 2ndary or severe - diltazem
Sympathectomy

What is Sjogrens syndrome?

Autoimmune infiltration of exocrine glands by lymphocytes.

Females
40-50

Features of Sjogrens?

Causes arthralgia, dry mouth and dry eyes

Other: dry  mucous membranes (genitals)
Causes: painful dry eyes
Dental Caries, dysphagia 
Enlarged parotids
Cough

Investigating Sjogrens?

ENA – Ro and La
ANA
CRP
ESR
Schirmers test

Treating Sjogrens?

Tear replacement
Sugar free lozenges, fluids, gels can be applied
Hydroxychloroquine

What is Systemic Sclerosis? | Who gets it?

Young people, tight hands You get thickening of the skin, wrinkle free face etc Due to inflammation, Ig and activated T cells infiltrate and cause sclerosis by fibroblasts of the limbs and organs

Features of Systemic Sclerosis?

Can be diffuse or limited cutaneous ``` Limited aka CREST syndrome Calcinosis Raynauds Esophageal Dysmotility Scleraderma Telectangasia ``` Diffuse – also affects lungs (fibrosis, HTN), heart (pansystolic murmer), kidneys, muscles Can cause organ failure within years

Investigation findings in Systemic Sclerosis?

``` ENA – anti-Scl 70 ANA, CRP, CRP Skin pinch test Microstomia Wrinkle Free Lung Function Barium Swallow Echo ```

How do you treat Systemic Sclerosis?

Symptom management - Raynauds, Reflux Immunosuppression - Lung Fibrosis Endothelin 1 antagonists Phosphodiesterase inhibitors

Examples of Small Vessel Vasculitis?

Behcets Wegeners Churg Strauss Goodpastures

Examples of Large Vessel Vasculitis?

Temporal Arteritis

Examples of Medium Vessel Vasculitis?

Kawasaki

What is Behcets?

Small vessel vasculitis More common in Mediterranean populations Middle aged Erythema Nodosum Mouth/Genital Ulcers

Who is affected by Wenegers?

Middle aged | Men

Symptoms of Wegeners?

Affects ELK ENT - epistaxis, nasal crusting, ulcers, sinusitis, saddle nose, stridor Lungs Kidneys

Treating Vasculitis?

High Dose steroids/ DMARDs for remission Maintain on Methotrexate, steroids (low dose) or AZA

Investigating Vasculitis

``` p/cANCA, CRP, ESR, U&Es, LFTs PV raised Biopsy of vessels/kidneys/organs for granulomas Urine Dipstick X Ray Chest CT Sinuses ```