Rheumatology - Soft Tissue

Question 1

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

Answer 1

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

Question 2

X Ray in PMR?

Answer 2

Coin Lesions in Lungs

Question 3

Complications

Answer 3

Glomerulonephritis

Coin Lesions in Lungs

Question 4

Investigation findings in PMR?

Answer 4

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

Question 5

How do you treat PMR?

Answer 5

Treat with Steroids

Question 6

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

Answer 6

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)

Question 7

Investigation findings in Polymyositis?

Answer 7

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

Question 8

How do you treat Polymyositis?

Answer 8

Steroids
Bisphosphnates
Immunosuppression

Question 9

What is Dermatomyositis?

Features?

Answer 9

Polymyositis + Skin Involvement]

Heliotrope rash

Gottrons papules

Question 10

Treatment of Dermatomyositis?

Answer 10

+ Hydroxychloroquine and sublock

Monitor (increase malignancy risk)

Question 11

What is SLE?

Who gets it?

Answer 11

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

Question 12

Features of SLE?

Answer 12

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

Question 13

Investigation findings in SLE?

Answer 13

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

Question 14

How do you treat SLE?

Answer 14

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

Question 15

Complications of SLE?

Answer 15

Renal Failure
Avascular Necrosis
Infections

Question 16

What is Anti-phospholipid syndrome?

Answer 16

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

Young, females, SLE

Question 17

Features of aPL Syndrome?

Answer 17

Clotting Problems
Livedo Reticularis
Obstetric Complications
Thrombocytopenia

Question 18

What is Raynauds?

Answer 18

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

Question 19

Features of Raynauds?

Answer 19

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

Question 20

Treating Raynauds?

Answer 20

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

Question 21

What is Sjogrens syndrome?

Answer 21

Autoimmune infiltration of exocrine glands by lymphocytes.

Females
40-50

Question 22

Features of Sjogrens?

Answer 22

Causes arthralgia, dry mouth and dry eyes

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

Question 23

Investigating Sjogrens?

Answer 23

ENA – Ro and La
ANA
CRP
ESR
Schirmers test

Question 24

Treating Sjogrens?

Answer 24

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

Question 25

What is Systemic Sclerosis?

Who gets it?

Answer 25

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

Question 26

Features of Systemic Sclerosis?

Answer 26

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

Question 27

Investigation findings in Systemic Sclerosis?

Answer 27

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

Question 28

How do you treat Systemic Sclerosis?

Answer 28

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

Question 29

Examples of Small Vessel Vasculitis?

Answer 29

Behcets
Wegeners
Churg Strauss
Goodpastures

Question 30

Examples of Large Vessel Vasculitis?

Answer 30

Temporal Arteritis

Question 31

Examples of Medium Vessel Vasculitis?

Answer 31

Kawasaki

Question 32

What is Behcets?

Answer 32

Small vessel vasculitis
More common in Mediterranean populations

Middle aged
Erythema Nodosum
Mouth/Genital Ulcers

Question 33

Who is affected by Wenegers?

Answer 33

Middle aged

Men

Question 34

Symptoms of Wegeners?

Answer 34

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

Question 35

Treating Vasculitis?

Answer 35

High Dose steroids/ DMARDs for remission

Maintain on Methotrexate, steroids (low dose) or AZA

Question 36

Investigating Vasculitis

Answer 36

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