Connective Tissue Disorders

Question 1

Give examples of connective tissue disorders?

Answer 1

Rheumatoid arthritis
Systemic/ discoid lupus erythematous
Systemic sclerosis
Sjogrens syndrome

Question 2

What is rheuatmoid arthritis?

Answer 2

Auto-immune condition

Multi-system inflammatory disease of synovial and adjacent tissues

Question 3

Aetiology of rheumatoid?

Answer 3

Females > males

Peak 35-50 years

Question 4

What causes rheumatoid?

Answer 4

Auto-immune

Autoantibodies - rheumatoid factor

Question 5

Issue with looking for rheumatoid factor?

Answer 5

Not very specific

Question 6

What more accurate marker in rheumatoid?

Answer 6

Anti-CCP

Question 7

Clinical features of rheumatoid?

Answer 7

Slow onset
Pain and stiffness of small joints
Fatigue
Anaemia
Weight loss
Muscle loss and wasting
Lung problems 
Lymphadenopathy

Question 8

Most common joint affects in rheumatoid?

Answer 8

Finger joints

Question 9

Is TMJ affected in rehumatoid?

Answer 9

in 30% have involvement

Question 10

What hand signs might see in rheumatoid?

Answer 10

Ulnar deviation/ swan neck deformity

Question 11

Extra-articular manifestation R.A?

Answer 11

Weight loss
Fatigue
Lymphadenopathy 
Rheumatoid nodules
Sjogrens

Question 12

How diagnose rheumatoid?

Answer 12

Bloods - raised ESR/ CRP, anti-CCP positive, rheumatoid factor positive

Look radiographic changes

Question 13

What guideline for management of rheumatoid?

Answer 13

NICE guidelines

Question 14

What do NICE suggest management rheumatoid?

Answer 14

Education 
Exercise - maintain muscle
Physio
Surgery 
Dietary advice

Question 15

When is surgery indicatied for RA?

Answer 15

If progressive deformity

Question 16

What medications are used RA?

Answer 16

DMARDs
Corticosteroids
Biological agents
Symptomatic relief

Question 17

What is DMARD and give examples?

Answer 17

Disease modifiying anti-rheumatic drug

e.g methotrexate/ hydroxychlorquine

Question 18

Give example biological agent?

Answer 18

Anti-TNFa

e.g Infliximab

Question 19

What medications are given for symptomatic relief of RA?

Answer 19

NSAIDs

Question 20

What is the oro-facial impact of RA?

Answer 20

Access
Antalo-axial joint dislocation
Impaired manual dexterity - OH
Secondary Sjogrens

Question 21

What may see is TMJ affected RA?

Answer 21

AOB

Question 22

Felty’s syndrome can be seen with RA - what might see?

Answer 22

Increased risk oral infections

- oral ulceration/ angular chelitis

Question 23

What are two forms of lupus erythematous?

Answer 23

Systemic and discoid

Question 24

What is lupus erythematous?

Answer 24

Immunologically mediated condition

Question 25

Where does discoid lupus affect?

Answer 25

Skin and oral mucosa

Question 26

What condition does discoid lupus look similar to?

Answer 26

Lichen planus

Question 27

How does discoid lupus present on skin?

Answer 27

Scaly, erythaematous patch | Atrophic, hypo-pigmented area

Question 28

Issue w/ lesions of discoid lupus on skin?

Answer 28

Can be premalignant

Question 29

How is discoid lupus diagnosed?

Answer 29

Clinical appearance Biopsy Circulation auto-antibodies

Question 30

What auto-antibodies would be present in discoid lupus?

Answer 30

ANA | dsDNA

Question 31

Management of discoid LE?

Answer 31

Can't cure Improve symptoms - difflam/ topical steroids to reduce inflammation Similar tx lichen planus

Question 32

What systemic features see in systemic LE?

Answer 32

``` Malar rash - cheeks/ bridge nose Polyarthirits Photosensitivity Oral lesion Renal/ cardiac lesions ```

Question 33

How common is it to have oral lesions w/ systemic LE?

Answer 33

40%

Question 34

How do oral lesions present in systemic LE?

Answer 34

Uni/bilateral white patch w/ central area erythema/ ulceration

Question 35

Pharmacological management of systemic LE?

Answer 35

Hydroxychloroquine Corticosteroids Cytotoxic - azathioprine/ methoitrexate

Question 36

What drugs can cause lupus-like reactions?

Answer 36

``` Carbamazepine Hydralazine Penicillamine Procanimadie Methyldopa ```

Question 37

What is systemic sclerosis?

Answer 37

Autoimmune disorder casuing dense collagen deposits in tissues of body

Question 38

What are types of SS?

Answer 38

Localised | Diffuse

Question 39

What is localised SS?

Answer 39

Limited skin face, hands and feet

Question 40

What is diffuse SS?

Answer 40

Extensive skin involvement w/ progression to visceral organs

Question 41

What has better prognosis diffuse or localised SS?

Answer 41

Localised --10-year survival = 75% | Diffuse -- 10 year survival = 55%

Question 42

What syndrome is linked to localised SS?

Answer 42

CREST syndrome

Question 43

What See in CREST syndrome?

Answer 43

``` C - calcinosis (calcium deposits) R - raynauds E - oEsophageal dysfunction S - sclerodactyly T - telangiectasia ```

Question 44

What are orofacial manifestations SS?

Answer 44

``` Facial skin rigidity Thinning lips Loss facial wrinkets Hypo-mobile tongue Dysphagia Xerostomia PDL widening Microstomia ```

Question 45

What are issues caused by microstomia in SS?

Answer 45

Poor access OH - increased caries/ perio | Issues: speech, eating

Question 46

How dx SS?

Answer 46

Difficult - clinical dx Skin biopsy PDL widening

Question 47

Management of SS?

Answer 47

Look improve circulation - nifedipine | Suppress immunity - corticosteroids/ DMARDs

Question 48

How tx microstomia in SS?

Answer 48

Exercise - stretching | Surgical approach - commissuotomy

Question 49

What is Sjogrens syndrome?

Answer 49

Autoimmune disease of exocrine glands (salivary/ lacrimal)

Question 50

Difference between primary and secondary sjogrens?

Answer 50

Primary - absence underlying connective tissue disorder | Secondary - presence of underlying connective tissue disorder (RA/ SLE/ SS)

Question 51

What see in sjogrens?

Answer 51

Focal lymphocytic infiltration of salivary and lacrimal glands

Question 52

Is it more common to see sjogrens in SLE or RA?

Answer 52

SLE SLE 30% pt have secondary sjogrens RA 15% have secondary sjogrens

Question 53

Causes of sjogrens?

Answer 53

Genetic predisposition | Viral agent - herpes virus/ retrovirus/ EBV

Question 54

Pathogenesis of sjogrens?

Answer 54

``` Lymphocytic infiltration of exocrine glands Hypertrophy of ductal epithelium Formation epimyoepithelial islands Acinar atrophy and fibrosis Hyperactivity of B-cells ```

Question 55

Symptoms of xerostomia related sjogrens?

Answer 55

``` Difficulty swallowing Sensitivity spicy food Altered taste - metalic Burning mucosa Lack taste Salivary gland swelling ```

Question 56

Oral sign sjogrens?

Answer 56

Oral mucosa - dry/ atropy/ ulcerated Tongue - dry/ red/ lobulated Increased caries Salivary gland - swelling

Question 57

What criteria is used for diagnosis of sjogrens?

Answer 57

European diagnostic criteria

Question 58

How diagnosis sjogrens?

Answer 58

Biopsy of salivary glands

Question 59

What are the 6 classifications of signs and symptoms in sjogrens?

Answer 59

``` I - ocular symptoms II - oral symptoms III - ocular signs IV - histopathology V - salivary gland involvement VI - autoantibodies ```

Question 60

In regards to classification what makes it primary sjogrens?

Answer 60

Must have 4 out of 6 | MUST have positive histopathology and autoantibodies

Question 61

In regards to classification what makes it secondary sjogrens?

Answer 61

``` Have underlying CT AND presence of ocular OR oral symptoms AND 2 out of III, IV, V ```

Question 62

What are those w/ sjogrens at higher risk of?

Answer 62

Lymphoma - predominantly B cells

Question 63

Management of Sjogrens?

Answer 63

Palliative - - Increase lubtrication - salivary substitutes - Maintain oral/detanl health- OHE/ F- toothpaste - Review candida status Therapeutic - Pilocarpine - Immunomodulating agents