Connective Tissue Disease 2

Question 1

name some rheumatological autoimmune diseases

Answer 1

SLE
Sjogren`s syndrome
Systemic sclerosis
Mixed connective tissue disease
Inflammatory myositis(Polymyositis/Dermatomyositis)
Anti-phospholipid antibody syndrome

Question 2

name the first phase in Raynauds

Answer 2

phase 1 - Ischemia

Question 3

name the second phase in Raynauds

Answer 3

phase 2 - cyanosis

Question 4

name the third phase in Raynauds

Answer 4

phase 3 - rubor

Question 5

who does Raynauds affect?

Answer 5

teenagers - benign

elderly - secondary to underlying autoimmune disease

Question 6

what is the treatment in Raynauds?

Answer 6

keep warm
vasodilators - Calcium channel blockers
PDE5 inhibitors - slinenafil

Question 7

name the 2 types of systemic sclerosis

Answer 7

varieties-Diffuse cutaneous systemic sclerosis (dsCC)and limited cutaneous systemic sclerosis(lsCC)

Question 8

what did systemic sclerosis use to be called?

Answer 8

scleroderma (skin thickening)

Question 9

name the skin involvement in limited cutaneous SSc

Answer 9

only below elbows and knees plus face

Question 10

what is the acronym in systemic sclerosis?

Answer 10

CREST

Question 11

name the manifestations in systemic sclerosis

Answer 11

sclerodactyly
telangiectasia
pulmonary fibrosis
pulmonary hypertension
scleroderma renal crisis

Question 12

what is the antibody that is important in systemic sclerosis?

Answer 12

anticentromere

Question 13

name the management in systemic sclerosis

Answer 13

Yearly ECHO and PFTs.
Treat Raynaud`s and digital ulcers.
Treat reflux with PPi +/_ranitidine+/_Gaviscon .
Pulmonary fibrosis- immunosuppression.
Rapidly progressive skin involvement-immunosuppression.
Pulmonary hypertension-Prostacyclin analogues, endothelin receptor antagonists, PDE5 inhibitors.
Tight control of blood pressure-ACE INHIBITORS.

Question 14

what is Sjogrens syndrome characterised by?

Answer 14

lymphocytic infiltration of exocrine glands

Question 15

what is typical of sjogrens syndrome?

Answer 15

Blepharitis.
Salivary gland inflammation.
Tooth decay.
Lymphoma.
Dry cough.
Multisystem involvement

Question 16

what is the key word in sjogrens syndrome?

Answer 16

dryness

Question 17

name the 2 antibodies important in sjogrens

Answer 17

Anti Ro and Anti La

Question 18

how do you diagnose sjogrens?

Answer 18

antibodies

salivary gland US and biopsy

Question 19

what is the treatment in sjogre’s syndrome?

Answer 19

Therapeutic spectrum.

Artificial tears and salivary supplements,vaginal lubricants.

Good dental hygiene-strong fluoride toothpaste-e.g.Duraphat.

Hydroxychloroquine for fatigue and arthralgia.

Immunosuppression for major organ involvement.

Question 20

how do you diagnose antiphospholipid antibody syndrome?

Answer 20

presence of antiphospholipid antibodies on 2 occasions 12 weeks apart