Scleroderma

Question 1

What is the overlap between Raynaud’s and connective tissue disease?

Answer 1

If Raynaud’s plus anti-centromere Ab, Scl-70 or PM-Scl –> 30 - 60%

If Raynaud’s plus low titre ANA, then about 10%

Question 2

What are the types of scleroderma?

Answer 2

Firstly there is localised.

This can be called “morphea” which is patches of sclerotic skin

There is also “linear scleroderma” which is called “en coup de sabre” by those fancy Europeans

Next up is “systemic sclerosis”

This comes in two flavours - limited cutaneous SSc, which is limited to hands, face and neck (I believe the technical limits is beyond wrists) - slightly more assoc with CREST

and diffuse cutaneous SSc, which is all over. Diffuse is at greater risk of visceral disease, particularly renal, lung and cardiac.

Question 3

why is nifedipine the treatment of choice for SSc?

Answer 3

it has a vasodilator effect, but it also has an antiplatlet effect which is very useful

Question 4

What is the management of SCL?

Answer 4

organ based approach, but must avoid all cold exposure

aggressive treatment of HTN - this prevent acute renal failure

how to treat the calcinosis? maybe colchicine, diltiazem? nothing really works

penicilliamine is trialled, but actually has no evidence.

cyclophosphamide is helpful for lung involvement

steroids should be used cautiously because it can precipitate a hypertensive crisis. (this is a weird event that i don’t understand pathophysiology)

Question 5

What is the treatment of HUS/TTP renal crisis in SCL?

Answer 5

Plasmapheresis is important

Question 6

What would you see on a renal biopsy in scleroderma?

Answer 6

It is about the endothelium - you see arteriolar fibrosis and necrosis

Question 7

What is the treatment for hypomotility in scleroderma?

Answer 7

prokinetic agents such as cisapride can be useful.

Question 8

What is the treatment for ILD in scleroderma?

Answer 8

There are two phases of lung disease in scleroderma.

acute, active inflammation - high dose steroids and cyclophosphamide

pulmonary hypertension stage - calcium channel blockers, prostaglandin inhibitors, endothelin blockers such as Bosentan and phosphodiesterase inhibitors such as sildenafil

Question 9

What is mixed connective tissue disease?

the typical marker is what?

what are some of the classical arthridities?

Answer 9

it is a classic overlap syndrome.

it has elements usually either SLE or Scl, with polymyositis and possibly rheumatoid arthritis.

the marker is U1RNP (but any of the RNP Ab) - THIS HAS TO BE PRESENT, OTHERWISE IT IS CALLED OVERLAP SYNDROME

typically it causes a Raynaud’s with a DIP arthritis (which is a bit different to RA)

cardiac and renal involvement is common

Question 10

what is the treatment of MCTD?

Answer 10

we usually focus on the individual systems.

SLE - steroids and plaquenil
RA - NSAIDs, MTX
vasculitis - steroids and cyclophosphamide
lungs - 'roids and cyclophos
Scl - ACEi, CCB etc

Question 11

What are the differences between limited cutaneous and diffuse cutaneous SSc on blood results? (as in, what are the antibodies?)

Answer 11

anti-CENPD = anti-centromere = limited (CREST particularly)

anti-Scl is more like diffuse

Question 12

what is Sjogren’s syndrome and what are the blood tests?

Answer 12

Overall it is an autoimmune condition associated with ocular and oral dryness and autoantibodies.

Ro/SSA is also found in the “Sjogren’s like” SLE variant.

At the severe end are patients with florid salivary gland enlargement, adenopathy, antibodies to the Ro/SSA and La/SSB antigens, cryoglobulinemia, hypocomplementemia, a propensity to develop non-Hodgkin lymphoma, and other extraglandular disease manifestations.

At the mild end are patients with moderate symptoms of dry eyes, dry mouth, a low titer of antinuclear antibody, and vague symptoms of fatigue, myalgias, and cognitive dysfunction. In many patients with mild disease, distinguishing SS patients from individuals with fibromyalgia or depression who have ocular and/or oral dryness caused or exacerbated by medications with anticholinergic side effects is a major challenge

Question 13

what is the Schirmer test?

Answer 13

This is a test of the tear duct. Put special paper on the outer third of the bottom eye lid and see how quickly tears are absorbed.

Question 14

What is the Rose Bengal test?

Answer 14

it is also a test of eye damage - this one is to see if there is “devitalisation” of eye tissues. Throw on the stain and damaged stuff lights up. (view by slit lamp)

Question 15

what is tear-break up time?

Answer 15

test of lacrimal function using fluorosceine.

Question 16

what renal condition is associated with Sjogren’s syndrome?

Answer 16

It is a distal renal tubular acidosis. It is a failure of acid secretion

Question 17

xray shows loss of bone “tufts” (which are the distal part of the distal phalynx). What is the diagnosis?

Answer 17

this is a scleroderma problem

Question 18

if you do nail fold capillaroscopy, and see abnormal blood vessels

what is the cause?

Answer 18

well, a quick google suggests that capillaroscopy is really only useful for SSc diagnosis compared with primary raynaud’s

if there is abnormality (bleeding points) –> SSc

if there is normal –> Raynaud’s

(I think this is the truth, better look it up later)

Question 19

what is the treatment of a scleroderma renal crisis?

Answer 19

blood pressure management, particularly ACEi

yep, apparently the acute management is an ACEi