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Rheumatology > Scleroderma > Flashcards

Scleroderma Flashcards

1
Q

Features of scleroderma renal crisis include:

A

HTN emergency (85% with BP>178/100)
headache
MAHA, thrombocytopenia, high Cr (or AKI in the absence of prior kidney disease)
Normal urine sediment with mild proteinuria

can have a rare version without HTN

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2
Q

Treatment of Scleroderma renal crisis

A

ACE i or captopril

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3
Q

Limited cutaneous systemic sclerosis (SSc) features are

A

Raynaud’s phenomenon, GERD, skin changes like telangiectasias, thickened skin (CREST)

anti centromere antibody

See skin changes on face and neck and below the elbows.

Pulm arterial HTN

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4
Q

Biomarker that increases risk for scleroderma renal crisis

A

anti-RNA polymerase III antibodies

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5
Q

How does ACE i help stop scleroderma renal crisis?

A

stops interstitial fibrosis and vascular dysfunction in glomerular arterial bed

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6
Q

What can precipitate scleroderma renal crisis

A

Steroids.

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7
Q

what causes scleroderma renal crisis?

A

intense renal vasoconstriction stimulates the release of renin and generation of angiotensin II which can worsen HTN and lead to progressive renal dysfunction. ACE i can stop progression of scleroderma renal fialure

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8
Q

why do we like captopril for treatment of scleroderma renal crisis?

A

rapid onset of action

treatment should be continued even if renal function intially worsens as some pts may need dialysis following long term BP control with ACE I.

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9
Q

what is associated with scleroderma?

A

joint pains, finger puffiness, digital tip pits, GERD, pulmonary fibrosis (bibasilar interstitial lung disease, telangiectasias and thickened skin.

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10
Q

When does Raynauds phenomenon occur in systemic sclerosis?

A

can occur weeks to years prior to fibrosis.

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11
Q

what is systemic sclerosis

A

same thing as scleroderma

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12
Q

types of scleroderma / systemic sclerosis (affects the skin)

A

defined by the tissues of the disorder:

1 limited cutaneous systemic scleroderma - generally has pulmonary arterial HTN (may not have pulm interstitial fibrosis) and see CREST syndrome (calcinosis, Ryanaud’s phenomenon, esophageal dysmotility dysfunction, sclerodactyly and telangiectasias), no organ fibrosis. anti-centromere

2 diffuse cutaneous systemic scleroderma - fibrosis affects large areas of skin (torso and upper arms and legs) AND involves internal organs- see cardiac dx and renal dx. Condition worsens quickly. anti -Scl 70

  1. Systemic sclerosis sine scleroderma - (internal organ involvement only)

Can see some cross over between each type.

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13
Q

localized finger edema (generally bilateral) myalgia, arthralgias, Raynaud’s and GERD

A

systemic sclerosis presentation.

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14
Q

what medical condition is associated with scleroderma?

A

ILD and pulmonary HTN (70%) and this is what will end up killing most of these people with systemic sclerosis

Limited cutaneous scleroderma is at greater risk for lung cancer too.

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15
Q

what is the earliest sign of systemic sclerosis?

A

see (sclerodactyly) or diffuse swollen fingers and hands from thickening of skin on fingers and hands and see vascular complications of fingers so that there are digital infarcts and subungaual infections and ischemic skin ulceration.

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16
Q

In limited cutaneous scleroderma (LcSSC) what areas of the skin are affected

A

skin over fingers below the elbows, hands, face and neck

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17
Q

In diffuse cutaneous scleroderma (DcSSC), where is the skin thickening?

A

see MORE skin involvement so above the elbow and on trunk.

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18
Q

salt and pepper appearance of skin

A

called poikiloderma - areas of hyperpigmentation mixed with hypopigmentation give skin the look. This is seen as a part of cutaneous changes from scleroderma

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19
Q

Systemic sclerosis joint involvement

A

seen in 12-65% of pts
there is 1-5% overlap with RA and will have positive anti CCP and RF titers.

Basically have classic RA with scleroderma features

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20
Q

can Systemic sclerosis also cause muscle disease?

A

yes. myalagia and proximal muscle weakness are common with elevated CK and serum adolase elevated. EMG will show myopathic changes.

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21
Q

Pts who have diffuse systemic sclerosis AND have ILD and pulmonary HTN (70%) can also have:

A

overlap with polymyositis

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22
Q

how does limited cutaneous scleroderma affect the GI system?

A

see esophageal dismotility of the lower 2/3rds (affects smooth muscle) and see GERD.

See also increased incidence of Barrett’s esophagus and adenocarcinoma

also can have gastric dysmotility and see early satiety

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23
Q

GAVE

A

gastric antral vascular ectasia (watermelon stomach)

seen in Diffuse cutaneous scleroderma and pts with positive RNA polymerase III antibiodies.

24
Q

what GI manifestations do we also see with diffuse systemic scleroderma?

A

GAVE and small intestine bacterial overgrowth (see malabsorption and diarrhea) this is from small intestine dysmotility and see pseudoobstruction

can also see large intestine dysmotility and resulting

25
Q

how to we diagnose small bowel obstruction ?

A

hydrogen breath test and can treat with empiric trial of antibiotics. like rifaximin

26
Q

what are the lung complications of systemic sclerosis (scleroderma)

A

pleuritis, pleural effusion and interstial lung disease
can see pulmonary arterial HTN and bronciolitis and pulmonary veno occlusive dx and respiratory muscle weakness and skin involvement over the chest that prevents chest expansion

27
Q

which type of scleroderma (limited or systemic) gets ILD?

A

50% of pts with systemic scleroderma (and has anti Scl70 abs) has interstitial lung dx

30% of limited cutaneous scleroderma get ILD.

seen more in men and worsen dx in AA.

28
Q

what findings are seen on CT with someone who has scleroderma and ILD

A

see non specific interstitial pneumonitis followed by usual interstitial pneumonitis.

29
Q

what predicts higher mortality and worse prognosis for scleroderma pts?

A

abnormal FVC on PFTs early in disease course (first 5 yrs)

or more than 20% of lung affected on baseline high res CT

30
Q

when to get PFTs and high res CT scan in scleroderma pts?

A

get it at time of diagnosis since ILD is so common in pts

31
Q

surveillance PFTs and high res CT scan in scleroderma pts?

A

PFTs with DLCO every 6 to 12 months for next five years after diagnosis.

no need for repeat high res CT for routine surveillance

32
Q

when should we be concerned for progression of ILD in scleroderma?

A

when there’s a decline in FVC of 10% or DLCO of 15% within 12 months

33
Q

prevalence of pulmonary arterial HTN in systemic sclerosis?

A

10% of all pts.

PResent with exertional dyspnea

34
Q

signs of worsening pulmonary arterial HTN in scleroderma

A

chest pain and edema worsens.

35
Q

what to order in suspected pulmonary arterial htn

A

get echocardiogram TTE
and PFTs
FVC/DLCO ratio >1.6 is suggestive of diagnosis.

Needs Right heart cath for accurate diagnosis

36
Q

how often to get surveillance echocardiograms for pts who have pulmonary arterial HTN and scleroderma?

A

every year and more frequently for new or worsening symptoms.

37
Q

is there cardiac involvement with scleroderma

A

yes and incidence is 10% of pts and can lead to myocardial fibrosis and can lead to myocardial ischemia, heart failure, and arrhythmias. high mortality.

38
Q

Does prophylactic ACEi use in scleroderma prevent scleroderma renal crisis?

A

no but prophylactic CCB may help prevent this.

39
Q

Treatment for stabilizing scleroderma

A

cyclophosphamide is used for stabilizing scleroderma. limited effect and it’s impact doesn’t last more than a year

Mycophenolate mofetil can be helpful with stabilization too (with less side effects)

40
Q

long term therapy for scleroderma (and ILD)

A

Mycophenolate mofetil can be helpful with stabilization and longterm therapy (for pts with systemic sclerosis with ILD.

41
Q

Truth about treatment for scleroderma

A

most tx is about controlling symptoms and organ specific. not great.

42
Q

Diagnosis of Scleroderma

A

based on American College of Rheum weighted point system- #of manifestations, pulmonary HTN, presence of autoantibodies

43
Q

antibody associated with diffuse scleroderma

A

anti Scl 70 (anti topoisomerase 1)

can also see anti RNA polymerase III

44
Q

antibody associated with limited cutaneous scleroderma

A

Anti- centromere (C for CREST)

45
Q

Scleroderma mimic:

A 
eosinophilic fasciitis
nephrogenic systemic fibrosis
scleredema 
Scleromyxedema
Chronic graft vs host dx
drug and toxin exposure
46
Q

eosinophilic fasciitis

A

orange ppel induration of proximal extremities with sparing of hands and face and peripheral eosinophilia and skin retraction over superficial veins

47
Q

biopsy of eosinophilic fasciitis?

A

full thickness biopsy shows demonstrates lymphocytes, plasma cells and eosinphils in deep fascia

Tx via glucosteroids

48
Q

nephrogenic systemic fibrosis

A

from MRI and gadolinium in pts with ESRD causing brawny wood like induration of extremities (spares the digits)

49
Q

Tx of nephrogenic systemic fibrosis

A

no good tx. See skeletal muscle fibrosis with contractures and or cardiac muscle involvement and see cardiomyopathy

50
Q

Scleredema is

A

indurated plaques or patches on back, shoulder girdle and neck

seen in DM2

51
Q

Scleromyxedema is

A

waxy yellow red papules over thickened skin of face upper trunk neck and arms. See deposition of mucin with large number of stellate fibroblasts in dermis.

This is seen with paraproteinemia and seen in MM or AL amyloidosis (seen in men)

52
Q

Chronic graft vs host skin changes

A

see lichen planus like skin lesions or localized generalized skin thickening.

Seen after hematopoietic stem cell transplant and may be seen after blood transfusion in immunocompromised tp

53
Q

drug and toxin exposure can cause scleroderma like changes and these drugs are:

A

bleomycin, docetaxel, pentazocine, L tryptophan and organic solvents.

54
Q

what is morphea?

A

this is a type of sclerosis that is only seen with the skin

no other systemic manifestations of systemic slcerosis

55
Q

morphea is categorized as

A

limited or general

limited morphea - one or more discrete plaques of skin involvement where diffuse morphea can diffusely involve the trunk and limbs. Patients do not have the typical extracutaneous manifestations of systemic sclerosis like GERD, lung dx, bowel dysmotility or Raynaud’s phenomenon.

Rheumatology (29 decks)

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