Scleroderma

Question 1

Features of scleroderma renal crisis include:

Answer 1

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

Question 2

Treatment of Scleroderma renal crisis

Answer 2

ACE i or captopril

Question 3

Limited cutaneous systemic sclerosis (SSc) features are

Answer 3

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

Question 4

Biomarker that increases risk for scleroderma renal crisis

Answer 4

anti-RNA polymerase III antibodies

Question 5

How does ACE i help stop scleroderma renal crisis?

Answer 5

stops interstitial fibrosis and vascular dysfunction in glomerular arterial bed

Question 6

What can precipitate scleroderma renal crisis

Answer 6

Steroids.

Question 7

what causes scleroderma renal crisis?

Answer 7

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

Question 8

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

Answer 8

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.

Question 9

what is associated with scleroderma?

Answer 9

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

Question 10

When does Raynauds phenomenon occur in systemic sclerosis?

Answer 10

can occur weeks to years prior to fibrosis.

Question 11

what is systemic sclerosis

Answer 11

same thing as scleroderma

Question 12

types of scleroderma / systemic sclerosis (affects the skin)

Answer 12

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

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

Can see some cross over between each type.

Question 13

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

Answer 13

systemic sclerosis presentation.

Question 14

what medical condition is associated with scleroderma?

Answer 14

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.

Question 15

what is the earliest sign of systemic sclerosis?

Answer 15

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.

Question 16

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

Answer 16

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

Question 17

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

Answer 17

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

Question 18

salt and pepper appearance of skin

Answer 18

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

Question 19

Systemic sclerosis joint involvement

Answer 19

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

Question 20

can Systemic sclerosis also cause muscle disease?

Answer 20

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

Question 21

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

Answer 21

overlap with polymyositis

Question 22

how does limited cutaneous scleroderma affect the GI system?

Answer 22

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

Question 23

GAVE

Answer 23

gastric antral vascular ectasia (watermelon stomach)

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

Question 24

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

Answer 24

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

Question 25

how to we diagnose small bowel obstruction ?

Answer 25

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

Question 26

what are the lung complications of systemic sclerosis (scleroderma)

Answer 26

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

Question 27

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

Answer 27

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.

Question 28

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

Answer 28

see non specific interstitial pneumonitis followed by usual interstitial pneumonitis.

Question 29

what predicts higher mortality and worse prognosis for scleroderma pts?

Answer 29

abnormal FVC on PFTs early in disease course (first 5 yrs) or more than 20% of lung affected on baseline high res CT

Question 30

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

Answer 30

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

Question 31

surveillance PFTs and high res CT scan in scleroderma pts?

Answer 31

PFTs with DLCO every 6 to 12 months for next five years after diagnosis. no need for repeat high res CT for routine surveillance

Question 32

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

Answer 32

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

Question 33

prevalence of pulmonary arterial HTN in systemic sclerosis?

Answer 33

10% of all pts. PResent with exertional dyspnea

Question 34

signs of worsening pulmonary arterial HTN in scleroderma

Answer 34

chest pain and edema worsens.

Question 35

what to order in suspected pulmonary arterial htn

Answer 35

get echocardiogram TTE and PFTs FVC/DLCO ratio >1.6 is suggestive of diagnosis. Needs Right heart cath for accurate diagnosis

Question 36

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

Answer 36

every year and more frequently for new or worsening symptoms.

Question 37

is there cardiac involvement with scleroderma

Answer 37

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.

Question 38

Does prophylactic ACEi use in scleroderma prevent scleroderma renal crisis?

Answer 38

no but prophylactic CCB may help prevent this.

Question 39

Treatment for stabilizing scleroderma

Answer 39

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)

Question 40

long term therapy for scleroderma (and ILD)

Answer 40

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

Question 41

Truth about treatment for scleroderma

Answer 41

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

Question 42

Diagnosis of Scleroderma

Answer 42

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

Question 43

antibody associated with diffuse scleroderma

Answer 43

anti Scl 70 (anti topoisomerase 1) can also see anti RNA polymerase III

Question 44

antibody associated with limited cutaneous scleroderma

Answer 44

Anti- centromere (C for CREST)

Question 45

Scleroderma mimic:

Answer 45

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

Question 46

eosinophilic fasciitis

Answer 46

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

Question 47

biopsy of eosinophilic fasciitis?

Answer 47

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

Question 48

nephrogenic systemic fibrosis

Answer 48

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

Question 49

Tx of nephrogenic systemic fibrosis

Answer 49

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

Question 50

Scleredema is

Answer 50

indurated plaques or patches on back, shoulder girdle and neck seen in DM2

Question 51

Scleromyxedema is

Answer 51

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)

Question 52

Chronic graft vs host skin changes

Answer 52

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

Question 53

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

Answer 53

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

Question 54

what is morphea?

Answer 54

this is a type of sclerosis that is only seen with the skin no other systemic manifestations of systemic slcerosis

Question 55

morphea is categorized as

Answer 55

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.