Scleroderma Flashcards
Features of scleroderma renal crisis include:
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
Treatment of Scleroderma renal crisis
ACE i or captopril
Limited cutaneous systemic sclerosis (SSc) features are
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
Biomarker that increases risk for scleroderma renal crisis
anti-RNA polymerase III antibodies
How does ACE i help stop scleroderma renal crisis?
stops interstitial fibrosis and vascular dysfunction in glomerular arterial bed
What can precipitate scleroderma renal crisis
Steroids.
what causes scleroderma renal crisis?
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
why do we like captopril for treatment of scleroderma renal crisis?
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.
what is associated with scleroderma?
joint pains, finger puffiness, digital tip pits, GERD, pulmonary fibrosis (bibasilar interstitial lung disease, telangiectasias and thickened skin.
When does Raynauds phenomenon occur in systemic sclerosis?
can occur weeks to years prior to fibrosis.
what is systemic sclerosis
same thing as scleroderma
types of scleroderma / systemic sclerosis (affects the skin)
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
- Systemic sclerosis sine scleroderma - (internal organ involvement only)
Can see some cross over between each type.
localized finger edema (generally bilateral) myalgia, arthralgias, Raynaud’s and GERD
systemic sclerosis presentation.
what medical condition is associated with scleroderma?
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.
what is the earliest sign of systemic sclerosis?
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.
In limited cutaneous scleroderma (LcSSC) what areas of the skin are affected
skin over fingers below the elbows, hands, face and neck
In diffuse cutaneous scleroderma (DcSSC), where is the skin thickening?
see MORE skin involvement so above the elbow and on trunk.
salt and pepper appearance of skin
called poikiloderma - areas of hyperpigmentation mixed with hypopigmentation give skin the look. This is seen as a part of cutaneous changes from scleroderma
Systemic sclerosis joint involvement
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
can Systemic sclerosis also cause muscle disease?
yes. myalagia and proximal muscle weakness are common with elevated CK and serum adolase elevated. EMG will show myopathic changes.
Pts who have diffuse systemic sclerosis AND have ILD and pulmonary HTN (70%) can also have:
overlap with polymyositis
how does limited cutaneous scleroderma affect the GI system?
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
GAVE
gastric antral vascular ectasia (watermelon stomach)
seen in Diffuse cutaneous scleroderma and pts with positive RNA polymerase III antibiodies.
what GI manifestations do we also see with diffuse systemic scleroderma?
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
how to we diagnose small bowel obstruction ?
hydrogen breath test and can treat with empiric trial of antibiotics. like rifaximin
what are the lung complications of systemic sclerosis (scleroderma)
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
which type of scleroderma (limited or systemic) gets ILD?
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.
what findings are seen on CT with someone who has scleroderma and ILD
see non specific interstitial pneumonitis followed by usual interstitial pneumonitis.
what predicts higher mortality and worse prognosis for scleroderma pts?
abnormal FVC on PFTs early in disease course (first 5 yrs)
or more than 20% of lung affected on baseline high res CT
when to get PFTs and high res CT scan in scleroderma pts?
get it at time of diagnosis since ILD is so common in pts
surveillance PFTs and high res CT scan in scleroderma pts?
PFTs with DLCO every 6 to 12 months for next five years after diagnosis.
no need for repeat high res CT for routine surveillance
when should we be concerned for progression of ILD in scleroderma?
when there’s a decline in FVC of 10% or DLCO of 15% within 12 months
prevalence of pulmonary arterial HTN in systemic sclerosis?
10% of all pts.
PResent with exertional dyspnea
signs of worsening pulmonary arterial HTN in scleroderma
chest pain and edema worsens.
what to order in suspected pulmonary arterial htn
get echocardiogram TTE
and PFTs
FVC/DLCO ratio >1.6 is suggestive of diagnosis.
Needs Right heart cath for accurate diagnosis
how often to get surveillance echocardiograms for pts who have pulmonary arterial HTN and scleroderma?
every year and more frequently for new or worsening symptoms.
is there cardiac involvement with scleroderma
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.
Does prophylactic ACEi use in scleroderma prevent scleroderma renal crisis?
no but prophylactic CCB may help prevent this.
Treatment for stabilizing scleroderma
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)
long term therapy for scleroderma (and ILD)
Mycophenolate mofetil can be helpful with stabilization and longterm therapy (for pts with systemic sclerosis with ILD.
Truth about treatment for scleroderma
most tx is about controlling symptoms and organ specific. not great.
Diagnosis of Scleroderma
based on American College of Rheum weighted point system- #of manifestations, pulmonary HTN, presence of autoantibodies
antibody associated with diffuse scleroderma
anti Scl 70 (anti topoisomerase 1)
can also see anti RNA polymerase III
antibody associated with limited cutaneous scleroderma
Anti- centromere (C for CREST)
Scleroderma mimic:
eosinophilic fasciitis nephrogenic systemic fibrosis scleredema Scleromyxedema Chronic graft vs host dx drug and toxin exposure
eosinophilic fasciitis
orange ppel induration of proximal extremities with sparing of hands and face and peripheral eosinophilia and skin retraction over superficial veins
biopsy of eosinophilic fasciitis?
full thickness biopsy shows demonstrates lymphocytes, plasma cells and eosinphils in deep fascia
Tx via glucosteroids
nephrogenic systemic fibrosis
from MRI and gadolinium in pts with ESRD causing brawny wood like induration of extremities (spares the digits)
Tx of nephrogenic systemic fibrosis
no good tx. See skeletal muscle fibrosis with contractures and or cardiac muscle involvement and see cardiomyopathy
Scleredema is
indurated plaques or patches on back, shoulder girdle and neck
seen in DM2
Scleromyxedema is
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)
Chronic graft vs host skin changes
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
drug and toxin exposure can cause scleroderma like changes and these drugs are:
bleomycin, docetaxel, pentazocine, L tryptophan and organic solvents.
what is morphea?
this is a type of sclerosis that is only seen with the skin
no other systemic manifestations of systemic slcerosis
morphea is categorized as
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.
