Scleroderma, Sjogren's & Myositis Flashcards
Scleroderma (Systemic Sclerosis)
etiology
Scleroderma (SS)
Etiology
- a systemic autoimmune disease with unknown origin, effecting all organ systems
- females > males
Three Features of the Disease
- Excessive deposition of ocllagen and other connective tissue molecules in skin and internal organs
- Vascular lesions of capillaries and small arteries
- alterations in the cellular and humoral immunity
high mortality rates: because the tissue fiberosis taht occurs in vital organs compromises function
Pathology - Collagen
- the excessive collagen deposition is due to overproduction of the collagen protein by fibroblasts
- there is alteration in the collagen regulation gene expression pathway due to cytokines and growth factors being released from the inflmmatory cells
- TGF-beta and SMAD pwathways
Pathology - Vascualr
- endothelial cell dysfunction: inital event the disease
- vascuarl abnormalities become apparent BEFORE tissue fiberosis
- cellular infiltrates with T cells and macrphages
Pathology - Immunity
- ANA pathology: triggers over activation of the system
Scleroderma
organ systems most commonly invovled
Organ Systems Involved
- always skin: collagen buildup
- Raynauds! (vasucular)
- GI tract
- lungs
can be (less comon)
bones/joints, muscles, heart, kidneys
Scleroderm: Skin Symptoms
limited v diffuse
face findings
other key signs
virtually everyone with scleroderma has skin findings
if they dont have the skin = scleroderma sine scleroderma
Symptoms
- thickened skin appears distally first and progresses proximally
- Limited Scleroderma: hands, feet, face, forearms and legs
- diffuse Scleroderma: upperarms, thighs, chest, abdomen also invovled
Face
- face becomes expressionless: no wrinkles
- talengiectagias
Diffused Hyper/hypo-pigmentation
- areas near hiar follicles are depigmeneted
Painful Ulcerations: fingertips and areas where the skin is so tight
cutaneous calcifications at fingertips (calcium deposits)
Raynauds and Scleroderma
Other Vascualture Symptoms of Scleroderma
Scleroderma: raynauds is the vascualr finding
- often happens before the skin findings
Raynauds
- temporary occculsion: white then blue, then excessve vasodilation leading to rednes and pain
- develops in virtually all pt. with scleroderma
Vascualr Symptoms
- capillarie heohrrages at the cuticle line : “WIre Loop” as the vessels try to go around the collagen blockage and divert
- necrotici lesions in the fingers and toes emergency
Scleroderma: Skeletal Symptoms
Skeletal Involvement
- symmetrical polyarthrialgia (pain) but TRUE arthritis or synovitis are rare
- contractures of teh hands and wrists with lost hand function
- carpel tunnel
- coarse leathery crepitus (tendon friction rub) rare but specific for diffuse scleroderma (since chest)
Xray Findings
- shows resporbtion and dissolution of teh distal phalanx : such bad circulation the bone goes away
- muscle fiber atrophy & possible polymyositis
Scleroderma: GI changes
GI Symptoms
- esophageal abnormalities in 90%
- incomplete LES and decreased/gone peristalsis leads to EXTREME REFLUX, odyniphagia and dysphaiga
- barretts esopahgus (and icnreased transition to CA) and lower esophageal stricture can occur
- causes hoarsness, and aspiration to pneumonitis
watermelon stomach: when there are big vessles that are dilated trying to compensate: high risk of GI bleeding
disordered gut motility: malabosrbtion, diarrhea, bacterial overgrowth
colonic disease with psueodiverticular (wide-mout sacculation)
Scleroderma: Respiratory Changes and Symptoms
tests for detecing these changes
lung involvement is the leading cause of morbitiy/mortality
Results in Pulmonary Fiberosis & Pulmonary Hypertension
PUlmonary Fiberosis
- insidious in onset
- hear fine inspiratory crackels as the lungs start to harden at the bases
Pulmonary Hypertension & Cor Pulmonale
- in those with limited and diffuse
Tests for Pulmonary invovledment
PFTs are the best
- if DLCO is low = pulmonary HTN
- if FVC/DLCO are both decreased = pulmonary fiberosis
CT can see the alvolitis: ground glass appearnce
Pulm HTn (lmitied)
interstital dx. (diffuse)
Renal Involvment in Scleroderma
renal involvement is the most deadly
- less commong, but it happens QUICKLY
- severe HTN and volume overload = lead to the crisis
Scleroderma Renal Crisis
- HTN, cardiac failure, MI, stroke = renal crisis
can be countered with prompt adminstration of ACE inhibitor
diffuse scleroderma at highest risk
Cardiac Involvement of Scleroderma
Cardiac
- heart will be effected; but symptomaitc andcardiac dysfunction less common
Tachyarrythmias and pericardial effusions are seens
Right heart strain due to pulmnary HTN can happen
Organ Invovlement Scleroderma
- neuro
- GU
- thyroid
- bile
Nero: periphearl neuropathy CNS is typically spared
GU: erectile dysfunction
thyroid: hypothyroid
can give secondayr sjogrens syndrome
pirmary biliar cirrhosis can happen too
Scleroderma: Diagnosis & prognosis
Diagnosis is made clinically
lab data can help decide what type of scleroderma
- ESR = usually normal
- ANA is typically positive in 90% +:
- diffuse = speckled or nucelor formation
- anti-centromere = limited
ANA findings
anti-centromere is most important = limited
anti-SCL70 (topoisomerase I) + RNA polymerase = diffuse
anti-scl70 = increased risk of pulmonary interstital involvement
Prognosis
highest risk in first 2-5 years
diffuse = shorter life span
limited = a bit longer
renal, cardiac or pulm involvemen t= worse off
Sjogrens Syndrome
Etiology
Etiology
- a chronic autoimmune disorder or the exocrine: mositrue producing glands
- creates a “Sicca Syndrome” of dry mouth and dry eyes
- reduced lacrimal and salivary gland function due to autoantibodies attacking
- second most common autoimmune after RA
Primary Sjogrens
- the dry eyes and mouth in a previously healthy individaul
Secondary Sjogrens
- the association of sjogrens from antoher rhum. disease: usually RA but also SLE too
Sjogrens Syndrome
Symptoms
Occular Specifics
slow, benging course of disease progression
Dry eyes and Dry Mouth = initial presenation for most pt.
Classic Traid
- keratoconjunctivitis sicca (dry eyes)
- dry mouth (xerostomia)
- arthritis
Occular Involvement
- chronic inflmamed lacrimal = decrease tear production
- dilation of conj. vessels & pericornal injection = red eye
- irregualr cornea
- burnchin, sandy, scratchy sensation and photosensitivtiy
- failure to treat dry eyes = ulcers and corneal perforation/melting and infection
Asked via these questions
- FB sensation in teh eye ?
- dry eye for 3+ months?
- using artifical tears 3+ months
Sjogrens Syndrome: Symptoms
Oral Involvement
Dry mouth due to decrease saliva production by the glands
- difficult to swallow
- inability to speak continuously
- changes in taste
- buring sensation in mouth
- **parotid and salivary galnd enlargement can occur in 60% of pt. **due to the destruction
the dry mouth can lead to
- cavities
- sialolithiasis (stones)
- oral candidias
- infections
- weight loss
Ask…
sympomts for 3+ months
recurrent or persistnat swollen glands
need liquid to swallow dry food
Sjogrens Syndrome : Symptoms
Arthritis
symmetrical polyarthritis simialr to RA but milder and non-erosive
