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Flashcards in Connective Tissue Disorders Deck (18)
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1

Sjogrens Syndrome:
-defiinition
-what is the primary form? secondary?
-what is the sicca complex?
-pathogenesis

def: chronic inflammatory disorder characterized by diminished lacrimal and salivary gland function. (autoimmune dz)

Primary form: not associated with any other dz

Secondary: tends to complicate other Rheumatic conditions, MC dz associated with secondary is RA.

Sicca complex= old name for SS, keratoconjunctivitis, xerostomia.


Pathogenesis:
-not well understood
-lymphocytic infiltrative rather than aby mediated.
(lymphocytic infiltration into lacrimal and salivary glands, where lymphocytes are not normally found, impairs function)

2

Sjogrens Syndrome:
-what are some mild sx? severe?
-what are some of the sx of extraglandular involvement?

Mild: dry eyes and mouth, maybe fatigue, myalgias, and cognitive dysfunction,

Severe: florid salivary enlargement, adenopathy, along with dry eyes and mouth.
*extraglandular involvement 25% and non-hodgkins lymphoma developed in 2.5%

Extraglandular involvement sx:
-vaginal dryness-- dyspareunia
-interstitial nephritis
-recurrent rhinitis and sinusitis
-bronchial dryness leading to dry cough

3

Sjogrens Syndrome:
-clinical manifestations
-criteria for dx

Manifestations:
-keratoconjunctivitis = gritty/sandy sensation, dry eyes
-xerostomia = dry
mouth

-salivary gland enlargement = firm and nontender. (MC is parotid)

Dx Criteria:
-ocular sx of inadequate tear production
-ocular signs of corneal damage
-oral sx of decreased salivary production
-gland histopathology
-presence of auto-abys *anti-Ro/SSA and/or anti-La/SSB

4

Complications of Xerostomia and keratoconjunctivitis in sjogrens syndome.

Keratoconjunctivitis:
-corneal abrasion
-infection

Xerostomia:
-dental caries
-oral candidiasis
-laryngotracheal reflux= freqeuent throat clearing, cough, substernal pain
-chronic esophagitis = d/t impaired clearance of acid and lack of the buffering effects of saliv
-weight loss
-nocturia

5

Primary Sjogrens Syndrome:
-dx criteria of primary and secondary SS
-Tests used in dx of SS

Dx: PRIMARY- no other CT disorder.
-positive salivary gland bx or anti-Ro/SSA or/and anti-La/SSB and satisfies a total of 4 of 6 items.
-items ocular signs, bx, salivary gland involvment, or autoabys

SECONDARY: a CTD is present and both of the following criteria are met:
-one item indicitive of ocular or oral dryness present
-any two of the following 3 objective items are present:
--ocular signs
--positive salivary gland bx
-abnormal test of salivary gland dysfunction

Dx tests:
-schirmer = tear test, measuring fluid level on filter paper, less than 5mm is abnormal.

-Rose Bengal test

-labs: CBC w/ diff, CMP, sed rate UA (sugar and protein)

- call the rheumatologist...

6

Sjogrens Syndrome:
-tx

Tx:
-topical therapy of dry eyes and dry mouth.
*management is done best by rheumatologist*
-avoid irritants or exacerbating drugs, low humidity atomospheres
-excellent oral hygiene
-topical moisturizers and lozenges
-stimulate salivary flow w/ sugarless candies (too much sorbitol can cause diarrhea)
-artificial saliva
-muscarinic agonists (cevimeline)
-replacement of tears: hypromellose or methylcellulose
-Topical cyclosporine (Restatsis)******

*if systemic dz then use hydroxychloroquine or methotrexate

7

Scleroderma:
-what is the difference between limited scleroderma and systemic sclerosis?

Limited scleroderma = CREST.
Systemic sclerosis is when the skin is affected but also with systemic internal organ involvement.

8

Systemic Sclerosis:
-peak onset in age
-pathophys

Age 30-50

Pathophys:
-vascular damage; ENDOTHELINS cause vasoconstriction & are fibrinogenic
-immune activation: activation of endothelial cells results in up regulation of adhesion molecules & leukocyte migration out of vasculature
-excessive synthesis of extracellular matrix w/ deposition of structurally normal collagen

9

Systemic Sclerosis: -dx

Dx:
-based upon the presence of charactersitic clinical findings
*the combination of skin induration pluse ONE or more of the following:
-heartburn and/or dysphagia of new onset
-acute onset of HTN* and renal insufficiency*
- dyspnea on exertion associated with interstitial pulm changes
-pulmonary HTN*
-diarrhea with malabsorption
-facial, lip, or hand telangiectasias
-digital infarctions aand or digital tip pitting

10

Systemic Sclerosis:
-tx

Tx:
-no tx that comprehensively addresses this dz. Tx is directed at specific organ manifestations:

-cutaneous manifestations = emollient creams to soften and decrease itch. Antihistamines and doxepin, potent topical steroids and methotrexate.

-raynauds = prevention, Ca channel blockers (nifedipine or amlodipine), alpha blockers, low dose ASA

-musculoskeletal

-GI = GERD is treated with prevention, PPI, promotility agent (azithromycin)

-Pulm manifestations = lung fibrosis; ground glass on CT. Rx cyclophosphamide

-cardiac manifestations= antiarrhythmics and cardiac pacing if needed.

-renal manifestations=
ACEi

-Erectile dysfunction* & dyspareunia

11

Systemic Sclerosis:
-what are the cutaneous manifestations?

Cutaneous:
-thickening and hardening of the skin of the fingers, hands, and face are the earliest.*
-pruritus*
-edema
-digital ulcers
-pitting at the fingertips
-telangiectasia

12

What is the MC initial manifestation of scleroderma?

Raynauds phenomenon is the initial manifestation of slceroderma.

13

Raynauds phenomenon:
-sx & cause
-when is this considered primary taynauds?? secodnary?
-Tx

Cause: exaggerated vascular response to cold temperature of emotional stress. Abnormal vasoconstriction of digital arteries and cutaneous arterioles.

Sx:
-sharply demarcated color changes (blue or white) of the skin of the digitis.
-with rewarming the skin blushes upon recovery.

Primary raynauds if these sx occur alone without evidence of any associated disorder.

Secondary if presenece of the disorder in association with related illness such as scleroderma.

Tx:
-prevention, Ca channel blockers (nifedipine or amlodipine), alpha blockers, low dose ASA

14

What is CREST syndrome? aka? Complications?

Calcinosis, Raynauds phenomenon, Esophageal dysmotility, sclerodactyly, Telangiectasia

aka: limited cutaneous slceroderma

Complications: pulm HTN and CHF.

15

Polymyositis/Dermatomyositis:
-definition
-pathophys
-what three rashes are MC seeN?

Def: inflamm muscle dz that can occur with or without eruption. with eruption - dermatomyositis and without is polymyositis

Pathophys: inflamm change of myocytes leads to proximal muscle weakness.

3 rashes:
-Gottrons Sign (PIP and MCP joints)
-Purple heliotrope eruption over eyelids.
-Shawl sign over anterior chest and over the shoulders.

16

Polymyositis/Dermatomyosities:
-sx
-dx

Sx:
-earliest is proximal muscle weakness, onset is insidious with gradual worsening over a period of several months.
-muscle weakness is symmetric and proximal.

Dx:
-clinical
-Check CPK, LDH, AST/ALT
-elevated ANA
-Muscle Bx
*medical hx to see if they are on statins since they cause myalgias.

17

Dermatomyositis:
-pathogenesis

Polymyositis:
-pathogenesis

pathogenesis:
-humorally-mediated
-cellular infiltrate focused around blood vessels*

Polymyositis pathogenesis:
-cellular infiltrate within the fascicle** (just the muscles)

18

Poly/Dermatomyositis:
-tx

Tx:
-cutaneous manifestations: mild-moderate topical steroids
-hydroxychlorquine or methotrexate
-Muscular manifestations:
high dose systemic steroids, PT, methotrexate and azathioprine may be initiated. (before starting methotrexate you need to get high dose steroids on board.)