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What is Sjogren's syndrome (SS)? Diff forms?

- chronic inflammatory disorder characterized by diminished lacrimal and salivary gland fxn
- primary form: not assoc w/ other diseases
- secondary: tends to complicate other rheumatic conditions (MC disease assoc w/ secondary SS is RA)
sicca complex: dry eyes/mouth


Pathogenesis of SS?

- not well understood
- considered lymphocytic infiltrative rather than ab mediated: lymphocytic infiltration into lacrimal and salivary glands, where lymphocytes are not normally found, impairs fxn
- thought to share common pathogenic features w/ other autoimmune diseases such as SLE


Mild to severe end of the spectrum of SS?

- mild end: moderate sxs of dry eyes, dry mouth, and maybe vague sxs of fatigue, myalgias, and cognitive dysfxn. These pts tend to have a low titer of ANA
- severe end: florid salivary gland enlargement, adenopathy, abs to Ro/SSa and La/SSB ags, cryoglobulinemia, hypocomplementemia, a propensity to develop non-Hodgkins lymphoma, and other extraglandular disease manifestations
- in a study done w/ 80 pts:
keratoconjunctivitis sicca and/or xerostomia occurred in ALL pts and were the only disease manifestation in 31%
- extraglandular involvement occurred in 25%
- non-hodgkin's lymphoma developed in 2.5%


Extraglandular involvement of SS?

- vaginal dryness in women -dyspareunia
- interstitial nephritis
- involvement of exocrine glands of the upper airways - can have recurrent rhinitis and sinusitis
- bronchial dryness: lead to dry cough - mistakenly tx for asthma or bronchitis


Which gland is MC affected?

- parotid gland


Ocular sxs of SS? What ?s can you ask your pt?

- keratoconjunctivitis sicca aka dysfxnl tear syndrome
- deficiency in tear production leading to sxs of dry eyes which usually presents insidiously over several yrs
- ask:
do you often feel a gritty or sandy sensation in your eys?
Have you had sxs of dry eyes on daily basis for more than 3 months?
how often do you use eye drops?


What are complications of KCS (keratoconjunctivitis sicca)?

- corneal ulceration
- infection of eyelid
- won't be able to tolerate contacts


Oral sxs of SS? questions to ask?

- xerostomia: pt complains of mouth dryness
- ask:
Do you have to wake up at night to drink water b/c your mouth is so dry?
Do you frequently drink liquids to help in swallowing dry foods?


Complications of xerostomia?

- dental caries: 65% of pts
- oral candidiasis: develops in 30-70% of pts, may cause oral pain, burning, and intolerance to spicy foods
- gingival recession
- other types of oral infections, such as bacterial infections of stensen's duct
- laryngotracheal reflux: which may lead to frequent throat clearing, cough, substernal pain and nocturnal awakening that stimulates panic attacks - result of decreased salivary flow, absence of normal gastric acid buffer, and reflux of gastric acid into esophagus and trachea
- chronic esophagitis: due to impaired clearance of acid and lack of buffering effects of saliva
- wt loss
- nocturia: drinking so much water


How will salivary gland enlargement present?

- occurs in 30-50% of pts w/ SS at some pt
- glands are typically firm, diffuse, and nontender
- can occur in both parotid, and submandibular glands - MC in parotid


Criteria for dx SS?

- ocular sxs of inadequate tear production
- ocular signs of corneal damage
- oral sxs of decreased saliva production
- salivary glands histopathology demonstrating foci of lymphocytes
- tests indicating impaired salivary gland fxn
- presence of autoabs (anti-Ro/SSA and/or anti-La/SSB)


Exclusion criteria for SS?

- prior head and neck irradiation
- infection w/ hep C
- lymphoma
- sarcoidosis
- graft versus host disease
- recent use of anti-cholinergic meds


Dx of primary SS?

- pts have no assoc CTD and exclusionary dxs cited
- pt has either positive salivary gland bx or anti-Ro/SSA and.or Anti-La/SSB and satisfies a total of 4/6 items
- the pt satisfies 3/4 objective items (ocular signs, bx, salivary gland involvement, or autoabs)


Dx of secondary SS?

made if CTD is present and both of the following criteria are met:
- at least 1 item indicative of ocular or oral dryness is present
- any 2 of the following 3 objective items are present:
ocular signs
positive salivary gland bx
abnormal tests of salivary gland fxn


Dx SS? (labs)?

- no single dx test
- thorough H and P
- following tests are recommended for use in clinical practice when SS is suspected:
- schirmer test
- rose bengal test
- basic lab testing:
CBC w/ diff
sed rate
UA (proteins, sugars)


Specialized lab testing for SS (most likely done by rheum)?

- ANAs
- RF
- abs to Ro/SSA, La/SSB, Sm, and RNP ags
- ab to anti-centromere B or topoisomerase, in pts w/ nucleolar pattern of ANA and sxs of overlap w/ systemic sclerosis
- ANCA in pts w/ evidence of vasculitis
- serum C3 and C4
- abs to ds-DNA, in pts w/ proteinuria
- serum cryoglobulins
- quantitive immunoglobulin levels (IgG, IgM, IgA)


How often are abs to Ro/SSA and La/SSB found in pts w/ SS?

- Anti-Ro/SSA abs found approx 70-97% of pts, high titers of these abs are assoc w/ greater incidence of extraglandular features - esp purpura, cutaneous vasculitis, and a demyelinating syndrome resembling MS
- anti-Ro/SSA abs are present in only 10-15% of pts w/ secondary SS assoc w/ RA

- Abs to La/SSB: unusual to encounter sera that contain anti-La/SSB activity w/o demonstrable abs to Ro/SSA in pts w/ SLE or SS


general Tx guidelines for SS?

- topical therapy for dry eyes and dry mouth
- tx of systemic manifestations of SS and recognition of therapies for other conditions that can exacerbate dryness complaints (when pt has extraglandular sxs and esp when there is assoc RA or other autoimmune disease, management is best done by a rheumatologist)


tx for sicca sxs?

- avoidance of irritants
- avoidance of exacerbating drugs
- avoidance of low humidity atmospheres
- a dry mouth isn't a painful mouth (consider candidiasis)
- irritated eyes despite sufficient hydration (consider blepharitis)
- excellent oral hygiene including brushing teeth, flossing, and dental prophylaxis is really impt
- topical moisturizers and lozenges


Tx for xerostomia - SS?

stim of existing salivary flow:
-sugarless candies
-dried fruit slices
-citrus flavored sugarless tabs (salivasure)
-sugar free gums, containing various sweetners - (educate pt that too much sorbitol cna cause diarrhea)
- muscarininc agonists (cevimeline)
- artificial saliva


Tx for KCS?

replacing or supplementing deficient tears:
- replacement of aqueous layer w/ hypromellose 0.3% of methylcellulose 0.3% (beneficial when the watery component is deficient as is usual in SS)
- spreading agents (dextran) help the aqueous layer spread over hydrophobic corneal and conjunctiva
- longer acting lubricant ointment at nigh
- topical cyclosporine (restasis)***


Immunosuppressive tx of systemic SS?

- hydroxychloroquine
- methotrexate


What is scleroderma? When does it become systemic sclerosis?

- means thickened, hardened skin
- spectrum: may be clinical feature of limited anatomic extent affecting only the skin and adjacent tissues or it may be assoc w/ systemic involvement. When the characteristic skin disorder is assoc w/ internal organ involvement the disease is termed Systemic sclerosis:
- disease w/ vascular, fibrotic, and immunologic manifestations and involves several systems including skin, GI tract, lungs, heart and kidneys


Epidemiology of systemic sclerosis?

- annual incidence of 1-2/100,000 individuals in US
- peak onset b/t 30-50


PP of systemic sclerosis?

combo of:
- vascular damage: endothelians cause vasoconstriction and are fibrinogenic, sera from pts w/ SSc has been found to be cytotoxic to endothelial cells
- immune activation: activation of endothelial cells results in up regulation of adhesion molecules, and leukocyte migration out of vasculature
- excessive synthesis of extracellular matrix w/ deposition of structurally normal collagen results


Dx systemic sclerosis?

- dx of systemic sclerosis and related disorders is based primarily upon presence of characteristic clinical findings
- combo of skin induration plus one or more of the following clinical features supports the dx:
- heartburn and/or dysphagia of new onset
- acute onset of HTN and renal insufficiency
- dyspnea on exertion assoc w/ interstitial pulm changes on radiography
- pulm HTN
- diarrhea w/ malabsorption
- characteristic facial, lip, or hand telangiectasia
- digital infarctions and or digitial tip pitting


Since there is no tx that comprehensively addresses SSc - what specific organ manifestations need to be tx?

- cutaneous manifestations
- raynauds phenomenon
- MSK manifestations
- GI
- pulm
- cardiac
- renal
-ED and dyspaurenia


Skin involvement of SSc?

characterized by variable thickening and hardening of the skin:
fingers, hands, and face are generally the earliest areas of body involved. Edematous swelling and erythema of skin may precede skin induration
- other prominent skin manifestations include:
digital ulcers
pitting at fingertips


Tx for cutaneous manifestations of SSC?

no tx proven to reverese skin thickening
- emollient creams should be used liberally to soften skin and decrease pruritus
- antihistamines and doxepin - pruritus
- UVA therapy
- other options: highly potent topical steroids and methotrexate
- combo of high-dose systemic glucocorticoids and low dose methotrexate has also been used


What is Raynauds phenomenon? Common in what diseases? diff b/t primary and secondary?

- MC initial manifestation of scleroderma (generally precedes other manifestations, sometimes by yrs or even decades)
- also assoc w/ SLE
- an exaggerated vascular response to cold temp or emotional stress
- manifested clinically by sharply demarcated color changes of skin of the digits due to abnormal vasoconstriction of digital arteries and cutaneous arterioles
- RP is considered primary if these sxs occur alone w/o evidence of any assoc disorder
- secondary RP: presence of disorder in assoc w/ related illness, such as scleroderma


Presentation of RP?

- sudden onset of cold fingers (or toes) in assoc w/ sharply demarcated color changes of skin pallor (white attack) and/por cyanotic skin (blue attack)
- w/ rewarming, the ischemic phase (white or blue attack) usually lasts for 15-20 min. the skin subsequently blushes upon recovery, thereby resulting in erythema or reperfusion


Benign course vs secondary course of RP?

- primary RP: commonly seen in general pop - occurs in otherwise healthy individuals, and almost always has a benign course
- in pts w/ SSc, RP - can lead to
ulcerations, digital infarctions, gangrene


Tx of RP?

- prevention: bundling up in cold temps
- CCBs (nifedipine, amlodipine)
- alpha blockers may be given along w/ or instead of CCBs
- low dosed ASA
- ED pills (Viagra)
- tx any assoc complications: debridement of ulcers, abx for secondary infections
- smoking cessation
- avoid BBs, caffeine, decongestants


What are the MSK manifestations of SSc?

- erosive, inflammatory arthritis similar to RA can occur
- generally tx w/ drugs such as methotrexate or tylenol or low dose steroids


What are GI manifestations of SSc? Tx?

- GI tract involved in all smooth musclce areas
- most frequent clinical manifestation is GERD
- tx:
preventative measures
promotility agent: erythromycin (avoid reglan)


Pulm manifestations of SSc? tx?

- lung fibrosis occurs commonly
- CT scan may show ground glass pattern
- tx: cyclophosphamide
- pts may also suffer from pulm HTN (get annual echos)
- increased risk of lung cancer (5x)


Cardiac manifestations of SSc? Tx?

- pts can have conduction abnormalities or dysrhythmias
- tx is w/ appropriate antiarrhythmics and cardiac pacing if needed


Renal manifestations of SSC? Tx, what should be avoided?

- pts may have an acute renal crisis seen early in the disease (typically has been assoc w/ use of moderate to high dose steroid therapy)
- b/f ACEI, SSc renal crisis caused renal failure in all pts and death in many
- tx:
avoid diuretics!!


What is CREST syndrome?

- calcinosis
- raynaud's phenomenon
- esophageal dysmotility
- sclerodactyly
- telangiectasia


PP of CREST syndrome?

- immune system produces anti-centromere and anti-nuclear abs
- often used to refer to limited cutaneous form of scleroderma
- ots often go on to develop pulm HTN and CHF


What is polymyositis/dermatomyositis? What rashes are characteristic?

- inflammatory muscle disease that can occur w/ or w/o a cutaneous eruption
- inflammatory damage of myocytes leads to proximal muscle wekaness
- 3 rashes are characteristic of dermatomyositis:
- rash over PIP and MCP (gottron's sign)
- purple (heliotrope) eruption over the eyelids
- erythematous poikiloderma (pigmentation, telangiectasia, purpura, pruritus, and atrophy) over upper anterior chest and around over the shoulders (shawl sign)


MC presenting feature of Polymyositsi/dermatomyositis?

proximal muscle weakness:
- onset is usually insidious, w/ gradual worsening over a period of several months b/f medical attention is sought
- distribution of weakness is typically symmetric and proximal
- myalgias and muscle tenderness occur in 25-50% of cases. These sxs tend to be mild, unlkie the more prominent muscle pain that occurs in polymyalgia rheumatica and fibromyalgia
- muscle atrophy is generally not seen in early cases, even in pts w/ marked weakness, but may occur in severe, long standing disease


Dx polymyositis/dermatomyositis?

- suggested clinically (esp w/ cutaneous manifestations)
- make sure to acquire excellent med hx (common meds that cause myalgias: statins and gemifibrozil)
- muscle enzyme eval:
CK, LDH, AST, ALT: almost all pts will have elevation of at least one and many will have elevations of all
- ANA elevated in up to 80% (specific autoabs)
- do muscle bx


Pathogenesis of DM?

- humorally mediated (more complement driven, B cells)
- cellular infiltrate focused around blood vessels
- abnormal muscle fibers grouped in one portion of the fascicle suggesting microinfarction


Pathogenesis of PM?

- muscle fibers - CD8 T cells
- cellular infiltrate w/in the fascicle
- abnormal muscle cells scattered throughout the fascicle


Tx of PM and DM?

- cutaneous manifestations:
mild to mod topical steroids
may reqr hydroxychloroquine or methotrexate
- muscular manifestations:
initial tx w/ high dose systemic steroids (usually tapering will begin in a month), PT program, sometimes other immunosupressant meds started including methotrexate (need to take folic acid, avoid preg) and azathioprine