Rheumatology - SLE, Sjogren Syndrome, Scleroderma Flashcards by Elisabeth Silver

what organ system can SLE manifest itself in?

any organ system

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what is the MOST COMMON cause of inpatient mortality with SLE?

infection and organ failure (kidney failure)

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SLE pts have a high risk for premature ___

artherosclerosis

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SLE may present as new dx in pt with what?

pericarditis, glomerulonephritis or neurologic disease

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most common presentation of new SLE?

glomerulonephritis

hematologic d/o (anemia, neutropenia, thrombocytopenia, thrombosis)

Arthritis and rash (Mallor rash)

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presentation of pt with established SLE?

flares

neuropsychiatric lupus (seizures, depression, psychosis)

premature CV disease

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what is the HALLMARK of SLE pathophysiology? SLE likely from?

auto antibodies nuclear proteins

likely from defective apoptosis

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what complement proteins are low in SLE?

C4 and C3

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at least 50% of pts with SLE have increased levels of ___

B-lymphocyte stimulator (BlyS) = growth factor antibodies

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to make SLE dx what do you need?

clinical and immunologic criteria

-pt must fit clinical picture PLUS lab abnormalities

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what antibodies are specific to SLE? what are their levels like in SLE?

anti-dsDNA or anti-Sm

their levels will be HIGH

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what is Lupus Nephritis?

Proteinuria >0.5g/d or 3+

Active urinary sediment
- >5RBC/HFP OR >5 WBC/HFP

if suspected, then bx

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neuropsychiatric disease of SLE dx how?

Lumbar puncture and CSF analysis (to exclude CNS infection), EEG, neuropsychiatric testing, and neuroimaging

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what is the cornerstone of SLE tx? and for acute lupus flares requiring hospitalization/

Glucocorticoids

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what is maintenance therapy for SLE?

Hydroxychloroquine

-reduces frequency of SLE flares and improves survival

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what do you use to control heart block in SLE?

immunoglobulins (IVIG)

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what med is newly approved for SLE?

Belimumab

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what is the mechanism of Belimumab?

Binds to and inhibits soluble human B lymphocyte stimulator (BlyS)

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what SLE pts get Belimumab as tx?

patients with SLE who have higher disease activity, are taking corticosteroids, and have elevated anti-DNA antibodies and low complement levels

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what is sjogren syndrome?

slowly progressive autoimmune rheumatic disorder in which the exocrine glands are the primary target tissue

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what are the main 2 manifestations of sjogren syndrome?

intense dryness of eye (xerophthalmia) and mouth (xerostomia)

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what can pts with sjogren syndrome develop?

malignant lymphoma

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most common pt affected with sjogren syndrome?

woman in 4th or 5th decade of life

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autoantibodies to what antigens are detected at time of dx of Sjogren syndrome?

Autoantibodies to Ro/SS-A and La/SS-B antigens

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what contributes to the xerostomia sx of sjogren syndrome?

impaired saliva production

b/c of impaired saliva production, ppl with sjogren syndrome are susceptible to the development of what?

new-onset and severe dental caries at the gum line in mid-adult life

primary sjogre's syndrome is diagnosed if?

- The patient presents with eye and/or mouth dryness - Eye tests disclose keratoconjunctivitis sicca - Mouth evaluation reveals the classic manifestations of the syndrome and/or - The patient’s serum reacts with Ro/SS-A and/or La/SS-B autoantigens

what glands are enlarged in sjogrens syndrome?

the parotid glands

tx for sjogrens syndrome is aimed at?

sx relief

tx for dry in sjogrens syndrome?

Replace deficient tears with hydroxypropyl methycellulose; polyvinyl alcohol; 0.5% methylcellulose; Hypo Tears

if corneal ulcers are present in sjogrens syndrome, what's the tx?

eye patching and boric acid ointments

what meds AVOIDED should be in sjogrens syndrome?

drugs that may decrease lacrimal and salivary secretions, like diuretics, HTN meds, anticholinergics, and antidepressants

what is the best tx for the xerostomia in sjogrens syndrome?

water replacement propionic acid gels may used to treat vaginal dryness

what med for sjogrens syndrome stimulate secretions?

pilocarpine (PO) or cevimeline

what med is for arthralgia and mile arthritis in sjogrens syndrome?

Hydroxychloroquine

what meds treat renal tubular acidosis in sjogrens syndrome?

sodium bicarb PO

what meds treat systemic vasculitis in sjogrens syndrome?

glucocorticoids and/or immunosuppressive agents (e.g. cyclophosphamide)

when is monoclonal antibody to CD20 used in sjogrens syndrome?

pts with systemic disease, particularly in those with vasculitis, arthritis, and fatigability

tx for sjogrens syndrome and lymphoma?

combo of anti-CD-20 with CHOP regimen

what is scleroderma?

systemic chronic sclerosis

what is scleroderma characterized by?

diffuse fibrosis of the skin and internal organ with significant disability, disfigurement and mortality

what are the 2 types of scleroderma disease?

limited disease (80% of patients) diffuse disease (20%)

sx's of limited scleroderma disease?

Thickening of skin confined to face, neck, and distal extremities (sausage-like fingers)

sx's of diffuse scleroderma disease?

Widespread thickening of skin, including truncal involvement, with areas of increased pigmentation and depigmentation

what is present in ALL pts with scleroderma?

Raynaud phenomenon and antinuclear antibodies

what are the systemic features of scleroderma?

- Gastroesophageal reflux - Gastrointestinal hypomotility - Pulmonary fibrosis - Pulmonary hypertension - Renal involvement

what are the causes of scleroderma?

- Unknown - Autoimmunity, endothelial cell damage and increased production extracellular matrix appear to play key pathogenic roles

what decades do sx's appear in for scleroderma? what gender is most affected?

3rd-5th decades women more affected

what is scleroderma also known as?

CREST syndrome (calcinosis cutis, Raynaud phenomenon esophageal motility disorder, sclerodactyly, and telangiectasia), the hardening of the skin (scleroderma) is limited to the face and hands

what are the GI problems in scleroderma d/t?

GAVE - develop angiodysplasia in the stomach that look like the skin of the watermelon (watermelon stomach) -makes blood vessels thin and bleed spontaneously

what is usually the initial sx of scleroderma?

Raynaud's phenomenon

what sx's of scleroderma are early features of diffuse scleroderma but infrequent in limited scleroderma?

Polyarthralgia, weight loss, and malaise

how long is the interval b/w diffuse scleroderma and Raynaud's phenomenon and onset of other disease?

weeks to months

how is long is the interval b/w limited scleroderma and Raynaud's phenomenon and onset of other disease?

years

what are signs of the early inflammatory "edematous" phase in scleroderma? what parts are affected first? what else is common in this phase?

Soft tissue swelling, puffy fingers, and intense pruritus The fingers, distal limbs, and face are usually affected first Diffuse hyperpigmentation of the skin, carpal tunnel syndrome arthralgias, muscle weakness, fatigue, and decreased joint mobility are common

the initial 4 years from scleroderma adisease onset is when the most damage to what organs? what happens if organ failure doesn't occur?

pulmonary and renal damage If organ failure doesn’t occur during this phase of dcSSc, the systemic process may stabilize

what is the scleroderma antibody? what is it directed against?

anti-SCL-70 | -directed against topoisomerase 3

what antibodies are highly specific for limited scleroderma?

Anticentromere antibodies

what antibodies in scleroderma are directed against RNA polymerases? what about antibodies against B23?

anti-RNAP I, II, III and anti-B23

what are the anti-RNAP antibodies in scleroderma associated with?

diffuse skin changes, cardiac and renal involvement, and increased mortality renal HTN crisis

what are the anti-B23 antibodies in scleroderma associated with?

pulmonary HTN

what is the main tx of scleroderma?

CCBs, eat slow, avoid sugars, don’t eat while lying down

how do you treat Raynaud's syndrome in scleroderma?

CCBs (e.g., long-acting nifedipine or losartan, or sildenafil)

how should pts with scleroderma and esophageal disease take their medications?

liquid or crushed form

how can esophageal reflux be reduced?

don't have late-night meals and by the use of PPIs

what PO prokinetic agents can pts with scleroderma and delayed gastric emptying take? what do they improve?

Erythromycin, Metoclopramide, or Cisapride | -improve dysphagia caused by esophageal hypomotility

HTN crises associated with scleroderma renal crisis need to be treated when and where and with what?

early and aggressively (in the hospital) with ACEIs | -captopril q6 hrs fxn tests modestly in pts with severe interstitial lung disease

what meds improve dyspnea and pulmonary fxn tests in pts with severe interstitial lung disease?

cyclophosphamide | -MUST BE ADMIN BY PHYSICIAN FAMILIAL WITH ITS USE B/C HIGHLY TOXIC DRUG

what meds treats skin disease, arthritis, and myositis in scleroderma?

Methotrexate (MTX)