Rheumatology - SLE, Sjogren Syndrome, Scleroderma Flashcards
what organ system can SLE manifest itself in?
any organ system
what is the MOST COMMON cause of inpatient mortality with SLE?
infection and organ failure (kidney failure)
SLE pts have a high risk for premature ___
artherosclerosis
SLE may present as new dx in pt with what?
pericarditis, glomerulonephritis or neurologic disease
most common presentation of new SLE?
glomerulonephritis
hematologic d/o (anemia, neutropenia, thrombocytopenia, thrombosis)
Arthritis and rash (Mallor rash)
presentation of pt with established SLE?
flares
neuropsychiatric lupus (seizures, depression, psychosis)
premature CV disease
what is the HALLMARK of SLE pathophysiology? SLE likely from?
auto antibodies nuclear proteins
likely from defective apoptosis
what complement proteins are low in SLE?
C4 and C3
at least 50% of pts with SLE have increased levels of ___
B-lymphocyte stimulator (BlyS) = growth factor antibodies
to make SLE dx what do you need?
clinical and immunologic criteria
-pt must fit clinical picture PLUS lab abnormalities
what antibodies are specific to SLE? what are their levels like in SLE?
anti-dsDNA or anti-Sm
their levels will be HIGH
what is Lupus Nephritis?
Proteinuria >0.5g/d or 3+
Active urinary sediment
- >5RBC/HFP OR >5 WBC/HFP
if suspected, then bx
neuropsychiatric disease of SLE dx how?
Lumbar puncture and CSF analysis (to exclude CNS infection), EEG, neuropsychiatric testing, and neuroimaging
what is the cornerstone of SLE tx? and for acute lupus flares requiring hospitalization/
Glucocorticoids
what is maintenance therapy for SLE?
Hydroxychloroquine
-reduces frequency of SLE flares and improves survival
what do you use to control heart block in SLE?
immunoglobulins (IVIG)
what med is newly approved for SLE?
Belimumab
what is the mechanism of Belimumab?
Binds to and inhibits soluble human B lymphocyte stimulator (BlyS)
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
what is sjogren syndrome?
slowly progressive autoimmune rheumatic disorder in which the exocrine glands are the primary target tissue
what are the main 2 manifestations of sjogren syndrome?
intense dryness of eye (xerophthalmia) and mouth (xerostomia)
what can pts with sjogren syndrome develop?
malignant lymphoma
most common pt affected with sjogren syndrome?
woman in 4th or 5th decade of life
autoantibodies to what antigens are detected at time of dx of Sjogren syndrome?
Autoantibodies to Ro/SS-A and La/SS-B antigens
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)
