Lecture 22: Connective Tissue Disorders and Vasculitides Flashcards Preview

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Flashcards in Lecture 22: Connective Tissue Disorders and Vasculitides Deck (45)
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1

What is Systemic Lupus Erythematosus and who does it commonly affect?

- multisystem inflammatory autoimmune disorder of autoAbs to nuclear antigens (Type 3 hypersensitivity)
- forms immune complexes

- African Americans/Hispanics > Caucasians

- F > M

2

What are Cutaneous, Arthritis, Hematologic, Cardiopulmonary, and Nephritis features of Systemic Lupus Erythematosus?

Cut: Malar "Butterfly" rash and photosensitivity
- over cheeks/nasal bridge (spares nasolabial folds)

A: inflammatory, symmetric, non-erosive

H: thrombosis

Cardio: PERICARDITIS
- constant, substernal "crushing" or sharp chest pain
- inc. risk of MI due to accelerated atherosclerosis

N: classification via histologic

3

What are 4 findings of Systemic Lupus Erythematosus serology?

- ANA (+)

- anti-DS DNA and Smith antibodies
- DS DNA correlates with disease activity

- decreased levels of C3/C4 = inc. consumption (returns towards normal during remission)

4

What kind of endocarditis is associated with SLE?

LIBMAN-SACKS ENDOCARDITIS

- caused by immune deposits collecting on heart valves

- NOT INFECTIOUS

5

What are 4 common treatments for SLE? (A, N, G, H)

- avoid sun exposure (use sunscreen)

- NSAIDS

- glucocorticoids (topical or systemic)

- hydroxychloroquine

6

What are two causes of Early Year SLE Mortality vs Later Year SLE Mortality?

Early: infections and kidney/CNS disease

Late: accelerated atherosclerosis and thromboembolic events

7

What are the 3 types of Antiphospholipid Antibodies? When should they be measured?

Type 1: causes false-positive syphilis test
- test for Treponemal Abs if syphilis is suspected

Type 2: Lupus anticoagulant
- inc. venous/arterial thrombosis and miscarriage
- inc. aPTT time, abnormal DRVVT

Type 3: Anti-cardiolipin Abs to Beta2GPI

**measure on two occasions 12 weeks apart and treat APS via anticoagulants INDEFINITELY**

8

What is the retinopathy associated with SLE and Antiphospholipid Syndrome?

Cotton Wool Spots

9

What is Lupus-like Syndrome (Drug-induced Lupus) and what two tests are it positive for?

What happens when suspected pts. take sulfa antibiotics?

- promotes demethylation of DNA with NO RENAL/CNS symptoms

- positive for ANA and ANTI-HISTONE Abs (95%)

- sulfa antibiotics cause SLE FLARE

10

What are the two antibodies found in mothers who give birth to children with Neonatal Lupus?

What is a serious complication of Neonatal Lupus?

- mothers will have the Anti Ro (SSA) or La (SSB) Abs

- Permanent complete heart block is a major complication that can occur in utero, at birth, or in the neonatal period

11

What is Discoid Lupus Erythematosus (DLE) and how can it be treated? (2)

- occurs independent or as a manifestation of SLE most commonly on the HEAD

- well-defined inflammatory plaques that can become atrophic, disfiguring scars

T: photoprotection and topical anti-inflammatory agents or systemic antimalarial drugs

12

What is Scleroderma, how does it differ between Caucasians and African Americans, and what is its treatment?

- thickening/hardening of skin with fibrosis of skin/visceral organs and obliteration of eccrine/sebaceous glands causing dry itchy skin

- Caucasians: secondary Raynauds FIRST
- African Amer: pigmentation change THEN Raynauds

T: no approved therapy; focus on control/slowing of symptoms

13

What is the difference between Localized, Limited, and Diffuse Scleroderma?

Localized: children with morphea (patches)
- discreet discoloration of skin
- no Raynauds and NOT systemic

Limited: fingers, toes, face, distal extremities
- CREST SYNDROME; indolent and slow progress
- Raynauds and Pulmonary Artery Hypertension
- digital ischemia

Diffuse: systemic (proximal extremities and trunk)
- early organ involvement (WORST PROGNOSIS)
- interstitial lung disease and renal crisis

14

What is CREST Syndrome and what is it commonly associated with?

- associated with Limited Cutaneous Systemic Scleroderma

C - cutaneous calcinosis
R - Raynauds (longstanding)
E - Esophageal dysmotility (GERD)
S - sclerodactaly
T - telangiectasia

15

What is the primary cause of Morbidity and Mortality in patients with Scleroderma?

What are the two manifestations seen in Diffuse and Limited Scleroderma and how are they diagnosed?

- most common cause is PULMONARY involvement

Diffuse = Interstitial Lung Disease
- fine velcro crackles (rales), chronic dry cough
- dx with pulmonary function test (PFT)/Lung CT

Limited = Pulmonary Artery Hypertension
- exertional dyspnea, angina, right heart failure
- dx with 2D echocardiogram or right heart cath.

**inc. risk of bronchoalveolar carcinoma**

16

What are two Renal manifestations of Scleroderma?

1. Chronic Kidney Disease

2. Renal Crisis
- malignant HTN, hemolytic anemia, prog. renal insuff.
- high dose of glucocorticoids can induce crisis

17

What are 3 common GI manifestations of Scleroderma?

1. Barrett esophagus - inc. risk of esophageal AdenoC

2. GAVE Syndrome = Watermelon Stomach
- gastric antral vascular ectasia

3. Primary biliary cirrhosis/cholangitis

18

What is a common MSK and Thyroid manifestation of Scleroderma?

MSK = Carpal Tunnel Syndrome

Thyroid = Hypothyroid (thyroid fibrosis)

19

What is the difference in serology between Limited and Diffuse Scleroderma?

What should you pay particular attention to when taking the history/physical of a pt. believed to have Scleroderma?

Limited: positive for anti-CENTROMERE Abs

Diffuse: positive for anti-SCL70 and anti-RNA POLY III
- anti-SCL70 = anti-DNA TOPOISOMERASE I

- should pay special attention to BLOOD PRESSURE since it is a heralding feature of renal disease

20

Who is Sjogren's Syndrome normally seen in, what symptoms does it present with, and what cancer it is strongly associated with?

- seen in Postemenopausal females (Mid 50's)

- causes SICCA symptoms (destruction due to inflammatory processes)
- dry eyes, mouth (inc. candida and dental caries)
- keratoconjunctivitis sicca (foreign body sensation)

- strongly associated with MALT Lymphoma

21

What test is used to measure quality of the tears secreted by patients with Sjogren's Syndrome?

Schirmer test

22

What are 4 serological findings of Sjogren's Syndrome and what biopsy should be performed and why?

ANA (+), Rhematoid Factor (+), Anti-Ro/La (+), polyclonal hypergammaglobulinemia

- do a LIP BIOSPY to see lymphoid foci in accessory salivary glands (ESSENTIAL FOR DIAGNOSIS)

23

What is the treatment for Sjogren's Syndrome and what drugs should be avoided by the patient?

- treatment is symptomatic, should have regular check-ups with dentist and ophthalmologist

- avoid ATROPINIC drugs and DECONGESTANTS (will further dry out the patient)

24

What weaknesses are characteristic of Inflammatory Myopathies and what enzymes will be elevated? (2)

- occurs more in females and African Americans

- see Systemic Bilateral Proximal Muscle weakness (difficulty rising from a chair or bathtub)

- muscle enzymes will be elevated (creatine kinase and aldolase) while ESR/CRP are often normal

25

Dermatomyositis (DM)

What is it and who is it seen in, what biopsy and serology are characteristic of it, and what is it at an increased risk of developing?

- muscle weakness (proximal --> distal) WITHOUT sensory symptoms seen in 7-15 yo and 30-60 yo (binodal distribution)

- perifascicular atrophy (biopsy) and Anti Jo-1 antibodies (serology)

- increased risk of OCCULT MALIGNANCY

26

What are 4 characteristic skin lesions of Dermatomyositis? (GP, HR, PE, V)

- Gottron's patches/papules (overlying knuckles)

- Heliotrope rash (purple, periorbital edema)

- Periungal erythema

- V-neck erythema (Poikiloderma or "Shawl Sign")

27

Polymyositis (PM)

What is it and who is it seen in, what biopsy/serology/labs are characteristic of it, and how is it treated?

- subacute proximal muscle weakness in 30-50 yo with NO SKIN CHANGES (vs. DM)

- endomysial inflammation (biospy), Anti Jo-1 (serology), and elevated CK (labs)

- treat with Glucocorticoids

28

Inclusion Body Myositis (IBM)

What is it and who is it seen in, what biopsy/serology/labs are characteristic of it, and how is it treated?

- finger/quadriceps weakness in white MALES 40-50 yo

- endomysial inflammation/rimmed vacuoles (biospy), mild or normal CK (labs), anti-cN1A autoabs (serology)

- treatment is SUPPORTIVE

29

What are 3 Systemic Vasculitides of SMALL vessels? (HSP, GS, GPA)

Henoch-Schonlein Purpura, Goodpasture Syndrome, Granulomatosis with Polyangiitis

30

What are 2 Systemic Vasculitides that vary in vessels? (EGPA, BS)

Eosinophilic Granulomatosis with Polyangiitis and Behcet Syndrome