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SBL (Skin, Blood, Lymph) Exam 1 and 2 > Lecture 22: Connective Tissue Disorders and Vasculitides > Flashcards

Lecture 22: Connective Tissue Disorders and Vasculitides Flashcards

1
Q

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

A
  • multisystem inflammatory autoimmune disorder of autoAbs to nuclear antigens (Type 3 hypersensitivity)
    • forms immune complexes
  • African Americans/Hispanics > Caucasians
  • F > M
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2
Q

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

A

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

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3
Q

What are 4 findings of Systemic Lupus Erythematosus serology?

A
  • 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)
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4
Q

What kind of endocarditis is associated with SLE?

A

LIBMAN-SACKS ENDOCARDITIS

  • caused by immune deposits collecting on heart valves
  • NOT INFECTIOUS
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5
Q

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

A
  • avoid sun exposure (use sunscreen)
  • NSAIDS
  • glucocorticoids (topical or systemic)
  • hydroxychloroquine
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6
Q

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

A

Early: infections and kidney/CNS disease

Late: accelerated atherosclerosis and thromboembolic events

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7
Q

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

A

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

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8
Q

What is the retinopathy associated with SLE and Antiphospholipid Syndrome?

A

Cotton Wool Spots

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9
Q

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

What happens when suspected pts. take sulfa antibiotics?

A
  • promotes demethylation of DNA with NO RENAL/CNS symptoms
  • positive for ANA and ANTI-HISTONE Abs (95%)
  • sulfa antibiotics cause SLE FLARE
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10
Q

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

What is a serious complication of Neonatal Lupus?

A
  • 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

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11
Q

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

A
  • 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

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12
Q

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

A
  • 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

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13
Q

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

A

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
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14
Q

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

A
  • associated with Limited Cutaneous Systemic Scleroderma
C - cutaneous calcinosis
R - Raynauds (longstanding)
E - Esophageal dysmotility (GERD)
S - sclerodactaly
T - telangiectasia
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15
Q

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?

A
  • 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

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16
Q

What are two Renal manifestations of Scleroderma?

A
  1. Chronic Kidney Disease
  2. Renal Crisis
    • malignant HTN, hemolytic anemia, prog. renal insuff.
    • high dose of glucocorticoids can induce crisis
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17
Q

What are 3 common GI manifestations of Scleroderma?

A
  1. Barrett esophagus - inc. risk of esophageal AdenoC
  2. GAVE Syndrome = Watermelon Stomach
    • gastric antral vascular ectasia
  3. Primary biliary cirrhosis/cholangitis
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18
Q

What is a common MSK and Thyroid manifestation of Scleroderma?

A

MSK = Carpal Tunnel Syndrome

Thyroid = Hypothyroid (thyroid fibrosis)

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19
Q

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?

A

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
Q

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

A
  • 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
Q

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

A

Schirmer test

22
Q

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

A

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

  • do a LIP BIOSPY to see lymphoid foci in accessory salivary glands (ESSENTIAL FOR DIAGNOSIS)
23
Q

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

A
  • treatment is symptomatic, should have regular check-ups with dentist and ophthalmologist
  • avoid ATROPINIC drugs and DECONGESTANTS (will further dry out the patient)
24
Q

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

A
  • 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
Q

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?

A
  • 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
Q

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

A
  • Gottron’s patches/papules (overlying knuckles)
  • Heliotrope rash (purple, periorbital edema)
  • Periungal erythema
  • V-neck erythema (Poikiloderma or “Shawl Sign”)
27
Q

Polymyositis (PM)

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

A
  • 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
Q

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?

A
  • 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
Q

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

A

Henoch-Schonlein Purpura, Goodpasture Syndrome, Granulomatosis with Polyangiitis

30
Q

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

A

Eosinophilic Granulomatosis with Polyangiitis and Behcet Syndrome

31
Q

What are 3 Systemic Vasculitides of MEDIUM vessels? (TO, PN, KD)

A

Thromboangiitis Obliterans (Buerger Disease), Polyarteritis Nodosa, and Kawasaki Disease

32
Q

What are 2 Systemic Vasculitides of LARGE vessels? (TA, GCA)

A

Takayasu Arteritis and Giant Cell Arteritis

33
Q

Henoch-Schonlein Purpura (IgA Vasculitis)

What is it and who does it affect, what is its tetrad (PP/A/AP/RD), and how is it treated?

A
  • common in small vessels of KIDS

T: palpable purpura (no thrombocytopenia), arthritis, abdominal pain, and renal disease (IgA deposits)

T: supportive and glucocorticoids

34
Q

Goodpasture Syndrome (Anti-Glomerular Basement Membrane)

What is it, what does it affect, and how is it treated?

A
  • small vessel deposits of anti-basement membrane autoantibodies (commonly glomerular and pulmonary capillaries)
  • pulmonary hemorrhage can cause death if not treated

T: plasmapheresis, glucocorticoids

35
Q

Granulomatosis w/Polyangiitis (Wegener’s Granulomatosis)

What is it and who does it affect, what are 3 common hallmarks (G, NV, G) and what physical finding does it commonly present with?

What is a major serological finding of GPA?

A
  • affects small vessels of MALES > 40 yo

H: Granulomatous inflammation, Necrotizing vasculitis, and glomerulnephritis

PF: Saddle Nose w/crusting and bleeding due to Respiratory Tract involvement (90% nasal)

Serology: ANCA (+)

36
Q

Eosinophilic Granulomatosis w/Polyangiitis (Churg-Strauss Syndrome)

What is it, what does it look like, and what is a major serological finding of EGPA?

A
  • affects small/medium vessels of RESPIRATORY Tract

H: asthma and eosinophilia with granuloma vasculitis

  • high eosinophils in circulating blood
  • palpable purpura and systemic necrosis

Serology: ANCA (+)

37
Q

Behcet Syndrome

What are two complications it can cause, what is its common Triad for SMALL vessels, and what is a major serological finding of it?

A
  • multisystem involvement that can cause Large Vessel aneurysms and DVT

Triad: recurrent mouth ulcers, genital ulcers, and uveitis (eye inflammation)

Serology: HLA-B51 (+)

use low dose glucocorticoids to treat

38
Q

Thomboangiitis Obliterans (Buerger Disease)

What is it and who does it affect, how does it progress, and what is its treatment?

A
  • segmental MEDIUM vessel disease in young MALES < 35 yo
  • occurs in SMOKERS; treat by STOPPING SMOKING
  • thrombosis of distal –> proximal vessels than can cause loss of digits and has a CORKSCREW appearance on angiography
39
Q

Polyarteritis Nodosa

What does it affect and what is it associated with, what does it cause in the Periphery/Renal/Lung, and what does a biopsy reveal?

A
  • affects segmental MEDIUM vessels of MALES and is associated with HBV –> check for serological HBV markers
  • Vaculitis Neuropathy (“foot drop”), Renin-mediated HTN, but SPARES the LUNGS
  • biopsy shows fibrinoid necrosis (inflammatory cells) WITHOUT granulomas
40
Q

Kawasaki Disease

What is it and who does it affect, what is a common physical manifestation, and what causes morbidity?

A
  • affects MEDIUM vessels of MALES < 5 yo (mainly Japan)
  • common physical finding is STRAWBERRY TONGUE
    • also fever, LAD, rash
  • morbidity from CORONARY involvement (either aneurysm or myocardial infarction that can occur years later)
41
Q

Takayasu Arteritis

What is it and who does it affect, what does it look like on biopsy, and what does it look like on angiography?

A
  • affects LARGE vessels (AORTA) in females < 40 yo (adolescent girls and young women in Asia)
  • called “pulseless disease” because it obliterates the peripheral pulses (but collaterals prevent limb loss)

Biopsy: granuloma with some GIANT CELLS

Angiography: long smooth tapered stenosis (athersclerosis = focal irregular lesions)

42
Q

What is a common eye manifestation of Takayasu Arteritis and what is it caused by?

A
  • causes Copper Wiring Retinopathy due to renal artery stenosis = HYPERTENSION
43
Q

Giant Cell Arteritis (Temporal Arteritis)

What is it and who does it affect, what are common clinical symptoms (JC/PR), and what is seen on biopsy?

A
  • affects LARGE vessels (cranial arteries and aorta) in women > 50 yo
  • headache, JAW CLAUDICATION, Polymyalgia Rheumatica, visual abnormalities (start glucocorticoids to prevent blindness!!)
  • ESR greatly increased, HLA-DR4 serology

Biopsy: temporal artery (GOLD STANDARD) with granulomatous vasculitis and MULTINUCLEATED Giant Cells

44
Q

What is Polymyalgia Rheumatica (PMR) and what two findings are elevated?

A
  • associated with GCA and is a stiffness, soreness, or muscle pain with FEELINGS of weakness as a result of pain (no TRUE objective weakness)
  • everything will be normal save for elevated ESR and CRP (no muscle inflammation, no muscle enzyme inflammation)
45
Q

What is the difference between Primary and Secondary Raynauds Phenomenon?

A

Primary: benign, symmetric, exaggerated response to cold
- females 15-30 yo

Secondary: occurs BECAUSE of something else

  • unilateral; mainly > 30 yo
  • MORE SEVERE –> ischemia

SBL (Skin, Blood, Lymph) Exam 1 and 2 (22 decks)

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