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1 STEP UW sclerodermos > Autoimmune + vasculitides (sleroderma sjogren) > Flashcards

Autoimmune + vasculitides (sleroderma sjogren) Flashcards

1
Q

The central disturbance in SLE is ……………………..

A

excessive autoantibody production due to loss of self-tolerance

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2
Q

What antibodies are the most characteristic feature of SLE?

A

anti-nuclear antibodies - ANA

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3
Q

Why there is complement consumption in SLE?

A

Autoantibodies bind to self antigens –> leads to formation of circulating immune complexes –> deposit in various organs + activates complement

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4
Q

What hematologic abnormalities occur in SLE?

A

pancytopenia + anemia due to chronic inflammation

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5
Q

Pancytopenia in SLE. What hypersensitivity?

A

autoantibodies against blood cell antigens = typer II hypersensitivity

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6
Q

Anemia in SLE. Mechanisms?

A
  1. Anemia - due to chronic inflammation;
  2. Autoimmune hemolytic anemia - due to antibodies agains RBCs antigens –> spherocytosis, positive direct Coombs, extravascular hemolysis
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7
Q

Thrombocytopenia in SLE. Mechanism?

A

Antiplatelet antibodies –> thrombocytopenia resembling ITP

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8
Q

Leukopenia in SLE. Mechanims?

A

Antibody mediated destruction of neutrophils

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9
Q

Where occurs effect that cause hematologic abnormalities in SLE?

A

chronic inflammatory effects on bone marrow + autoimmune hemolysis

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10
Q

What causes lupus nephritis in SLE. What hypersensitivity?

A

Immune complex deposition in the mesangium, subendothelial and/or subepithelial spaces. type III hypersensitivity

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11
Q

What is the most common pattern in lupus nephritis in SLE?

A

diffuse proliferative GN

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12
Q

What are 4 test changes are in lupus nephritis?

A

incr. creatinine, proteinuria, hematuria or RBC’s on urinalysis

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13
Q

What 3 antibodies in SLE?

A

ANA, anti-dsDNA, anti-Smith

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14
Q

SLE ANA. Specificity and sensitivity?

A

Low spec, but high sensitivity

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15
Q

SLE anti-dsDNA. Specificity and sensitivity?

A

high spec, low sensitivity (60proc)

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16
Q

SLE anti-Smith. Specificity and sensitivity?

A

high spec, low sensitivity (20-30proc)

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17
Q

Against what structures are anti-Smith antibodies?

A

antibodies against small nuclear ribonuclear ribonucleoproteins

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18
Q

What changes are in mouth in SLE?

A

ulcers on hard palate

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19
Q

SLE is assoc. with increased risk of what disorders? Why?

A

Arterial and venous thrombosis - due to antiphospholipid antibodies production

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20
Q

what antibodiies cause drug-induced lupus?

A

antihistone antibody

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21
Q

What medications cause drug-induced lupus?

A

procainamide, hydralazine, isoniazid, D-penicillamine

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22
Q

What are the symptoms in drug-induced lupus?

A

Symptoms resemble SLE, but no renal or CNS involvement. ANA positiver by definition.

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23
Q

What hypersensitivity is henoch schonlein purpura?

A

Type III - IgA complexes deposits triggers complement activation and inflammation of small vessels throughout the body.

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24
Q

HSP follows what infection?

A

upper resp. tract infection

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25
Q

4 manifestations of HSP?

A

Palpable purpura/petechiae on the lower exctremities
Arthritis/arthralgias
abdominal pain/GI bleeding
Renal injury

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26
Q

where in the vessels occurs inflammation in HSP?

A

Within papillary dermis –> nonthrombocytic palpable purpura on dependent areas (buttocks, lower extremities)

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27
Q

immunocomplexes in HSP consists of …….(2)

A

IgA and C3

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28
Q

What are pathophysiological changes in HSP in vessels?

A

Leukocytoclastic inflammation = small superficial vessels are damaged by perivascular neutrophils inflammation –> results in fibrin depositions in the wall (fibrinoid necrosis) + RBCs extravasation

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29
Q

What is leukocytoclasis?

A

neutrophil breakdown?

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30
Q

How is called neutrophils breakdown?

A

Leukocytoclasis

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31
Q

Leukocytoclasis leads to the formation of ……………..

A

Perivascular nuclear debris

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32
Q

Treatment of HSP in children?

A

benign and self-limiting

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33
Q

What risk increases HSP in adults?

A

renal complications –> nephrotic syndrome + acute kidney injury

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34
Q

Patho DIC vs HSP?

A

DIC - intravascular fibrin deposition without vessel inflammation
HSP - vessel inflammation + fibrin thrombi MAY BE seen

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35
Q

Churg Strauss vs HSP manifestation?

A

Churg - renal, skin and joint involvement BUT INITIALLY PRESENTS WITH ASTHMA SYMPTOMS - which are not seen in HSP

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36
Q

What mechanism cause colicky abdominal pain in HSP?

A

local vasculitis

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37
Q

What joints are affected in HSP?

A

hips, knees, ankles

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38
Q

location of IgA complex depositions in kidney?

A

Mesangium

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39
Q

What neurological manifestation ins SLE?

A

psychosis (cognitive dysfunction), seizures

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40
Q

2 sjogren complications in eyes and mouth?

A 
corneal ulcerations (due to diminished lacrimal gland function);
dental caries (due to diminished salivary gland function)
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41
Q

Dry eyes in sjogren are called …………….

A

keratoconjuctivitis sicca

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42
Q

dry mouth is called ……….

A

xerostomia

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43
Q

Why may occur arthralgias and cutaneous vasculitis in sjogren?

A

due to deposition of circulating complexes

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44
Q

Arthritis in SLE?

A

symmetric, migratory

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45
Q

In what areas are affected glands in sjogren?

A

salivary, lacrimal;

vaginal gland involvement is also common.

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46
Q

How in 2 ways can manifest sjogren?

A

independently or as another autoimmune syndrome (eg RA)

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47
Q

What are 2 specific antibodies in sjogren? What 2 are nonspecific?

A

specific - anti-Ro/SSA and anti La/SSB.

Often seen RF and ANA

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48
Q

biopsy of sjogren in salivary glands?

A

periductal lymphocytic infiltrates + germinal centers (proliferatin lymphocytes)

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49
Q

How looks glandular tissue in sjogren biopsy?

A

atrophic and fibrotic

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50
Q

what muscle weakness is in polymyositis?

A

symetrical proximal (upper and lower extremities). Painless or diffuse myalgias

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51
Q

how differs dermatomyositis and polymyositis?

A

Similar, but dermatomyositis assoc with significant skin findings (heliotrope rash and Gottron papules)
Polymositis - no skin findings

52
Q

2 ways how can manifest polymyositis and dermatomyositis’?

A

independently or as a paraneoplastic manifestation of an underlying malignancy (esp. adenocarcinoma)

53
Q

What muscle enzymes can be elevated in poly/dermatomyositis?

A

creatine kinase and aldolase

54
Q

What antibodies in poly/dermatomyositis?

A

anti-Jo-1 (anti-histidyl-tRNA synthetase) - less sensitive and more specific. ANA - in most cases, so it is sensitive but less specific

55
Q

how to differentiate poly and dermatomyositis?

A

biopsy

56
Q

Polymyositis patho?

A

endomysial inflammation without prominent vascular involvement in a scattered or pathchy distribution

57
Q

dermatomyositis patho?

A

perivascular inflammaition in a segment pattern without vasculopathy

58
Q

what is endomysial inflammation?

A

direct invasion of individual muscle fibers

59
Q

What is perifascicular inflammation?

A

inflammation localized around blood vessels and the septa between muscle fascicles

60
Q

What triggers polymyositis?

A

inflammatory myopathy triggered by unknown, possibly viral, antigens

61
Q

What is the immune response in polymyositis? Against what?

A

cell mediated against myocytes

62
Q

what immunology alterations probably lead to polymyositis?

A

increased expression of MHC I antigens on sarcolema leads to autoantigens to CD8 cytotoxic cells that subsequently initiate myocyte destruction

63
Q

what happens to muscle fibers in polymyositis’?

A

regeneration and fibrosis of muscle fibers

64
Q

What types of cells infiltrate endomysium in polymyositis’??

A

macrophages and CD8 lymphocytes

65
Q

what happens to joints and muscle in systemic sclerosis?

A

joint - arthralgias and contractures;

muscle weakness is not seen

66
Q

What biopsy changes seen in myastenia gravis and eaton lambert?

A

none, speciment is normal

67
Q

how often occurs muscle weakness in myasthenia gravis and eaton-lambert?

A

myastenia - episodic;

eaton lambert - fatigable weakness

68
Q

How manifest polymyalgia rheumatica?

A

shoulder and pelvic gridle muscles pain + systemic symptoms. Muscle weakness is not seen

69
Q

what is heliotrope rash?

A

erythematous or violaceous edematous eruption on the periorbital area/upper eyelids and cheeks

70
Q

What is gottron papules?

A

raised erythematous plaques/red or violaceous, flat-tropped papules over joints and bony prominences, esp. on the hands.

71
Q

What portion is affected in perifascicular inflammation?

A

contiguous portion of the fascicle and surrounding vessels (ie occurring along the periphery of the fascicles)

72
Q

For what disease need to evaluate patients with poly/dermatomyositis?

A

malignancy (esp. adenocarcinoma in ovary, lung, pancreas)

73
Q

what is innitial treatment of dermatomyositis?

A

systemic glucocorticoids (prednisone)

74
Q

Hypothyroid myopathy commonly presents with proximal muscle weakness. How differs from derma/polymyositis?

A

Prominent muscle pain and often have other hypothyroid features (eg, weight gain, cold intolerance).

75
Q

What skin layer is affected in systemic sclerosis?

A

dermal layer

76
Q

Why there is dermal layer expansion in systemic sclerosis?

A

due to diffuse deposition of collagen

77
Q

What happens to adipose tissue and dermal appendages in systemic sclerosis?

A

atrophy

78
Q

There is atrophy of what 2 structures in systemic sclerosis in dermal layer?

A

atrophy of intradermal adipose tissue and dermal appendages

79
Q

What is considered ad dermal appendages?

A

hair follicles, glands

80
Q

What system is seriously affected in systemic sclerosis apart from skin?

A

lungs –> interstitial lung disease and pulmonary hypertension

81
Q

interstitial lung disease manifests in ………… systemic sclerosis

A

diffuse cutaneous SS

82
Q

pulmonary hypertension manifests in ………… systemic sclerosis

A

limited cutaneous SS. often assoc with CREST syndrome

83
Q

Atrophy of skin structures in systemic sclerosis leads to …………

A

thinning of the epidermis

84
Q

What is renal injury in systemic sclerosis?

A

SS is assoc with scleroderma renal crisis

85
Q

Why occurs scleroderma renal crisis?

A

due to vascular injury, not glomerulonephritis

86
Q

Sclerodermal renal crisis manifest as …………….

A

hypertensive emergency with end-organ damage.

Casts are not seen because it is not GN

87
Q

Diffuse scleroderma is assoc with antibodies?

A

anti DNA topoisomerase I (Scl-70)

88
Q

Antibodies in CREST?

A

anti-centromere

89
Q

CREST acronym’?

A

Calcinosis, raynaud, esophageal dismotility, sclerodactyly, teleangiectasias

90
Q

(CREST) Esophageal dysmotility is the result of …………….
It manifest as ………

A

Fibrosis of the distal esophagus with associated esophageal hypomotility. It manifests with symptoms of gastroesophageal reflux (heartburn and regurgitation).

91
Q

What is sclerodactyly?

A

thickening of the skin in the hands and feet

92
Q

What is initial presentation of sclerodactyly?

A

it begins as non-pitting edema of the hands and fingers

93
Q

What is later manifestation of sclerodactyly?

A

Skin becomes thickened, tight and shinny. Thinning of the skin (atrophy) follows.

94
Q

What is teleangiactasiias?

A

dilated blood vessels on the skin of face, hands, upper trunk, mucosa

95
Q

Pathogenesis of systemic sclerosis ultimately results in …………….. (2)

A

excess fibrosis and vasculopathy

96
Q

What 3 types of cells are involved in systemic sclerosis?

A

Vascular endothelial cells, T lymphocytes and fibroblasts

97
Q

Vascular endothelial cells function in systemic sclerosis?

A

produce excessive endothelin 1 –> vasoconstriction and activation of fibroblasts + increase expression of adhesion molecules (facilitates binding of lymphocytes)

98
Q

T lymphocytes function in systemic sclerosis?

A

migrate into the tissues and increase expression of numerous cytokines that activate tissue fibroblasts

99
Q

Dermal fibroblasts function in systemic sclerosis?

A

Produce increasing quantities of collage in ground substance –> leads to thickening and stiffening of the tissues.

100
Q

Early symptoms of sclerosis?

A

finger swelling and mild pruritus

101
Q

With progression of the systemic sclerosis, patients may experience ……………… (4)

A

Dermal sclerosis (sclerodactyly), dermal ulcers, nail atrophy, and digital calcinosis

102
Q

Antibodies against presynaptic Ca channels –> results in ………… (2)

A

decreased scetylchiline release and muscle weakness

103
Q

Lambert Eaton is strongly assoc with …………. Why?

A

Small cell lung cancer.

Likely due to the immune recognition of voltage gated Ca channels that are present on the malignant cells.

104
Q

What symtoms occur in lambert eaton due to autonomic dysfunction?

A

Autonomic symptoms such dry mouth or impotence

105
Q

Why there is postexercise facilitation of muscle weakness in lambert eaton?

A

Muscle strength and deep tendon reflexes improve with exercise or repetitive movements (due to accumulation of calcium within the axon terminal

106
Q

If cranial nerve and resp. muscle manifestaion occur in lambert eaton, then when?

A

If occur, then in later stage of the diseases.

In contrast - MG facial, periocular and bulbar weakness occur before extremity weaknes.

107
Q

MG is caused by ………..

A

impaired functioning of nicotinic cholinergic receptors at the neuromuscular junction due to interference by IgG autoantibodies.

108
Q

What class of antibodies occur in MG pathophysoilogy?

A

IgG and/or IgM autoantibodies

109
Q

How to confirm diagnosis of MG?

A

adminiter anticholinesterase agents –> increased amount of ACh at the NMJ

110
Q

Why ice packs can improve symptoms caused by weakness of superficial muscles (eg. ptosis)?

A

Acetylcholinesterase activity decreases at lower temperatures

111
Q

What type of hypersensitivity is MG?

A

type II - IgG and/or IgM autoantibodies that bind cell surface antigens and/or extracellular matrix production.

112
Q

What organ pathology is related to MG?

A

Thymus: thymoma or thymic hyperplasia

113
Q

Thymus is derived from …………… as are ……………

A

third pharyngeal pouch, as are inferior parathyroid glands.

114
Q

What 2 structures are derived from 3rd pharyngeal pouch?

A

thymus and inferior parathyroid glands

115
Q

1st pharyngeal pouch gives rise to …………….

A

epithelium of middle ear and auditory tube

116
Q

2nd pharyngeal pouch gives rise to …………….

A

epithelium of palatine tonsil crypts

117
Q

epithelium of middle ear and auditory tube are derived from ……… pouch

A

1st pharyngeal pouch

118
Q

epithelium of palatine tonsil crypts derived from ……… pouch

A

2nd pharyngeal pouch

119
Q

The superior parathyroid glands and ultimobranchial body are derived from ……………. pouch

A

4th pharyngeal pouch

120
Q

4th pharyngeal pouch gives rise to ……………

A

The superior parathyroid glands and ultimobranchial body

121
Q

Tympanic membrane is derived from ……….

A

pharyngeal membrane (1st? wtf ten buvo zymejimas)

122
Q

Epithelium of external canal is derived from …………

A

pharyngeal groove (1st? wtf ten buvo zymejimas)

123
Q

Pharyngeal groove gives rise to ……………

A

epithelium of external ear canal

124
Q

Pharyngeal membrane gives rise to …………

A

tympanic membrane

125
Q

The floor of the foregut gives rise to ……………

A

Thyroid diverticulum

126
Q

The thyroid diverticulum migrates caudally to the ………….

A

neck

127
Q

The thyroid diverticulum migrates caudally to the neck, but remains attached ……………………

A

To the floor of the mouth by the thyroglossal duct

1 STEP UW sclerodermos (2 decks)

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