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Medicine > Rheuma > Flashcards

Rheuma Flashcards

1
Q

Autoimmune disease where organs undergo damage from autoantibodies

A

SLE

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2
Q

SLE autoantibody

A

Antinuclear antibody ANA

NOT LIMITED to SLE also with CT and autoimmune disease

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3
Q

99% of SLE patients

A

Women of childbearing years 1:700

All are susceptible

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4
Q

Most autoimmune diseases are

With ratio

Age range

Race

A

Female

9:1

20-30yrs

Severe in Blacks

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5
Q

Pathogenesis for SLE

A 
Genes (HLA-DR3) Environment
Abnormal immune response
Autoantibodies immune complex
Inflammation
Damage
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6
Q

Fundamental defect in SLE

A

Failure to maintain self tolerance

Body no longer recognizes self from non-self

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7
Q

Factors that predispose SLE formation

A 
HLA D2,3,8
UV Light
Infection (EBV)
Medications (Procainamide, Isoniazid, Sulfonamide, PTU, Hydralazine, Methyldopa)
Smoking
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8
Q

Clinica manifestations of SLE

A 
Skin 
Oral ulcer
Non-scarring Alopecia
Synovitis (at least 2) 
Serositis (pleurisy, pericarditis)
Renal Protein/Crea <0.5
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9
Q

Criteria for Dx SLE

A

More than or equal to 4 criteria

1 clinical, 1 immunologic

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10
Q

Dx criteria in SLE

>4 criteria at given time

A 
Malar
Discoid
Photosensitivity
Painless oral ulcer
Symmetric arthritis
Serositis
Renal, neuro, hematologic, immunologic
Antinuclear antibody
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11
Q

Clinical manifestation of SLE and Prevalance

A

Systemic 95% (fatigue, malaise, fever)
Musculoskeletal (arthralgia) 95%
Hematologic (Anemia) 85%
Cutaneous (photosensitivity) 80%

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12
Q

Most common cutaneous manifestion of SLE

A 
Photosensitivity 70%
Malar 50%
Oral ulcer 40%
Alopecia 40%
Discoid 20%
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13
Q

Most common hematologic manifestation of SLE

A

*Anemia 70%
Leukopenia 65%
Lympho 50%
*Thrombocytopenia 15%

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14
Q

Most common neurologic disorder in SLE

A

Cognitive decline 50%

Mood disorder 40%

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15
Q

Patients with SLE are predisposed to stroke because of

A

Hypercoagulable state

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16
Q

Musculo manifestations of SLE

A

Intermittent polyarthritis most common
Rhupus
Ischemic necrosis of bone
Myalgia

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17
Q

Slightly raised erythema

Scaly on the face, cheeks and nose

A

Photosensitive rash

Butterfly/Malar rash

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18
Q

Acute SLE Rash

A

Photosensitive
Slightly raised erythema
Occasionally scaly

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19
Q

Scaly, red patches

Circular red-rimmed flat lesions

A

Subcutaneous Cutaneous Lupus Erythematosus

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20
Q

Rough, circular, slightly raised, scaly, hyperpigmented rims
Depigmented atrophic center
Violaceous, hyperpigmented, atrophic plaque with follicular plugging and scarring

A

Discoid Lupus Erythematosus

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21
Q

Most serious manifestation of SLE

Leading cause of mortality in first decade of disease

A

Nephritis

Half - nephrotic (hypoalbuminemia, edema, hyperlipidemia)
Most - hypertensive

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22
Q

Class I Lupus Nephritis

A

Minimal mesangial

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23
Q

Class IV Lupus nephritis

A

Diffuse Lupus Nephritis

poor prognosis

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24
Q

Wire loop appearance in microscope

A

Diffuse Lupus Nephritis

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25
Q

Most frequent renal stage of lupus nephritis

A

Class IV Diffuse Lupus Nephritis

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26
Q

CNS Lupus manifestation

A 
Cognitive dysfunction 
most common manifestation difficulty with memory and reasoning
Seizure
Psychosis
Lupus Cerebritis
Stroke (thrombitis)
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27
Q

Most common pulmonary manifestation of SLE with or without pleura effusion

A

Pleuritis

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28
Q

Most frequent cardiac manifestation of SLE

Responds to anti-inflammatory therapy and infrequently leads to tamponade

A

Myocarditis

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29
Q

Most serious cardiac manifestations of SLE (2)

A

Myocarditis

Fibrinous endocarditis of Libmana-Sacks

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30
Q

Most common cause of myocarditis

A

Cocksackie B

beating heart

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31
Q

Hand foot and mouth disease

A

Coxsackie A

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32
Q

Coxsackie B also causes

A

Bornholm Disease

Pleurodynia

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33
Q

Presence of antibody to phospholipid
Acceleration to atherosclerosis
Hypercoagulability and acute thrombotic events

A

Vascular occlusion

TIA, stroke and MI

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34
Q

APA accelerates atherosclerosis hence hypercoagulability

A

thrombotic event

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35
Q

Anemia in SLE

A

Normochromic, normocytic or
Hemolytic

EPO decreased from kidney failure

Leukopenia
Thrombocytopenia

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36
Q

GI SLE manifestation autoimmune causing abdominal pain

A

Intestinal vasculitis

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37
Q

During SLE flares GI manifestations:

A 
n&amp;v diarrhea
vasculitis
perforation
ischemia
bleeding
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38
Q

Ocular SLE manifestation

A

Sicca syndrome
Nonspecific conjunctivitis

rarely threaten vision

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39
Q

Serious ocular manifestation of SLE

A 
retinal vasculitis
optic neuritis (also in MS)
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40
Q

Musculoskeletal SLE

A

Intermittent polyarthritis

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41
Q

Cardiac SLE

A

Pericarditis

Myocarditis

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42
Q

Autoantibodies in SLE

A

Antinuclear antibody (ANA) 98%
Antihistone (Drug induced SLE) 70%
Antiphospholipid (APAS) 50%

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43
Q

Best autoantibody screening test in SLE

A

ANA 98%

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44
Q

High specificity autoantibody test
Associated with renal disease
Poor prognosis

A

Anti-dSDNA

Anti-Smith

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45
Q

3 widely accepted tests that measure different antibodies in SLE

A

anticardiolipin (beef heart) Calcutta antigen
anti2-glycoprotein
lupus anticoagulant

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46
Q

Non-specific Ab but presence fulfills 1 classification criteria of SLE

A

APAS

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47
Q

Neonatal lupus ab
Sicca syndrome Sjogrens
SLE

A

Anti-Ro/SS-A

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48
Q 
Positive ANA
fever
malaise
arthralgia
serositis
rash
Drugs
A

Drug induced SLE

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49
Q

Drug inducing SLE

A 
Procainamide
Hydralazine
Isoniazid
Methyldopa
Simvastatin
Lithium
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50
Q

Dx and Tx of Lupus

A 
Symptoms suggestive 
Other labs: ANA, CBC
ANA +
Definitive SLE criteria >/=4 or possible <4 Tx
All tests normal
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51
Q

Treatment goals

A

Induce remission of acute flare

Maintain tx to supress symptom and prevent organ damage

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52
Q

Life organ threatening SLE TX

A

High dose glucocorticoids
Mycophenolate mofetil myfortic acid
Cyclophosphamide (do not exceed 6 mos)

Maintain with mycophenolate and azathioprine

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53
Q

Conservative SLE tx

A

Analgesic

Antimalarial (reduce dermatitis, arthritis and fatigue)

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54
Q

Hydroxychloroquine SE (2)

A

Retinal tox

Prolonged QT interval

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55
Q

SLE tx corticosteroid

A

1g Methylprednisone sodium succinate per day at least 3 days -5
Pulse therapy

Maintain on prednisone after
0.5-1g/kg/dayc

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56
Q

Chronic
Slowly progressive autoimmune disease affecting exocrine glands

Middle aged women 9:1

A

Sjogren’s

Prevalance for primary sjogren’s: 0.5-1%

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57
Q

Most common exocrine gland affected in Sjogren’s

A

Salivary gland

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58
Q

Sjogren’s pathogenesis

A

Lymphocytic infiltration

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59
Q

Clinical manifestation of Sjogren’s

A

Oral: xerostomia, diff swallowing, atrophy of filliform papillae, parotid gland enlargement

Ocular: sandy, gritty feeling under eye, dec tearing, erythema, itching, dry eyes!

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60
Q

Also causes xeropthalmia

A

Vit A deficiency

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61
Q

Sicca syndrome

A

Dry eyes
Dry mouth
Keratoconjunctivitis sicca

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62
Q

most common Extraglandular manifestation of Sjogren’s

A

Arthritis/arthralgia 60
Reynaud’s 37%
Fatigability/myalgia 25%
Lymphoma 6% MALT

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63
Q

Differential diagnosis for Sicca

A

HIV

Young male
Lacks autoantibodies Ro/SS-A and or B
Lymphocytic infiltration of CD8 lymphocytes

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64
Q

No age preference
Lack autoantibodies to Ro
Granuloma in salivary gland
Negative HIV

A

Sarcoidosis

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65
Q

Middle aged wimeb
Ro/SS A +
Lymphoid infiltrates of CD4+
HLADR3

A

Sjogren’s

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66
Q

Dx of Sjogren’s

Strongly supported by

A

Salivary gland biopsy

+ anti-Ro SS-A
+ anti-La SS-B

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67
Q

Cardiolipin

A

SLE

APAS

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68
Q

Carbonic anhydrase II

A

Sjogren’s

Systemic sclerosis

69
Q

Centromere assoc proteins

A

Systemic sclerosis

70
Q

Sjogren’s tx

A

Corticosteroid and immunosuppresive

71
Q

Sjogren’s is also assoc with

A 
RA
SLE
Scleroderma
Primary biliary cirrhosis
Autoimmune thyroid
Chronic active hepatitis
Mixed CTD
72
Q

Autoantibody associated with primary biliary cholangitis

A

Antimitochondrial antibody

73
Q

Most common lymphoma in Sjogren’s

A

MALT lymphoma

74
Q

Hepatobiliary involvement in sjogren

A

Primary biliary cirrhosis

75
Q

Tx of Sjogren’s

A

Azathioprine

Mycophenolate mofetil

76
Q

To lubricate dry mouth

A

Water

77
Q

To lubricate eyes

A

Artifical tears

78
Q

Arthritis in Sjogren’s

A

Hydroxychloroquine

Methotrexate

79
Q

Reynaud’s in Sjogren’s

A

Nifedipine

80
Q

Renal Acidosis in Sjogren’s

A

Bicarbonate

81
Q

Lymphoma in Sjogren’s

A

CHOP and anti CD20

Cyclophosphamide
Doxorubicin Adriamycin
Vincristine
Prednisone

82
Q

Dilated cardiomyopathy

Cardiac toxicity

A

Doxorubicin

83
Q

Hemorrhagic cystitis

A

Cyclophosphamide

84
Q

Vincristine

A

Oncovin

85
Q

R in CHOP monoclonal antibody

A

rituximab

86
Q

Methotrexate inhibits the enzyme

A

Dihydrofolate reductase

87
Q

RA affects

A

0.5-1%

F 2-3:1

88
Q

RA Gene

A

HLA DRB1
0401 high risk

Europe and East Asian

89
Q

Alleles known to confer greatest risk of RA within

A

Major Histocompatibility Complex MHC

90
Q

Function of MHC

A

Bind peptide fragments from pathogens and display for cell surface recognition

91
Q

Most common disease susceptibility alleles in Asians (Jap, Koreans, Chinese) in RA

A

0405

0901

92
Q

Most risk of RA is associated with allelic variation

A

HLA DRB1 gene

93
Q

RA environmental risk factors for RA

A 
1 Smoking RR 1.5-3.5 related to RF and Anti CCP Ab (+) disease
2 EBV
3 Mycoplasma
4 Parvo B19
5 Silicone dust and mineral oil exposure
94
Q

Infectious mononucleosis pathognomonic of

A

EBV Downey Type II cell

95
Q

RA Pathology hallmark

A

1 synovial inflammation and proliferation
2 focal bone erosion
3 thinning of articular cartilage

96
Q

RA has two cell types

A

1 Type A Synoviocyte from mac

2 Type B Synoviocyte from fibroblast

97
Q

Most abundant RA cell type that produce structural components of joints such as collagen, fibronectinr and laminin

A

Type B Synoviocyte

98
Q

Bone

Cartilage

BM

Collagen Types

A

I

II fibrocartilage, symphisis pubis

IV

99
Q

25-55 y, F

Symmetrical, polyarthritis >5 joints

A

RA

100
Q

Earliest joint involved in RA

A

Small joint of hands and feet

101
Q

Early morning stiffness in RA

A

> 1 hr that eases with physical activity

102
Q

RA symptom duration

A

> 6 weeks

103
Q

Once disease is established, Most frequently involved joints in RA (3)

A

1 wrist
2 MCP
3 PIP

104
Q

RA spares

A

1 TLS

2 DIP of fingers and IP of toes

105
Q

Hallmark of RA leading to dec ROM, reduced grip strength and trigger finger

A

Flexor tendon synovitis

106
Q

Boutinniere Deformity

A

PIP flexion
DIP hyperExtension
MCP hyperExtension

107
Q

Swan Neck Deformity

A

PIP hyperExtension

DIP flexion

108
Q

MCP in RA become chronically

A

ulnarly deviated

109
Q

Heberden nodes are nodes on

A

DIP

110
Q

Bouchard nodes are nodes on

A

PIP

111
Q

Z line deformity is

A

subluxation of 1st MCP with hyperExtension of 1st IP joint

112
Q

Hammer toe

A

Flexion of PIP of toe

MTP Extension

113
Q

Piano key movement

A

Inflammation of ulnar styloid
Tenosynovitis of ECU
Subluxation of distal ulna at the styloid

114
Q

Boutinniere Deformity

A

PIP flexion
DIP hyperExtension
MCP hyperExtension

115
Q

Swan Neck Deformity

A

PIP hyperExtension

DIP flexion

116
Q

MCP in RA become chronically

A

ulnarly deviated

117
Q

Heberden nodes are nodes on

A

DIP

118
Q

Bouchard nodes are nodes on

A

PIP

119
Q

Z line deformity is

A

subluxation of 1st MCP with hyperExtension of 1st IP joint

120
Q

Hammer toe

A

Flexion of PIP of toe

MTP Extension

121
Q

Claw toes

A

Flexion of both PIP and DIP

Sparing the big toe

122
Q

Constitutional signs and symptoms of RA

A 
Most severe cases have cachexia TNF alpha 
Depression 
Malaise
Fatigue
Fever
Weight loss
123
Q

Cachectin

A

TNF a

124
Q

Constitutional signs and sx in RA reflect

A

High degree of inflammation

Precede onset of joint symptoms

125
Q

Fever in RA raises suspicion of

A

Systemic vasculitis
Infection

> 38.3 101F

126
Q

Extraarticular manifestions in RA develop in

A

40% of patients

May occur prior to onset

127
Q

Extraarticular manifestions of RA

A

Cervical myelopathy (neuro)
Keratoconjunctivitis sicca Sjogren’s, Scleritis
Anemia, Felty syndrome
Xerostomia
Pleural effusion, nodules
Pericarditis, MI, cardiomyopathy, mitral regurg
Membranous nephropathy, secondary amyloidosis
Hypoandrogenism
Pyoderma gangrenosum
Osteoporosis

128
Q

Piano key movement

A

Inflammation of ulnar styloid
Tenosynovitis of ECU
Subluxation of distal ulna at the styloid

129
Q

IBS assoc with pyoderma gangrenosum

A

Ulcerative colitis

130
Q

30-40%
Firm nontender adherent to periosteum tendon, bursa
Develop in areas subject to repeated trauma (forearm, sacral, achilles)
Lungs, pleura, pericardium and peritoneum

A

Rhematoid nodule

131
Q

Clinicial features of RA Pulmonary

A

Pleuritis most common
ILD
Pulmonary nodule

132
Q

Pleuritis present with

A

Pleuritic chest pain and dyspnea

Pleural friction rub and exudative effusion

133
Q

Rare subset of pulmonary nodulosis characterized by development of nodules and pneumoconiosis following silica exposure

A

Caplan’s syndrome

134
Q

Caplan’s syndrome

A

Pulmonary nodule
pneumoconiosis
silica

135
Q

Caplan’s is associated with

A

RA

136
Q

Most frequently involved site of extraarticular affectation of RA

A

Pericardium

137
Q

Cardiomyopathy in RA

A

Necrotizing and granulomatous myocarditis
CAD
Diastolic dysfunction

138
Q

Most common valvular abnormality in RA

A

Mitral regurgitation

139
Q

Caplan’s syndrome

A

Pulmonary nodule
pneumoconiosis
silica

140
Q

Rare 1%
Most common in long standing disease
Rheumatoid factor
Hypocomplementenemia

A

Vasculitis

141
Q

Cutaneous signs of RA

A 
Gangrene digital infarct
Petechiae
Purpura
Livedo reticularis
Ulcer
142
Q

Amantadine and minocycline may cause

A

Livedo reticularis

Also used in influenza
pd

143
Q

Most common hematologic feature of RA

A

Anemia
Normo, normo
Degree parallels inflamm
Thrombocytosis

144
Q

Parasite assoc with B12 deficiency

A

D latum

145
Q

Felty Syndrome

A 
1%
Neutropenia
Splenomegaly
Nodular RA
Thrombocytopenia
146
Q

Most common lymphoma in RA

2-4% inc risk

A

Diffuse large B cell Lymphoma

147
Q

Most common form of non hodgkin lymphoma in adults
Assoc with RA
Also in HIV

A

Diffuse Large B Cell Lymphoma

148
Q

Most common cause of death in RA

A

Cardiovascular disease

Inc incidence of CAD and carotid atherosclerosis

CHF 2 fold higher

149
Q

Bone problem in RA

A

Osteoporosis in 20-30% of px

From chronic glucocorticoid use
Disability related immobilitg

150
Q

Associated condition in RA

A

hypoandrogenism

151
Q

Criteria for RA

A

Joint involvement
Serology
Acute phase reactant (CRP)
Duration of symptoms (6wk)

More than 6 fulfills requirement

152
Q

Changes in 2010 ACR/EULAR classification for RA

A

Inclusion of Anti cyclic citrullinated protein antibody

No longer takes into account presence of rheumatoid nodule or radiographic joint damage

153
Q

RA Lab results

A

75-80% with RF

also seen in Sjogrens, SLE

154
Q

Anti CCP
Specificity
Prognosis

A

95%

poor prognosis

155
Q

Synovial fluid in RA predominantly has

A

neutrophil

5000-50000

156
Q

Earliest radio finding in RA

A

Juxtaarticular osteopenia
Soft tissue swelling, symmetric joint space loss, subchondral erosion
Joint sublux and collapse

157
Q

Imaging with greatest sensitivity for RA

A

MRI

detects synovitis, joint effusion and early bone marrow changes

158
Q

RA tx

A

NSAIDs

Glucocorticoid

159
Q

Glucocorticoid in RA

A

Low to moderate dose

5-10mg/day

160
Q

Risk for osteoporosis in glucocorticoids

A

> 5mg/day for more than 3 months

161
Q

DMARD of choice for RA

A

Methotrexate 10-25mg/week

162
Q

Hydroxychloroquine
Dose
SE

A

DMARD
200-400mg/day
Retinal toxicity, cardiotoxicity

163
Q

Other DMARD tx

A

Leflunomide 10-20mg/day
Sulfasalazine 500mg also in IBS
se: Hemolytic anemia and SJS

164
Q

Biologic agents in RA

AE

A

Anti TNF a
Infliximab
Adalimumab

AE: opportunistic funga infection and TB activation

165
Q

RA Prognosis

A

10% spont remission within 6 mos

MC cause of death
IHD

166
Q

RA Prognosis

A

10% spont remission within 6 mos

MC cause of death
IHD

167
Q

Most common cause of MONOarticular arthritis

A

TB

168
Q

Lupus flare

A

Fatigue
Arthritis
Mucocutaneous lesion

169
Q

Autoantibody specific tor lupus like sx in INH use

A

Anti histone

Medicine (29 decks)

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