Rheuma Flashcards
1
Q
Autoimmune disease where organs undergo damage from autoantibodies
A
SLE
2
Q
SLE autoantibody
A
Antinuclear antibody ANA
NOT LIMITED to SLE also with CT and autoimmune disease
3
Q
99% of SLE patients
A
Women of childbearing years 1:700
All are susceptible
4
Q
Most autoimmune diseases are
With ratio
Age range
Race
A
Female
9:1
20-30yrs
Severe in Blacks
5
Q
Pathogenesis for SLE
A
Genes (HLA-DR3) Environment Abnormal immune response Autoantibodies immune complex Inflammation Damage
6
Q
Fundamental defect in SLE
A
Failure to maintain self tolerance
Body no longer recognizes self from non-self
7
Q
Factors that predispose SLE formation
A
HLA D2,3,8 UV Light Infection (EBV) Medications (Procainamide, Isoniazid, Sulfonamide, PTU, Hydralazine, Methyldopa) Smoking
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
9
Q
Criteria for Dx SLE
A
More than or equal to 4 criteria
1 clinical, 1 immunologic
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
11
Q
Clinical manifestation of SLE and Prevalance
A
Systemic 95% (fatigue, malaise, fever)
Musculoskeletal (arthralgia) 95%
Hematologic (Anemia) 85%
Cutaneous (photosensitivity) 80%
12
Q
Most common cutaneous manifestion of SLE
A
Photosensitivity 70% Malar 50% Oral ulcer 40% Alopecia 40% Discoid 20%
13
Q
Most common hematologic manifestation of SLE
A
*Anemia 70%
Leukopenia 65%
Lympho 50%
*Thrombocytopenia 15%
14
Q
Most common neurologic disorder in SLE
A
Cognitive decline 50%
Mood disorder 40%
15
Q
Patients with SLE are predisposed to stroke because of
A
Hypercoagulable state
16
Q
Musculo manifestations of SLE
A
Intermittent polyarthritis most common
Rhupus
Ischemic necrosis of bone
Myalgia
17
Q
Slightly raised erythema
Scaly on the face, cheeks and nose
A
Photosensitive rash
Butterfly/Malar rash
18
Q
Acute SLE Rash
A
Photosensitive
Slightly raised erythema
Occasionally scaly
19
Q
Scaly, red patches
Circular red-rimmed flat lesions
A
Subcutaneous Cutaneous Lupus Erythematosus
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
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
22
Q
Class I Lupus Nephritis
A
Minimal mesangial
23
Q
Class IV Lupus nephritis
A
Diffuse Lupus Nephritis
poor prognosis
24
Q
Wire loop appearance in microscope
A
Diffuse Lupus Nephritis
25
Most frequent renal stage of lupus nephritis
Class IV Diffuse Lupus Nephritis
26
CNS Lupus manifestation
```
Cognitive dysfunction
most common manifestation difficulty with memory and reasoning
Seizure
Psychosis
Lupus Cerebritis
Stroke (thrombitis)
```
27
Most common pulmonary manifestation of SLE with or without pleura effusion
Pleuritis
28
Most frequent cardiac manifestation of SLE
Responds to anti-inflammatory therapy and infrequently leads to tamponade
Myocarditis
29
Most serious cardiac manifestations of SLE (2)
Myocarditis
| Fibrinous endocarditis of Libmana-Sacks
30
Most common cause of myocarditis
Cocksackie B
| beating heart
31
Hand foot and mouth disease
Coxsackie A
32
Coxsackie B also causes
Bornholm Disease
| Pleurodynia
33
Presence of antibody to phospholipid
Acceleration to atherosclerosis
Hypercoagulability and acute thrombotic events
Vascular occlusion
| TIA, stroke and MI
34
APA accelerates atherosclerosis hence hypercoagulability
thrombotic event
35
Anemia in SLE
Normochromic, normocytic or
Hemolytic
EPO decreased from kidney failure
Leukopenia
Thrombocytopenia
36
GI SLE manifestation autoimmune causing abdominal pain
Intestinal vasculitis
37
During SLE flares GI manifestations:
```
n&v diarrhea
vasculitis
perforation
ischemia
bleeding
```
38
Ocular SLE manifestation
Sicca syndrome
Nonspecific conjunctivitis
rarely threaten vision
39
Serious ocular manifestation of SLE
```
retinal vasculitis
optic neuritis (also in MS)
```
40
Musculoskeletal SLE
Intermittent polyarthritis
41
Cardiac SLE
Pericarditis
| Myocarditis
42
Autoantibodies in SLE
Antinuclear antibody (ANA) 98%
Antihistone (Drug induced SLE) 70%
Antiphospholipid (APAS) 50%
43
Best autoantibody screening test in SLE
ANA 98%
44
High specificity autoantibody test
Associated with renal disease
Poor prognosis
Anti-dSDNA
| Anti-Smith
45
3 widely accepted tests that measure different antibodies in SLE
anticardiolipin (beef heart) Calcutta antigen
anti2-glycoprotein
lupus anticoagulant
46
Non-specific Ab but presence fulfills 1 classification criteria of SLE
APAS
47
Neonatal lupus ab
Sicca syndrome Sjogrens
SLE
Anti-Ro/SS-A
48
```
Positive ANA
fever
malaise
arthralgia
serositis
rash
Drugs
```
Drug induced SLE
49
Drug inducing SLE
```
Procainamide
Hydralazine
Isoniazid
Methyldopa
Simvastatin
Lithium
```
50
Dx and Tx of Lupus
```
Symptoms suggestive
Other labs: ANA, CBC
ANA +
Definitive SLE criteria >/=4 or possible <4 Tx
All tests normal
```
51
Treatment goals
Induce remission of acute flare
| Maintain tx to supress symptom and prevent organ damage
52
Life organ threatening SLE TX
High dose glucocorticoids
Mycophenolate mofetil myfortic acid
Cyclophosphamide (do not exceed 6 mos)
Maintain with mycophenolate and azathioprine
53
Conservative SLE tx
Analgesic
| Antimalarial (reduce dermatitis, arthritis and fatigue)
54
Hydroxychloroquine SE (2)
Retinal tox
| Prolonged QT interval
55
SLE tx corticosteroid
1g Methylprednisone sodium succinate per day at least 3 days -5
Pulse therapy
Maintain on prednisone after
0.5-1g/kg/dayc
56
Chronic
Slowly progressive autoimmune disease affecting exocrine glands
Middle aged women 9:1
Sjogren’s
Prevalance for primary sjogren’s: 0.5-1%
57
Most common exocrine gland affected in Sjogren’s
Salivary gland
58
Sjogren’s pathogenesis
Lymphocytic infiltration
59
Clinical manifestation of Sjogren’s
Oral: xerostomia, diff swallowing, atrophy of filliform papillae, parotid gland enlargement
Ocular: sandy, gritty feeling under eye, dec tearing, erythema, itching, dry eyes!
60
Also causes xeropthalmia
Vit A deficiency
61
Sicca syndrome
Dry eyes
Dry mouth
Keratoconjunctivitis sicca
62
most common Extraglandular manifestation of Sjogren’s
Arthritis/arthralgia 60
Reynaud’s 37%
Fatigability/myalgia 25%
Lymphoma 6% MALT
63
Differential diagnosis for Sicca
HIV
Young male
Lacks autoantibodies Ro/SS-A and or B
Lymphocytic infiltration of CD8 lymphocytes
64
No age preference
Lack autoantibodies to Ro
Granuloma in salivary gland
Negative HIV
Sarcoidosis
65
Middle aged wimeb
Ro/SS A +
Lymphoid infiltrates of CD4+
HLADR3
Sjogren’s
66
Dx of Sjogren’s
Strongly supported by
Salivary gland biopsy
+ anti-Ro SS-A
+ anti-La SS-B
67
Cardiolipin
SLE
| APAS
68
Carbonic anhydrase II
Sjogren’s
| Systemic sclerosis
69
Centromere assoc proteins
Systemic sclerosis
70
Sjogren’s tx
Corticosteroid and immunosuppresive
71
Sjogren’s is also assoc with
```
RA
SLE
Scleroderma
Primary biliary cirrhosis
Autoimmune thyroid
Chronic active hepatitis
Mixed CTD
```
72
Autoantibody associated with primary biliary cholangitis
Antimitochondrial antibody
73
Most common lymphoma in Sjogren’s
MALT lymphoma
74
Hepatobiliary involvement in sjogren
Primary biliary cirrhosis
75
Tx of Sjogren’s
Azathioprine
| Mycophenolate mofetil
76
To lubricate dry mouth
Water
77
To lubricate eyes
Artifical tears
78
Arthritis in Sjogren’s
Hydroxychloroquine
| Methotrexate
79
Reynaud’s in Sjogren’s
Nifedipine
80
Renal Acidosis in Sjogren’s
Bicarbonate
81
Lymphoma in Sjogren’s
CHOP and anti CD20
Cyclophosphamide
Doxorubicin Adriamycin
Vincristine
Prednisone
82
Dilated cardiomyopathy
| Cardiac toxicity
Doxorubicin
83
Hemorrhagic cystitis
Cyclophosphamide
84
Vincristine
Oncovin
85
R in CHOP monoclonal antibody
rituximab
86
Methotrexate inhibits the enzyme
Dihydrofolate reductase
87
RA affects
0.5-1%
| F 2-3:1
88
RA Gene
HLA DRB1
0401 high risk
Europe and East Asian
89
Alleles known to confer greatest risk of RA within
Major Histocompatibility Complex MHC
90
Function of MHC
Bind peptide fragments from pathogens and display for cell surface recognition
91
Most common disease susceptibility alleles in Asians (Jap, Koreans, Chinese) in RA
0405
| 0901
92
Most risk of RA is associated with allelic variation
HLA DRB1 gene
93
RA environmental risk factors for RA
```
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
Infectious mononucleosis pathognomonic of
EBV Downey Type II cell
95
RA Pathology hallmark
1 synovial inflammation and proliferation
2 focal bone erosion
3 thinning of articular cartilage
96
RA has two cell types
1 Type A Synoviocyte from mac
| 2 Type B Synoviocyte from fibroblast
97
Most abundant RA cell type that produce structural components of joints such as collagen, fibronectinr and laminin
Type B Synoviocyte
98
Bone
Cartilage
BM
Collagen Types
I
II fibrocartilage, symphisis pubis
IV
99
25-55 y, F
| Symmetrical, polyarthritis >5 joints
RA
100
Earliest joint involved in RA
Small joint of hands and feet
101
Early morning stiffness in RA
>1 hr that eases with physical activity
102
RA symptom duration
>6 weeks
103
Once disease is established, Most frequently involved joints in RA (3)
1 wrist
2 MCP
3 PIP
104
RA spares
1 TLS
| 2 DIP of fingers and IP of toes
105
Hallmark of RA leading to dec ROM, reduced grip strength and trigger finger
Flexor tendon synovitis
106
Boutinniere Deformity
PIP flexion
DIP hyperExtension
MCP hyperExtension
107
Swan Neck Deformity
PIP hyperExtension
| DIP flexion
108
MCP in RA become chronically
ulnarly deviated
109
Heberden nodes are nodes on
DIP
110
Bouchard nodes are nodes on
PIP
111
Z line deformity is
subluxation of 1st MCP with hyperExtension of 1st IP joint
112
Hammer toe
Flexion of PIP of toe
| MTP Extension
113
Piano key movement
Inflammation of ulnar styloid
Tenosynovitis of ECU
Subluxation of distal ulna at the styloid
114
Boutinniere Deformity
PIP flexion
DIP hyperExtension
MCP hyperExtension
115
Swan Neck Deformity
PIP hyperExtension
| DIP flexion
116
MCP in RA become chronically
ulnarly deviated
117
Heberden nodes are nodes on
DIP
118
Bouchard nodes are nodes on
PIP
119
Z line deformity is
subluxation of 1st MCP with hyperExtension of 1st IP joint
120
Hammer toe
Flexion of PIP of toe
| MTP Extension
121
Claw toes
Flexion of both PIP and DIP
| Sparing the big toe
122
Constitutional signs and symptoms of RA
```
Most severe cases have cachexia TNF alpha
Depression
Malaise
Fatigue
Fever
Weight loss
```
123
Cachectin
TNF a
124
Constitutional signs and sx in RA reflect
High degree of inflammation
| Precede onset of joint symptoms
125
Fever in RA raises suspicion of
Systemic vasculitis
Infection
>38.3 101F
126
Extraarticular manifestions in RA develop in
40% of patients
May occur prior to onset
127
Extraarticular manifestions of RA
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
Piano key movement
Inflammation of ulnar styloid
Tenosynovitis of ECU
Subluxation of distal ulna at the styloid
129
IBS assoc with pyoderma gangrenosum
Ulcerative colitis
130
30-40%
Firm nontender adherent to periosteum tendon, bursa
Develop in areas subject to repeated trauma (forearm, sacral, achilles)
Lungs, pleura, pericardium and peritoneum
Rhematoid nodule
131
Clinicial features of RA Pulmonary
Pleuritis most common
ILD
Pulmonary nodule
132
Pleuritis present with
Pleuritic chest pain and dyspnea
| Pleural friction rub and exudative effusion
133
Rare subset of pulmonary nodulosis characterized by development of nodules and pneumoconiosis following silica exposure
Caplan’s syndrome
134
Caplan’s syndrome
Pulmonary nodule
pneumoconiosis
silica
135
Caplan’s is associated with
RA
136
Most frequently involved site of extraarticular affectation of RA
Pericardium
137
Cardiomyopathy in RA
Necrotizing and granulomatous myocarditis
CAD
Diastolic dysfunction
138
Most common valvular abnormality in RA
Mitral regurgitation
139
Caplan’s syndrome
Pulmonary nodule
pneumoconiosis
silica
140
Rare 1%
Most common in long standing disease
Rheumatoid factor
Hypocomplementenemia
Vasculitis
141
Cutaneous signs of RA
```
Gangrene digital infarct
Petechiae
Purpura
Livedo reticularis
Ulcer
```
142
Amantadine and minocycline may cause
Livedo reticularis
Also used in influenza
pd
143
Most common hematologic feature of RA
Anemia
Normo, normo
Degree parallels inflamm
Thrombocytosis
144
Parasite assoc with B12 deficiency
D latum
145
Felty Syndrome
```
1%
Neutropenia
Splenomegaly
Nodular RA
Thrombocytopenia
```
146
Most common lymphoma in RA
| 2-4% inc risk
Diffuse large B cell Lymphoma
147
Most common form of non hodgkin lymphoma in adults
Assoc with RA
Also in HIV
Diffuse Large B Cell Lymphoma
148
Most common cause of death in RA
Cardiovascular disease
Inc incidence of CAD and carotid atherosclerosis
CHF 2 fold higher
149
Bone problem in RA
Osteoporosis in 20-30% of px
From chronic glucocorticoid use
Disability related immobilitg
150
Associated condition in RA
hypoandrogenism
151
Criteria for RA
Joint involvement
Serology
Acute phase reactant (CRP)
Duration of symptoms (6wk)
More than 6 fulfills requirement
152
Changes in 2010 ACR/EULAR classification for RA
Inclusion of Anti cyclic citrullinated protein antibody
No longer takes into account presence of rheumatoid nodule or radiographic joint damage
153
RA Lab results
75-80% with RF
| also seen in Sjogrens, SLE
154
Anti CCP
Specificity
Prognosis
95%
| poor prognosis
155
Synovial fluid in RA predominantly has
neutrophil
| 5000-50000
156
Earliest radio finding in RA
Juxtaarticular osteopenia
Soft tissue swelling, symmetric joint space loss, subchondral erosion
Joint sublux and collapse
157
Imaging with greatest sensitivity for RA
MRI
| detects synovitis, joint effusion and early bone marrow changes
158
RA tx
NSAIDs
| Glucocorticoid
159
Glucocorticoid in RA
Low to moderate dose
| 5-10mg/day
160
Risk for osteoporosis in glucocorticoids
>5mg/day for more than 3 months
161
DMARD of choice for RA
Methotrexate 10-25mg/week
162
Hydroxychloroquine
Dose
SE
DMARD
200-400mg/day
Retinal toxicity, cardiotoxicity
163
Other DMARD tx
Leflunomide 10-20mg/day
Sulfasalazine 500mg also in IBS
se: Hemolytic anemia and SJS
164
Biologic agents in RA
AE
Anti TNF a
Infliximab
Adalimumab
AE: opportunistic funga infection and TB activation
165
RA Prognosis
10% spont remission within 6 mos
MC cause of death
IHD
166
RA Prognosis
10% spont remission within 6 mos
MC cause of death
IHD
167
Most common cause of MONOarticular arthritis
TB
168
Lupus flare
Fatigue
Arthritis
Mucocutaneous lesion
169
Autoantibody specific tor lupus like sx in INH use
Anti histone
