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From Flashcardlet > 7.6.2013(connective Tissue Disorders) 30 > Flashcards

7.6.2013(connective Tissue Disorders) 30 Flashcards

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0
Q

P ANCA predominant diseases, associated with MPO

A 
Microscopic polyangitis
Churg Strauss 
Crescentric glomerulonephritis 
Renal limited vasculitis
Goodpasture(30%)
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1
Q

Medium vessel vasculitis

A

PAN
cutaneous PAN
kawasaki
Burger

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2
Q

P ANCA not associated with MPO

A 
IBD
IE
RA
SLE
HIV
autoimmune hepatitis
Sclerosing cholangitis
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3
Q

ANCA negative small vessel vasculitis

A

HSP
essential cryoglobulinemic vasculitis
Cutaneous Leukocytoclastic angitis(Hypersensitivity Vasculitis)

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4
Q

Age of presentation of wegener

A

No age predilection but rare in children

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5
Q

Vasculitis associated with thrombocytosis

A

Wegener

Kawasaki

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6
Q

Complement levels in wegener

A

Normal

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7
Q

Most common manifestation of wegener

A

URT vasculitis

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8
Q

Ocular manifestations of wegener

A 
Orbital pseudotumor
Scleritis/episcleritis
Keratitis
Conjunctivitis
Uveitis
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9
Q

Most common symptom of PAN

A

Musculoskeletal

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10
Q

Cause of death in PAN

A

Cardiovascular,GIT

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11
Q

Skin lesions of PAN

A

Livido reticularis
Digital gangrene
Palpable Purpura

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12
Q

Most specific symptom of temporal arteritis

A

Jaw claudication

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13
Q

Risk of blindness in temporal arteritis correlates with

A

IL-6

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14
Q

Infections associated with Kawasaki

A

Mycoplasma
HIV
S.aureus

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15
Q

Serum complements in HSP

A

Normal

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16
Q

Distribution of Purpura in HSP

A

Buttocks

Lower extremities

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17
Q

Migratory non deforming arthritis seen in

A

Wegener

HSP

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18
Q

HLA in Behcet

A

HLAB5

HLADR5

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19
Q

Behcet affects

A

Young people

Both males and females equally affected

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20
Q

Types of oral ulcers in Behcet

A

Minor aphthous
Major aphthous
Herpetiform

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21
Q

Features of Behcet oral ulcers

A

Painful

Heal without scarring

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22
Q

Which ulcers are more specific for Behcet

Oral or genital

A

Genital

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23
Q

Feature of genital ulcers

A

Produce scars

Scrotal scars

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24
Thrombosis in Behcet
Superficial and deep venous thrombosis | Arterial
25
GIT involvement in Behcet
Picture similar to Crohn
26
Antibodies in Behcet
ASCA | Alpha enolase of endothelial cells
27
Marker of CNS Behcet
IL-6
28
Rx of Behcet
``` Steroids Colchicine Interferon alpha(Refractory uveitis,CNS) Azathioprine Thalidomide ```
29
Skin lesion in Behcet
Erythema nodosum Pseudofolliculits Pseudo pustular lesions Acneform nodules
30
Infections causing reiter
``` GIT: Shigella Salmonella Campylobacter Yersinia GENITAL: chlamydia ```
31
Nail changes in reiter
Onycholysis Hyperkeratosis Distal yellowish discolouration
32
Cardiac complications of reiter
AR | Cardiac conduction defects
33
Neurological involvement in Behcet
Dural vein thrombosis
34
Mucocutaneous features of reiter
Oral ulcers Circinate balanitis Keratoderma blenorrhagica
35
PAN only affects
Muscular arteries
36
Microaneurysms are characteristic of
PAN
37
Disease recurrence is high in
Wegener
38
Microscopic polyangitis
Microscopic PAN
41
Immunoglobulins elevated in wegener
IgA
42
ANCA negativity in wegener
10% in disseminated wegener | 30% in limited wegener
43
Definitive diagnosis of wegener
Biopsy
44
Goodpasture occurs in
Young male smoker
45
Arteries spared in PAN
Pulmonary | Bronchial arteries may be involved(very rare)
46
Common causes of mononeuritis multiplex
``` Classic PAN Microscopic PAN RA SLE MCTD ```
47
Fever in Kawasaki lasts for
At least 5 days
48
Sodium in Kawasaki
Hypernatremia
49
Albumin levels in Kawasaki
Hypoalbumunemia
50
Lab abnormalities in Kawasaki
``` Anemia Leukocytosis Thrombocytosis High ESR Elevated CRP hypoalbunemia Hypernatremia Sterile pyuria Elevated transaminases and GGT CSF pleocytosis Leukocytosis in synovial fluid ```
51
Rash in Kawasaki
Polymorphous exanthem
52
Coronary artery aneurysms in Kawasaki are detected by
2D ECHO
53
Coronary artery aneurysms occur after
2-3wks
54
Corticosteroid Rx in Kawasaki
Persistent fever despite 2 doses of IVIG
55
Age group commonly affected in HSP
2-7 | Infants and adults are also affected
56
HSP associated intusucception
Ileoileal
57
Geographical distribution of Behcet
World wide
58
Arthritis in Behcet
Non deforming | Affects knees and ankles
59
Genital Behcet don't affect
Glans | Urethra
60
Characteristic eye finding in Behcet
Recurrent bilateral hypopyon
61
hypopyon in HLA B27 associated uveitis is
UNILATERAL
62
ACR criteria for cutaneous small vessel vasculitis
Age>16yrs Medication at disease onset Palpable Purpura Biopsy of venule shows granulocytes in peri vascular or extravascular location
63
Most specific antibodies for SLE
AntiSm
64
Antibodies in sjogren
SSA(Ro) | SSB(La)
65
Decreased risk for lupus nephritis | Antibodies
SSA | SSB
66
Antibodies in neonatal lupus
SSA(Ro)
67
Antibodies in MCTD
Ab to ribonucleoprotein antigen containing ULRNP
68
Diffuse systemic sclerosis | Antibody
Anti DNA topoisomerase (scl70)
69
Anti bodies in inflammatory myopathy
Histidyl-tRNA synthetase | Anti-Jo1
70
Differences btw classical and drug induced lupus
CNS and renal involvement rare Not associated with anti dsDNA Less predilection for females Resolves following discontinuation of medication
71
HLA associated with lupus following hydralzine
HLAD4
72
Antiarrythmics that can cause lupus like reaction
Procainamide | Quinidine
73
Anti hypertensive that can cause lupus
Hydralzine Methyl DOPA Captopril Acebutalol
74
Anticonvulsant that can cause lupus
Carbamazepine
75
Antibiotics associated with lupus
Isoniazid | Minocycline
76
Antipsychotic associated with lupus
Chlorpromazine
77
Anti inflammatory drugs associated with lupus
D penicillamine | Sulfasalazine
78
Anti thyroid drugs associated with lupus
PTU
79
Renal disorder in SLE
Protenuria >0.5g/d Or >=3+ Cellular casts
80
Neurological manifestations of SLE
Seizures | Psychosis
81
Malar rash
Fixed erythema,flat or raised over the malar eminence
82
Arthritis in SLE
Peripheral non erosive
83
Discoid rash
Erythematous circular raised patches with adherent keratotic scaling and follicular plugging,atrophic scarring may be present
84
Ocular manifestations of SLE
Conjunctivitis Episcleritis Retinal vasculitis Optic neuritis
85
Pulmonary manifestations of SLE
``` Pleuritis Pleural effusion Interstitial fibrosis Intra alveolar hemorrhage Shrinking lung syndrome ```
86
Shrinking lung syndrome
Diaphragmatic weakness in SLE
87
Wire loop lesions
``` Class IV(commonly) Class III>class V ```
88
Lupus nephritis with sub epithelial deposits
Class V membranous lupus
89
Class VI lupus nephritis
>90% of glomeruli globally sclerosed without residual activity
90
SLE pts with associated sjogren
Lesser risk of renal involvement
91
Poor prognostic factors for SLE nephritis
``` Blacks Male gender Extremes of age Hypertension Severe extra renal (esp neuropsychiatric) lupus activity Pregnancy Nephritic urinary sediment Azotemia Anemia Thrombocytopenia Thrombotic microangiopathy Hypocomplementemia(falling titre) High anti-DNA(rising titer) Persistent severe Nephritic syndrome(thrombotic or atherosclerotic diathesis) ```
92
Pathological findings indicating poor prognosis
``` Contracted kidney size Class III IV mixed membranous and proliferative disease Cellular crescents(>25% of glomeruli) Fibrinoid necrosis(>25% of glomeruli) Very high activity index Moderate to high chronicity index Combination of active(cellular crescent) and chronic features(interstitial fibrosis) Extensive subendothelial deposits ```
93
Cholasma
Non Erythematous malar rash
94
Erythematous malar rash
SLE photo sensitivity Contact dermatitis
95
Obstetrical complications of lupus anticoagulant syndrome
``` Recurrent thrombotic event Thrombocytopenia Recurrent spontaneous abortions IUGR PIH placental abruption ```
96
Lab features in lupus anticoagulant syndrome
``` Elevated Anti cardiolipin ab Anti beta 2 glycoprotein-1 Lupus anticoagulant Thrombocytopenia Hemolytic Anemia ```
97
Lab criteria for diagnosis of APLAS
Elevation of anti cardiolipin abs on two or more occasions atleast 12wks apart
98
Pregnancy morbidity in lupus anticoagulant
Three or more unexplained Consecutive abortions 10wks | One or more premature births>34wks because of placental insufficiency or eclampsia
99
Pulmonary manifestation of APLAS
Pulmonary hypertension due to recurrent subclinical pulmonary emboli
100
Acute false positive VDRL <6months
``` Recent viral infection/immunisation Genital herpes HIV Malaria Panteral drug use ```
101
Chronic false positive VDRL >6months
``` Ageing Autoimmune disorders SLE RA parenteral drug use ```
102
Rx for women with recurrent pregnancy loss associated with APLAS
Aspirin+heparin
103
Viral causes of B/L parotid enlargement
``` Mumps Influenza Epstein bar Coxsackie 4 CMV HIV ```
104
Metabolic causes of B/L parotid enlargement
DM hyperlipoproteinemia Chronic pancreatitis Cirrhosis
105
Endocrine causes of B/L parotid enlargement
Acromegaly | Gonadal hypofunction
106
Immune causes of B/L parotid enlargement
Sarcoidosis Amyloidosis Sjogren
107
Indications of corticosteroids in SLE
``` Thrombocytopenic Purpura Hemolytic Anemia Nephritis Pericarditis Myocarditis Convulsions ```
108
Indications for low dose aspirin
Preeclampsia IUGR post MI
109
Poor prognostic factors in RA
Elevated Rheumatoid factor C reactive protein Haptoglobin
110
Radiographic features in RA
Juxtaarticular osteoporosis Joint erosion Narrowing of jt spaces
111
Jt not involved in RA
DIP
112
RA affects
Women 3:1
113
Extraarticular manifestations are seen in ___________ % of pts of RA
40% | And in 15% these are severe
114
Rheumatoid nodules are seen in ___________ % of pts
20%
115
Felty syndrome
RA Splenomegaly Neutropenia Thrombocytopenia and Anemia may also be seen
116
False positive RF | Infectious causes
``` IM Hepatitis B TB leprosy Syphilis Subacute bacterial endocarditis Visceral leishmaniasis Schistosomiasis Malaria ```
117
False positive RF
``` SLE Sjogren Chronic liver disease Sarcoidosis IPF ```
118
Type of Anemia in RA
Normochromic Normocytic
119
Possible infectious causes for RA
``` Mycoplasma Epstein Barr virus CMV parvovirus Rubella ```
120
HLA associated with RA
``` HLADR 1 4 6 9 10 14 ```
121
HLA protective against RA
``` HLADR 2 3 5 7 ```
122
HLAB27 arthritis
``` AS psoriatic arthritis Reiter Enteropathic arthritis Undifferentiated spondyloarthropathy ```
123
DMARDS
``` Methotrexate (DMARD of choice) Gold Penicillamine Chloroquine Sulfasalazine ```
124
Chrysotherapy
Gold treatment in RA
125
Gold preparations
i.m gold sodium thromalate or aurothioglucose(more effective than oral gold) Oral auranofin
126
Indications of systemic steroids in RA
To tide over acute crisis Vasculitis: causes mononeuritis multiplex Pericarditis Perforating eye lesions
127
Bone changes in systemic sclerosis
Resorption of terminal phalanges Ribs Clavicle Angle of mandible
128
GIT involvement in systemic sclerosis
Pseudo obstruction
129
Pneumatosis intestinalis is seen in
NEC | systemic sclerosis involving small intestine
130
Raynaud phenomenon in CREST
May precede skin disease by years In diffuse scleroderma,Raynaud occurs within 1yr or at the time of skin changes
131
Pulmonary arterial hypertension is seen in
CREST syndrome, after 10-15yrs of disease Interstitial fibrosis occurs in diffuse disease
132
Skin involvement in CREST
Distal to elbow | Face
133
Skin involvement in diffuse SSc
Distal and proximal extremities Face TRUNK Rapid progression
134
Billiary cirrhosis is seen in
CREST syndrome
135
Scleroderma is common in
Women 3:1 Peak age: 3rd to 5th decade
136
Skin changes in systemic sclerosis
Diffuse tanning in the absence of sun exposure
137
Rheumatologic causes of Raynaud
``` Scleroderma SLE RA sjogren Dermatomyositis Polymyositis Vasculitis ```
138
Mechanical causes of Raynaud
Vibration Frostbite Thoracic outlet syndrome Vascular embolus
139
Haematological disorders causing Raynaud
Cryoglobulinemia Paraproteinemia Polycythemia Cold agglutinins
140
Endocrine disorders causing Raynaud
Hypothyroidism Carcinoid Pheochromocytoma
141
Drugs associated with Raynaud
``` Sympathomimetics Serotonin agonists Bleomycin,cisplatin,carboplatin,vinblastine Ergotamine Caffeine Nicotine ```
142
Extra glandular manifestations of sjogren
``` Arthritis Raynaud Vasculitis Lymphoma RTA ```
143
Most common autoimmune disorder associated with sjogren
Rheumatoid arthritis
144
Genomic imprinting is seen in
Albright hereditary dystrophy Beckwidth wideman Angelman Praderwilli
145
Skin involvement in sarcoidosis
Erythema nodosum | Lupus pernio
146
Bodies in sarcoidosis
Schaumann bodies Asteroid bodies Residual bodies
147
Heerfordt syndrome
``` Uveoparotid fever Fever B/L parotid enlargement Anterior uveitis 7th nerve palsy ```
148
Lofgren syndrome
Arthritis Erythema nodosum B/L hilar adenopathy
149
X ray pattern of pulmonary sarcoidosis
Type1 : b/L hilar adenopathy with no parenchymal changes Type2: B/L hilar adenopathy with diffuse parenchymal changes Type3: diffuse parenchymal disease with no hilar adenopathy
150
CD4:CD8 ratio in sarcoidosis
Normal in BLOOD | elevated in affected organs only
151
ACE levels indicate
Activity of disease | Elevated in 2/3 of pts with sarcoidosis
152
Gallium 67 scan is used in diagnosis of
Sarcoidosis
153
Kveim siltzbach test
Intradermal injection of heat treated suspension of sarcoidosis spleen extract
154
Conditions associated with HLAB27
``` AS reiter Reactive arthritis Psoriatic arthritis JRA ACUTE ANTERIOR UVEITIS ```
155
Type of arthritis in seronegative spondyloarthritis
Assymmetric oligoarthritis
156
Conditions associated with HLA B8
Myasthenia gravis | Graves disease
192
BAL in wegener shows
High amount of neutrophils
194
Causes of false positive VDRL
``` Syphilis SLE RA malaria Leprosy Relapsing fever Hepatitis Infectious mononucleosis Tropical pulmonary eosinophilia ``` SRini MaLaR HIMalayas T
195
Causes of false positive VDRL
``` Syphilis SLE RA malaria Leprosy Relapsing fever Hepatitis Infectious mononucleosis Tropical pulmonary eosinophilia ``` SRini MaLaR HIMalayas T
196
Intrahepatic amyloid
Caroli
197
Causes of false positive VDRL
``` Syphilis SLE RA malaria Leprosy Relapsing fever Hepatitis Infectious mononucleosis Tropical pulmonary eosinophilia ``` SRini MaLaR HIMalayas T
198
Intrahepatic amyloid
Caroli
199
Intrahepatic amyloid
Caroli
200
Lab features of wegener
RF+ | Hypergammaglobulinemia

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