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Pathology > Multi-Systems: Various Pathology > Flashcards

Multi-Systems: Various Pathology Flashcards

1
Q
  • Fixed erythema, Flat or Raised
  • Over the Malar eminences “Butterfly shaped”
  • Tending to spare the Nasolabial folds
  • UV light initiates or exacerbates
  • Immunofluorescence (IF) reveals IC deposition along the basement membrane (Band Test)
A

Malar Rash

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2
Q
  • Erythematous raised patches w/ adherent Keratotic scaling and Follicular plugging
  • Atrophic scarring may occur in Older Lesions
A

Discord Rash

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3
Q
  • Rash as a result of unusual reaction to sunlight
  • UV light photosensitivity
  • -> radiation induces Apoptosis of Keratinocytes
  • -> releasing sequestered Intracellular Nuclear antigens
  • -> Ab against nuclear anitgens forming Immunocomplexes (ICs) (Band Test)
  • -> Vasculitis
A

Photosensitivity

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4
Q
  • Oral or Nasopharyngeal ulceration
  • Usually painless
  • Observed by a physician
A

Oral Ulcers

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5
Q
  • NonErosive - NonDeforming arthritis involving two or more peripheral joints - Symmetric in Hands and Wrists
    • Proximal Interphalangeal
    • Metacarpophalangeal
  • Tenderness
  • Swelling
  • Effusion
A

Arthritis

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6
Q
  • Pleuritis - convincing history of pleuritic pain or rub heard by a physician or w/ or w/out Pleural effusion
    • Interstitial Fibrosis –> Restrictive Lung Disease
  • -> Resulting in a Higher than 80% FEV1/FVC ratio
  • Pericarditis - documented by electrocardiogram or rub or w/ or w/out Pericardial effusion
    • **​Sterile vegitations over the Mitral valve (Apex)
  • -> Valve deformityandMitral Regurgitation**
  • -> Libman-Sacks Endocarditis
A

Serositis

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7
Q
  • Kidney is the most common Visceral organ involved
  • Chronic Renal failure is the most common cause of Death
  • Nephritic Syndrome
    • ​Hematuria, RBC casts in Urine, HTN
  • Persistant Proteinuria > 0.5 g/dL or > 3 if quantitation not performed
  • Cellular casts - may be RBC, Hemoglobin, Granular, Tubular, or Mixed
A

Renal Disorder

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8
Q
  • Headaches (most common)
  • Vessel thrombosis a/w Anti-phospholipid Syndrome
  • Seizures - In the absence of offending drugs or known metaboic derangements (Uremia, Ketoacidosis, or Electrolyte imbalance)
  • Psychosis - In the absence of offending drugs or known metabolic derangements (Uremia, Ketoacidosis, or Electrolyte imbalance)
A

Neurologic Disorder

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9
Q
  • Hemolytic anemia - w/ reticulocytosis
  • Leukopenia - < 4 x109 total on two or more occasions
  • Lymphopenia - < 1.5 x109 on two or more occasions
  • Thrombocytopenia - < 100 x109 in the absence of offending drugs
A

Hematologic Disorder

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10
Q
  • Anti-DNA antibody to Native DNA in Abnormal titer
  • Anti-SM - presence of Ab to Sm nuclear antigen
  • Positive finding of Antiphospholipid Ab
    • Abnormal IgG or IgM anticardiolipin antibodies
    • Positive for Lupus anticoagulant
    • False-positive for Syphilis
A

Immunologic Disorder

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11
Q
  • Abnormal titer of Antinuclear antibody (Serum ANA) by Immunoflurescence or equivalent assay at any point in time and in the Absence of Drugs to be a/w drug induced Lupus
    • Hydralazine
    • Procainamide
    • Isoniazid
    • D-penicillamine
A

Antinuclear Antibody

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12
Q

(11) Criteria for Systemic Lupus Erythematosus?

A
  1. Malar rash
  2. Discoid rash
  3. Photosensitivity
  4. Oral ulcers
  5. Arthritis
  6. Serositis
  7. Renal disorder
  8. Neurologic disorder
  9. Hematologic disorder
  10. Immunologic disorder
  11. Antinuclear antibody
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13
Q

Double-stranded DNA Ab

A

SLE

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14
Q

U1-RNP Ab

A

SLE

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15
Q

Sm antigen (Smith) Ab

(core protein in small RNP particles)

A

SLE

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16
Q

Ro / La Nucleoprotein Ab

(SS-A / SS-B)

Congenital Heart Block

Neonate

A

SLE

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17
Q

anti-Pl Ab

(Phospholipid-protein complexes)

A

SLE

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18
Q

anti-Scl 70

DNA topoisomerase 1 Ab

Diffuse skin disease, Lung disease

A

Systemic Sclerosis

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19
Q

Centromeric Proteins A, B, C

(CENPs)

Limited Skin Disease
Ischemic Digital Loss
Pulmonary HTN

A

Systemic Sclerosis

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20
Q

RNA Polymerase III Ab

Acute Onset
Sclerodermal Renal Crisis
Cancer

A

Systemic Sclerosis

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21
Q

Ro / La

(SS-A / SS-B)

w/ No Association

A

Sjogren Syndrome

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22
Q

Jo1 Ab

Histidyl aminoacyl-tRNA synthetase

Interstitial Lung Disease
Raynaud Phenomenon

A

Autoimmune Myositis

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23
Q

Mi-2 Nuclear antigen Ab

Dermatomyositis
Skin Rash

A

Autoimmune Myositis

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24
Q

MDA5 Ab

(Cytoplasmic receptor for Viral RNA)

Vascular Skin Lesions
Interstitial Lung Disease

A

Autoimmune Myositis

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25
Q

TIFIγ Nuclear Protein Ab

Dermatomyositis
Cancer

A

Autoimmune Myositis

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26
Q

CCP Ab

(Cyclic Citrullinated Peptides

Various citrullinated proteins

A

Rheumatoid Arthritis

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27
Q

Rheumatoid Factor Ab

A

Rheumatoid Arthritis

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28
Q

Anti-acetylcholine receptor Ab

(1x)

A

Myasthenia Gravis

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29
Q

Anti-basement Membrane Ab

(1x)

A

Goodpasture Syndrome

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30
Q

Anti-centromere Ab

(1x)

A

CREST Syndrome Diffuse Systemic Sclerosis

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31
Q

Anti-endomysial IgA Ab

(1x)

A

Celiac Disease

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32
Q

Anti-gliadin IgA Ab

(1x)

A

Celiac Disease

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33
Q

Anti-histone Ab

(1x)

A

Drug-Induced Lupus

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34
Q

Anti-insulin Ab

(2x)

A

Systemic Lupus Erythematosus

Type 1 Diabetes

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35
Q

Anti-intrinsic Factor Ab

(1x)

A

Pernicious Anemia

36
Q

Anti-microsomal Ab

(1x)

A

Hashimoto Thyroiditis

37
Q

Anti-Smith (Sm) Ab

(1x)

A

Systemic Lupus Erythematosus

38
Q

Anti-SS-A Ab

(Ro)

(2x)

A

Sjogren Syndrome

Systemic Lupus Erythematosus

39
Q

Anti-SS-B Ab

(La)

(1x)

A

Sjogren Syndrome

40
Q

Anti-Thyroglobulin Ab

(2x)

A

Systemic Lupus Erythematosus

Hashimoto Thyroiditis

41
Q

Anti-tissue Transglutaminase IgA Ab

(1x)

A

Celiac Disease

42
Q

Anti-DNA Topoisomerase Ab

(1x)

A

Diffuse Systemic Sclerosis

43
Q

Anti-mitochondrial Ab

(2x)

A

CREST Syndrome

Primary Biliary Cirrhosis

44
Q

Anti-myeloperoxidase Ab

(1x)

A

Microscopic Polyangiitis

45
Q

Anti-nuclear Ab

(3x)

A

Systemic Lupus Erythematosus

Systemic Sclerosis

Dermatomyositis

46
Q

Anti-proteinase 3

(4x)

A

Polymyositis

MCTD

Primary Biliary Cirrhosis

Wegener Granulomatosis

47
Q

Anti-ribonucleoprotein Ab

(2x)

A

MCTD

Systemic Lupus Erythematosis

48
Q

Anti-TSH receptor Ab

(1x)

A

Graves Disease

49
Q

(4x) Environmental Triggers for SLE

A
  1. Infectious agents (EBV)
  2. Ultraviolet light
    • Induces Apoptosis of Keratinocytes
      - -> Releasing sequestered Intracellular nuclear antigens –> Immunocomplexes –> Vasculitis
      - -> Malar rash
  3. Estrogen
    • Triggers B cells against DNA
  4. Medications (4x)
50
Q

(2x) Coxsackievirus a/w autoimmune disorder?

A

Myocarditis (B3)

Type I Diabetes Mellitus (B4)

51
Q

(1x) Measles virus a/w autoimmune disorder?

A

Allergic Encephalitis

52
Q

(1x) CMV a/w autoimmune disorder?

A

Systemic Sclerosis

53
Q

(3x) EBV a/w autoimmune disorders?

A

Hepatitis B

Systemic Lupus Erythematosus (SLE)

Rheumatoid Arthritis (RA)

54
Q

(1x) Human Herpes Virus (HHV-6)

Influenza A Virus

Both a/w autoimmune disorder?

A

Multiple Sclerosis

55
Q

CREST Syndrome is a Acronym for what?

A
  • Limited Systemic Sclerosis
  • C - Calcification
  • R - Raynaud phenomenon
  • E - Esophageal dysmotility
  • S - Sclerodactyly (tapered, claw-like)
  • T - Telangiectasias (multiple punctate blood vessel dilations)
56
Q
  • Females 30 - 40 y.o.
  • Black females greatest population
  • CD4 TH2 cells
  • Anti-Scl-70 (Anti-Topoisomerase I Ab)
  • PDGF and TGF-β
  • Raynaud phenomenon
  • Gastrointestinal Hypomotility and Dysphagia
  • Malabsorption due to Loss of Villi and Diverticula
  • Constipation
  • Pulm. HTN –> Right Heart Failure –> cor Pulmonale
  • Renal problems w/ Vasculitis and Malignant HTN
A

Systemic Sclerosis

57
Q
  • Female dominant, esp. w/ Black Population
  • 40 - 60 y.o.
  • Increased Risk of Lung and Bladder cancer
  • Fever, Morning stiffness, Fatigue, Weight loss
  • Symmetrical and Proximal Muscle weaknes w/ or w/out Pain in both Upper and Lower extremities, Trunk Shoulders and Hips
  • Dysphagia in Upper Esophagus
  • Interstitial Lung disease
  • Serum ANA, Anti-transfer RNA synthetase (Jo-1) Ab
A

Polymyositis

58
Q
  • Reddish-purple papules - “Gottron patches” - Knuckles / Proximal Interphalangeal (PIP) joints Both Hands
  • Purple-red eyelids - Heliotrope eyelids “Racoon eyes”
  • CD4 T cells –> Capillaries in Skeletal Muscle
  • -> Ab and Complement –> capillary damage
  • -> Ischemia and Atrophy of Muscle fibers
A

Dermatomyositis

59
Q
  • Female dominant
  • 15 - 25 y.o.
  • T cells and B cells –> Anti-Ribonucleoprotein (U1-RNP)
  • Raynaud phenomenon and Sclerodactyly
  • Arthralgia and Arthritis involving the Hands
  • Esophageal dysmotility
  • Pulmonary HTN, Pleuritis –> a/w anti-Phospholibid Ab
  • Pericarditis
  • Trigeminal neuralgia –> Face pain
  • Serum ANA, Rheumatoid factor, anti-dsDNA,
    anti-DNA Topoisomerase
A

Mixed Connective Tissue Disease (MCTD)

60
Q
  • Fatigue, Dyspnea, Pitting Edema, Parasthesias, Weight Loss, Diarrhea of Malabsorption, Macroglossia
  • Restrictive Cardiopathy, Dementia (Alzheimer disease), Hepatomegaly w/ Pressure Atrophy, Muscle weakness
  • Fibrillar protein deposited in Interstitial, β-pleated sheets
  • Congo red and Apple Green Birefringence
  • Immunoglobulin light chains; λ chains > κ chains
  • Bence jones proteins = Light chains in Urine
  • Serum Amyloid A (SAA)
  • Amyloid Precursor Protein (APP)
  • Transthyretin; carrier for Thyroxine / Retinoic Acid (Vit. A)
  • β2-Microglobulin = Light chain component of MHC
A

Amyloidosis

61
Q

AL

λ Chains
Amyloid derived from Immunoglobulin Light Chains

A

Immunocyte Dyscrasias - Systemic Amyloidosis

(Primary Amyloidosis)

62
Q

AA

Amyloid derived from Serum Amyloid A Protein

A

Reactive Systemic Amyloidosis - Systemic Amyloidosis

(Secondary Amyloidosis)

63
Q

Aβ2m

Amyloid derived from β2-microglobulin

A

Hemodialysis-associated Amyloidosis - Systemic Amyloidosis

(Chronic Renal Failure)

64
Q

AA

Derived from Serum Amyloid Protein, Autosomal Recessive

IL-1

A

Familial Mediterranean Fever - Hereditary Amyloidosis

65
Q

ATTR

Amyloid derived from Transthyretin, Autosomal Dominant

A

Familial Amyloidotic Neuropathies - Hereditary

Peripheral and Autonomic Nerve Disorders

66
Q

Amyloidosis of Elderly Patients

(70+ years old)

A

Systemic Senile Amyloidosis - Hereditary

Predominantly Involves the Heart

(Restrictive cardiomyopathy, Conduction defects)

67
Q

Aβ

Amyloid derived from Amyloid Precursor Protein, Chrom 21)

A

Senile Cerebral Amyloidosis - Localized

Alzheimer disease

68
Q

A Cal

Amyloid derived from Calcitonin

A

Medullary Carcinoma of Thyroid - Endocrine Amyloid

Sporadic and Familial MEN IIa and MEN IIb

69
Q

AIAPP

Amyloid derived from Islet Amyloid Polypeptide

A

Islets of Langerhans - Endocrine Amyloid

Type II Diabets Mellitus

70
Q
  • “Sand in my eyes” - Keratoconjunctivitis sica
  • “Can’t swallow crackers” - Xerostomia or Dry Mouth
  • Rheumatoid arthritis
  • Female dominant
  • Dental caries
  • Autoimmune destruction of Minor Salivary Glands and Lacrimal Glands
  • Anti-SS-A (Anti-Ro)
  • Anti-SS-B (Anti-La)
  • Serum ANA, Serum RF
  • Confirm w/ Lip Biopsy
A

Sjogren Syndrome

71
Q
  • Chronic Joint Inflammation
  • Females, 30 - 50 y.o.
  • HLA-DR4
  • EBV, Parvovirus, HH-6, Mycoplasma
  • Inflamed synovial cells –> Triggers B cells
  • RF is a IgM Ab w/ specificity for Fc portion of IgG
  • -> RF + IgG = ICs –> Activate Compliment –> C5a
  • -> Chemotactic for Neutrophils and Leukocytes
  • Chronic Synovitis and Pannus (ILs, TNF) –> Desroy Articular Cartilage –> Fusion of Joint (Ankylosis)
  • Swan Neck deformity and Boutonniere deformity
A

Rheumatoid Arthritis

72
Q
  • Fever of unknown origin
  • Middle aged Men
  • HBsAg, HCV, HBV a/w Polyarteritis Type III
  • Acute and Chronic inflammation
  • Focal vasculitis produces Aneurysms (angiography)
    • Medium sized vessels, spares Pulmonary
  • Kidnesy (Renal failure, Hematuria)
  • Heart (Acute MI)
  • Bloody diarrhea
  • Ischemic Ulcers on Skin
  • Testicular Pain
A

Polyarteritis Nodosa

73
Q
  • Metabolic syndrome, Obesity, DM2, Insulin resistance, Dyslipidemia, HTN
  • Excessive synthesis of TGs in the Liver
  • DHAP + NADH –> G3-P + 3x FAs –> TG
  • Apoprotein B (apoB)-100 protein component of VLDL serves to enhance the secretion into the Blood
  • Kwashiorkor –> Increased intake of Carbs and no Protein
  • -> CHO (carbs) –> DHAP and more TGs
  • -> No protiens –> reduced apoB-100 –> less secretion
  • Yellow-liver discoloration
  • Pushing nucleus to side
A

Nonalcoholic Fatty Liver Disease (NAFLD)

(Kwashiorkor)

74
Q
  • Micronodular cirrhosis (ALL pts.)
  • Diabetes Mellitus Type I (75 - 80%) “Bronze Diabetes”
  • Abnormal skin pigmentation (75 - 80%)
  • 40 - 50 y.o. in Men, Later in women, Menses counters
  • Hereditary - regulation of Intestinal absorption of dietary Iron is abnormal –> Net Iron accumulation (sig. @ 20gm)
    • HFE gene C282Y (whites) and H63D (world),
      HLA-A3, Transferrin receptor 2 (TFR2), Hepcidin
  • Secondary - consequence of Parenteral administraion
A

Hemochromatosis

75
Q
  • Kayser-Fleisher Rings (70%)
    • Deposits in Descemet membrane in the Cornea
  • CNS disease
    • Deposits in Putamen –> Parkinsonism
    • Deposits in Subthalamic nucleus –> Hemiballismus
    • Deposits in Cerebral Cortex –> Dementia
  • Hepatosplenomegaly (50%)
  • Hemolytic anemia
  • Renal disease and Proximal tubule dmg –> Fanconi synd.
A

Wilson Disease

76
Q
  • Autosomal dominant (codominant)
  • Most common cause of Cirrhosis in Children
  • A/w Panacinar Emphysema
  • Accumulation of AAT in Hepatocytes
    Hepatitis –> Cirrhosis –> Liver damage
    –> Increased risk of Hepatocellular carcinoma
A

α1-Antitrypsin Deficiency (AAT)

77
Q
  • Multisystem noninfectious Granulomatous disease that produces chronic interstitial Fibrosis
  • Blacks and Non-smokers
  • F > M
  • CD4 TH cells (Mold, Mildew, Pesticides, Mycobacterial KatG protein) –> Cytokines –> Noncaseating Granulomas
  • Granulomas located in Lung and Dyspnea
  • Violaceous rash (nose and cheeks –> Lupus pernio)
  • Erythema nodosum
  • Blurry vision, Glaucoma, Corneal Opacities
  • Granulomatous Hepatitis
  • Enlarge Salivary and Lacrimal Glands
  • Central Diabetes Insipidus
  • Calcium Renal Stones, Nephrocalcinosis
A

Sarcoidosis

78
Q
  • Monosodium Urate (MSU)
  • Men > 30 y.o.
  • Primary - Deficiency of Hypoxanthine-guanine phosphoryltransferase (HGPRT) in Lesch-Hyhan Syn.
  • Secondary - Underexcretion of Uric Acid
    • Lead poisoning, Alcoholism, Red meat, Seafood, Thiazides, Beer
  • Overproduction of Uric acid - Leukemia, Psoriasis
  • UA crystals create Tophus –> Destroy adjacent bone
  • -> Erosive Arthritis
A

Gout

79
Q
  • Characteristics
    • Rheumatoid factor (RF) negative
    • Axial skeleton (spondylitis)
    • HLA-B27 Positive
    • M > F
    • Sacroiliitis w/ or w/out Peripheral arthritis
  • Types
    • Ankylosing
    • Reactive arthritis
      • Reiter syndrome, A/w Gastroenteritis
    • Psoriatic arthritis
    • Enteropathic arthritis
A

Spondyloarthritis

(Seronegative Spondyloarthropathies)

80
Q
  • Morning Stiffness w/in the Sacroiliac Joint
  • -> eventually involves the Vertebral column
  • -> Fusion of vertebrae (bamboo spine)
  • -> Kyphosis (forward curvature)
  • Leads to reduced Chest wall movement
    • Nonpulmonary Restrictive Lund Disease
  • Aortitis w/ Aortic Regurgitation
  • Anterior Uveitis
    • Blurry vision, may lead to Blindness
A

Ankylosing Spondylitis (AS)

81
Q
  • Urethritis due to Chlamydia trachomatis
  • Arthritis and Achilles Tendon periostitis
    • Bone formation at junction of Achilles tendon w/ Plantar Fascia
  • Conjunctivitis - non-infectious
  • Circinate Balanitis
    • Rash on distal Shaft and Glans Penis
    • Appears as Vesicles
    • Shallow ulcerations
A

Reiter Syndrome

82
Q
  • Sausage-shaped DIP joints
    • Finger or Toe
  • Radiographs show Erosive Joint Disease
    • “Pencil-in-cup” deformity
  • Extensive nail pitting
A

Psoriatic Arthritis

83
Q
  • Most common non-gonococcal cause of Septic Arthritis
  • Tx: Nafcillin + Cephalosporin (3rd generation)
A

Staphylococcus aureus - Septic Arthritis

84
Q
  • Most common Septic arthritis in Urban areas
  • Disseminated Gonococcemia
  • Deficiency of C6 - C9 complement
  • Septic arthritis - Knee
  • Tenosynovitis - Wrist and Ankles
  • Dermatitis - Pustules on Wrists and Ankles
  • Tx: Ceftriaxone
A

Neisseria gonorrhoeae - Septic Arthritis

85
Q
  • Borrelia burgdorferi (spirochete) transmitted by Ixodes
  • Vesicular red center “Bull’s eye” Lesion
    • Empirical therapy is recommended
  • Fever, Flu-like symptoms, chills, Headache, Arthralgia and Neck Stiffness
  • Skin Lesions and Lymphadenopathy
  • Migratory Polyarticular Arthritis (Bursa, Tendons, Joints)
  • Neuroborreliosis w/ Cranial Neuropathy (Bell’s Palsy) Bilaterally, Nucal rigidity
A

Lyme Disease (LD) arthritis

Pathology (36 decks)

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