Multi-Systems: Various Pathology Flashcards by James Wren

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)

Malar Rash

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

Discord Rash

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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

Photosensitivity

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Oral or Nasopharyngeal ulceration
Usually painless
Observed by a physician

Oral Ulcers

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

Arthritis

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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

Serositis

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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

Renal Disorder

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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)

Neurologic Disorder

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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

Hematologic Disorder

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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

Immunologic Disorder

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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

Antinuclear Antibody

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(11) Criteria for Systemic Lupus Erythematosus?

Malar rash
Discoid rash
Photosensitivity
Oral ulcers
Arthritis
Serositis
Renal disorder
Neurologic disorder
Hematologic disorder
Immunologic disorder
Antinuclear antibody

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Double-stranded DNA Ab

SLE

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U1-RNP Ab

SLE

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Sm antigen (Smith) Ab

(core protein in small RNP particles)

SLE

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Ro / La Nucleoprotein Ab

(SS-A / SS-B)

Congenital Heart Block

Neonate

SLE

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anti-Pl Ab

(Phospholipid-protein complexes)

SLE

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anti-Scl 70

DNA topoisomerase 1 Ab

Diffuse skin disease, Lung disease

Systemic Sclerosis

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Centromeric Proteins A, B, C

(CENPs)

Limited Skin Disease
Ischemic Digital Loss
Pulmonary HTN

Systemic Sclerosis

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RNA Polymerase III Ab

Acute Onset
Sclerodermal Renal Crisis
Cancer

Systemic Sclerosis

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Ro / La

(SS-A / SS-B)

w/ No Association

Sjogren Syndrome

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Jo1 Ab

Histidyl aminoacyl-tRNA synthetase

Interstitial Lung Disease
Raynaud Phenomenon

Autoimmune Myositis

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Mi-2 Nuclear antigen Ab

Dermatomyositis
Skin Rash

Autoimmune Myositis

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MDA5 Ab

(Cytoplasmic receptor for Viral RNA)

Vascular Skin Lesions
Interstitial Lung Disease

Autoimmune Myositis

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TIFIγ Nuclear Protein Ab Dermatomyositis Cancer

Autoimmune Myositis

CCP Ab (Cyclic Citrullinated Peptides Various citrullinated proteins

Rheumatoid Arthritis

Rheumatoid Factor Ab

Rheumatoid Arthritis

Anti-acetylcholine receptor Ab | (1x)

Myasthenia Gravis

Anti-basement Membrane Ab | (1x)

Goodpasture Syndrome

Anti-centromere Ab | (1x)

CREST Syndrome Diffuse Systemic Sclerosis

Anti-endomysial IgA Ab | (1x)

Celiac Disease

Anti-gliadin IgA Ab | (1x)

Celiac Disease

Anti-histone Ab | (1x)

Drug-Induced Lupus

Anti-insulin Ab | (2x)

Systemic Lupus Erythematosus Type 1 Diabetes

Anti-intrinsic Factor Ab | (1x)

Pernicious Anemia

Anti-microsomal Ab | (1x)

Hashimoto Thyroiditis

Anti-Smith (Sm) Ab | (1x)

Systemic Lupus Erythematosus

Anti-SS-A Ab (Ro) (2x)

Sjogren Syndrome Systemic Lupus Erythematosus

Anti-SS-B Ab (La) (1x)

Sjogren Syndrome

Anti-Thyroglobulin Ab | (2x)

Systemic Lupus Erythematosus Hashimoto Thyroiditis

Anti-tissue Transglutaminase IgA Ab | (1x)

Celiac Disease

Anti-DNA Topoisomerase Ab | (1x)

Diffuse Systemic Sclerosis

Anti-mitochondrial Ab | (2x)

CREST Syndrome Primary Biliary Cirrhosis

Anti-myeloperoxidase Ab | (1x)

Microscopic Polyangiitis

Anti-nuclear Ab | (3x)

Systemic Lupus Erythematosus Systemic Sclerosis Dermatomyositis

Anti-proteinase 3 | (4x)

Polymyositis MCTD Primary Biliary Cirrhosis Wegener Granulomatosis

Anti-ribonucleoprotein Ab | (2x)

MCTD Systemic Lupus Erythematosis

Anti-TSH receptor Ab | (1x)

Graves Disease

(4x) Environmental Triggers for SLE

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)

(2x) Coxsackievirus a/w autoimmune disorder?

Myocarditis (B3) Type I Diabetes Mellitus (B4)

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

Allergic Encephalitis

(1x) CMV a/w autoimmune disorder?

Systemic Sclerosis

(3x) EBV a/w autoimmune disorders?

Hepatitis B Systemic Lupus Erythematosus (SLE) Rheumatoid Arthritis (RA)

(1x) Human Herpes Virus (HHV-6) Influenza A Virus Both a/w autoimmune disorder?

Multiple Sclerosis

CREST Syndrome is a Acronym for what?

* Limited Systemic Sclerosis * **C** - Calcification * **R** - Raynaud phenomenon * **E** - Esophageal dysmotility * **S** - Sclerodactyly (tapered, claw-like) * **T** - Telangiectasias (multiple punctate blood vessel dilations)

* Females 30 - 40 y.o. * Black females greatest population * CD4 T_H2 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

Systemic Sclerosis

* 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

Polymyositis

* 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**

Dermatomyositis

* 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

Mixed Connective Tissue Disease (MCTD)

* 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

Amyloidosis

AL λ Chains Amyloid derived from Immunoglobulin Light Chains

Immunocyte Dyscrasias - Systemic Amyloidosis (Primary Amyloidosis)

AA Amyloid derived from Serum Amyloid A Protein

Reactive Systemic Amyloidosis - Systemic Amyloidosis (Secondary Amyloidosis)

Aβ₂m Amyloid derived from β₂-microglobulin

Hemodialysis-associated Amyloidosis - Systemic Amyloidosis (Chronic Renal Failure)

AA Derived from Serum Amyloid Protein, Autosomal Recessive IL-1

Familial Mediterranean Fever - Hereditary Amyloidosis

ATTR Amyloid derived from Transthyretin, Autosomal Dominant

Familial Amyloidotic Neuropathies - Hereditary Peripheral and Autonomic Nerve Disorders

Amyloidosis of Elderly Patients | (70+ years old)

Systemic Senile Amyloidosis - Hereditary Predominantly Involves the Heart (Restrictive cardiomyopathy, Conduction defects)

Aβ Amyloid derived from Amyloid Precursor Protein, Chrom 21)

Senile Cerebral Amyloidosis - Localized Alzheimer disease

A Cal Amyloid derived from Calcitonin

Medullary Carcinoma of Thyroid - Endocrine Amyloid Sporadic and Familial MEN IIa and MEN IIb

AIAPP Amyloid derived from Islet Amyloid Polypeptide

Islets of Langerhans - Endocrine Amyloid Type II Diabets Mellitus

* "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

Sjogren Syndrome

* 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

Rheumatoid Arthritis

* 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

Polyarteritis Nodosa

* 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

Nonalcoholic Fatty Liver Disease (NAFLD) (Kwashiorkor)

* 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

Hemochromatosis

* 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.

Wilson Disease

* 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

α₁-Antitrypsin Deficiency (AAT)

* Multisystem noninfectious Granulomatous disease that produces chronic interstitial Fibrosis * Blacks and Non-smokers * F \> M * CD4 T_H 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

Sarcoidosis

* 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

Gout

* 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

Spondyloarthritis | (Seronegative Spondyloarthropathies)

* 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

Ankylosing Spondylitis (AS)

* 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

Reiter Syndrome

* Sausage-shaped DIP joints * Finger or Toe * Radiographs show Erosive Joint Disease * "Pencil-in-cup" deformity * Extensive nail pitting

Psoriatic Arthritis

* Most common non-gonococcal cause of Septic Arthritis * Tx: Nafcillin + Cephalosporin (3rd generation)

*Staphylococcus aureus -* Septic Arthritis

* 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

*Neisseria gonorrhoeae* - Septic Arthritis

* *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

Lyme Disease (LD) arthritis