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Autoimmune Disorders

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Pathology | Physician Assistant (PA) School Study Aid

Autoimmune Disorders
63 Picmonics to Learn | 2 hrs 9 mins
Anticentromere Antibodies
Autoimmune Disorders
CREST Syndrome (Limited Scleroderma)
Diffuse Scleroderma
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49 secs
Systemic Lupus Erythematosus (SLE) Diagnosis and Clinical Features
IM DAMN SHARP acronym
Immunoglobulins
Malar rash
Discoid rash
Antinuclear antibody
Mucositis
Neurologic disorders
Serositis
Hematologic disorders
Arthritis
Renal disorders
Photosensitivity
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2 mins
Systemic Lupus Erythematosus (SLE) Mechanism, Treatment and Complications
Mechanism and Characteristics
Systemic Autoimmune Disease
9:1 female to male ratio
Treatment
Glucocorticoids
NSAIDS
Hydroxychloroquine
Cyclophosphamide
Complications
Lupus nephritis
Libman-Sacks Endocarditis
Secondary Antiphospholipid Antibody Syndrome
Other
Drug-induced lupus
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2 mins
Antihistone Antibodies
Drug-induced Lupus
SHIPP Acronym
Sulfonamides
Hydralazine
Isoniazid (INH)
Phenytoin
Procainamide
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1 min
Polymyositis / Dermatomyositis Disease
Pathophysiology
Idiopathic Inflammatory Myopathy
More Common in Women
Endomysial Inflammation with CD8+ Cells (Polymyositis)
Perimysial Inflammation with CD4+ Cells (Dermatomyositis)
Signs and Symptoms (Both)
Symmetric Proximal Muscle Weakness
Dysphagia
Lung and Cardiac Involvement
Signs and Symptoms (Dermatomyositis)
Malar Rash
Shawl-like Shoulder Rash
Gottron Papules on Knuckles
Heliotrope Rash
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3 mins
Polymyositis / Dermatomyositis Diagnosis and Treatment
Diagnosis
Increased CK (CPK)
Increased Aldolase
Anti-JO-1 Antibody
Anti-SRP Antibody
Antinuclear Antibody
Abnormal EMG
Biopsy
Treatment
Corticosteroids
Methotrexate
Azathioprine
Treatment For Resistant Disease
Intravenous Immunoglobulin (IVIG)
Cyclophosphamide
Rituximab
Calcineurin Inhibitors
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2 mins
Vitiligo
Pathophysiology
Autoimmune Destruction of Melanocytes
Presentation
Hypopigmentation
Acral Areas and Around Orifices
Diagnosis
Clinical Presentation + Woods Lamp
Risk Factors
Other Autoimmune Diseases
Treatment
Glucocorticoids
Tacrolimus
Phototherapy
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2 mins
Myasthenia Gravis
Mechanism
Antibodies to Acetylcholine Receptor
Symptoms
Ptosis
Diplopia
Weakness with Muscle Use
Respiratory Death
Thymus Associated
Treatment
Acetylcholinesterase Inhibitors
Plasmapheresis
Thymectomy
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1 min
Graves' Disease Characteristics
Women 20-40 years old
Pathophysiology
Anti-TSH Receptor Antibodies
Symptoms
Ophthalmopathy
Exophthalmos
Hyperthyroidism
Goiter
Pretibial Myxedema
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1 min
Graves' Disease Labs and Treatment
Labs
Decreased TSH
Increased T3
Increased T4
Radioactive Iodine Uptake (RAIU)
Treatment
Beta blocker
Methimazole
Radioiodine Ablation
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1 min
Hashimoto's Thyroiditis
Symptoms
Hypothyroidism with Bouts of Hyperthyroidism
Enlarged, Nontender Thyroid
Mechanism
Autoimmune
Anti-Thyroid Peroxidase (TPO)
Anti-Thyroglobulin
Lab Findings
Hurthle Cells
Lymphoid Follicles
Associations
HLA-DR5 and HLA-DR3
Increased Risk Of Non-Hodgkin Lymphoma
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2 mins
Sjögren’s Syndrome
Mechanism
Autoimmune Exocrine Gland Destruction
Symptoms (Classic Triad)
Xerostomia
Xerophthalmia
Arthritis
Diagnosis
Anti SS-B (La)
Anti SS-A (Ro)
Schirmer's Test
Salivary Gland Biopsy
Treatment
Symptomatic Treatment
Complications
Lymphoma
Other
Sicca Syndrome
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4 mins
Pemphigus Vulgaris
Pathophysiology
Autoantibodies against desmosomes
Symptoms
Flaccid Bullae on Skin
Oral Mucosa Involvement
Intraepidermal Acantholysis
Fluid Loss
Infection
Nikolsky Positive
Diagnosis
Biopsy showing Autoantibodies
Treatment
IVIG and Plasmapheresis
Oral Steroids
Immunosuppressants
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2 mins
Anti-desmoglein Antibodies
Pemphigus Vulgaris
Flaccid Blisters
Skin and Oral Mucosa
Positive Nikolsky's Sign
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59 secs
Bullous Pemphigoid
Linear Deposits of IgG
Affects Hemidesmosomes
Epidermal Basement Membrane
Tense Blisters on Skin
Nikolsky Negative
Contain Eosinophils
Rarely Affect Oral Mucosa
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2 mins
Rapidly Progressive Glomerulonephritis
Pathogenesis
Nephritic Syndrome
Crescent Shape
Crescents Consist of Fibrin and C3b
With Parietal Cells, Monocytes, and Macrophages
IMMUNOFLUORESCENCE
Linear Immunofluorescence
Negative Immunofluorescence
Granular Immunofluorescence
Etiology
Goodpasture Syndrome
Granulomatosis with Polyangiitis (Formerly Wegener's)
Microscopic Polyangiitis
Prognosis
Poor Prognosis
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3 mins
Anti-glomerular Basement Membrane Antibodies
Glomerulonephritis
Goodpasture's Disease
Linear immunofluorescence
Hemoptysis
Hematuria
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1 min
Primary Sclerosing Cholangitis Mechanisms
Mechanism
Unknown Mechanism
"Onion Skin" Fibrosis
"Beading" of Bile Ducts
Symptoms
Pruritus
Hepatosplenomegaly
Jaundice
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2 mins
Primary Sclerosing Cholangitis Labs and Treatment
Labs
Increased Conjugated Bilirubin
Increased ALK-P
Increased IgM
Associated Pathologies
Ulcerative Colitis
Cholangiocarcinoma
Secondary Biliary Cirrhosis
Treatment
Liver Transplant
Stent
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2 mins
Primary Biliary Cholangitis (Primary Biliary Cirrhosis)
Mechanism
Autoimmune Destruction of Bile Ducts
Intrahepatic bile ducts
Cholestasis
Occurs in Middle-Aged Females
Signs and Symptoms
Fatigue
Pruritus
Skin Hyperpigmentation
Hepatomegaly
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3 mins
Primary Biliary Cholangitis (Primary Biliary Cirrhosis) Diagnosis and Treatment
Diagnosis
Positive Antimitochondrial Antibodies
Increased Alkaline Phosphatase (ALK-P)
Increased Cholesterol
Treatment
Ursodiol
Liver Transplant
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2 mins
Sarcoidosis Characteristics and Labs
Mechanism
Unknown Mechanism
Immune-Mediated
Noncaseating Granulomas
Vitamin D Activation
Labs
Hypercalcemia
Elevated ACE
Asteroid and Schaumann Bodies
Bronchoalveolar Lavage shows Increased CD4:CD8 Ratio
X-ray Shows Bilateral Hilar Lymphadenopathy
Treatment
Corticosteroids
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2 mins
Sarcoidosis Symptoms
Black Females
Symptoms
Asymptomatic
Uveitis
Interstitial Fibrosis
Erythema Nodosum
Hepatomegaly
Myocardial Sarcoidosis
Lupus Pernio
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1 min
Amyloidosis
Etiologies
Primary
Multiple Myeloma
Secondary
Tuberculosis (TB)
Rheumatoid Arthritis
Pathology
Congo Red Stain
Apple Green Birefringence
Complications
Nephrotic Syndrome
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2 mins
Rheumatoid Arthritis Assessment
Mechanism
Destruction of Connective Tissue and Synovial Membrane
Signs and Symptoms
Inflammation of Joints
Morning Stiffness
Nodules
Joint Deformities
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1 min
Rheumatoid Arthritis Interventions
Lifestyle Modifications
Heat and Cold Applications
Therapeutic Exercise
Balance Rest and Activity
Drug Therapy
NSAIDs
DMARDs
Glucocorticoids
Considerations
PT/OT
Reconstructive Joint Surgery
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2 mins
Gout Disease
Acute Inflammatory Monoarthritis
Epidemiology
Primarily in Males
Pathophysiology
Precipitation of Monosodium Urate Crystals
Underexcretion of Uric Acid
Overproduction of Uric Acid
Negatively Birefringent, Needle-Shaped
Symptoms
Swollen, Red, Painful Joint
Asymmetric Joint Distribution
Podagra
Tophus
After Large Meal or Alcohol
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2 mins
Gout Treatment
Acute (Acute-angle)
NSAIDs
Glucocorticoids
Colchicine
Impairs Leukocyte Chemotaxis
Chronic Gout (Crone Goat)
Inhibit Xanthine Oxidase
Allopurinol
Febuxostat
Inhibit Reabsorption of Uric Acid
Probenecid
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1 min
HLA-B27 Associations
Associations
PAIR Acronym
Psoriatic Arthritis
Ankylosing Spondylitis
Inflammatory Bowel Disease
Reactive Arthritis
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1 min
Ankylosing Spondylitis
Chronic Inflammatory Arthritis
"Bamboo Spine"
HLA-B27
Uveitis
Aortic Regurgitation
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1 min
Psoriasis
Presentation
Epidermal Hyperproliferation Keratinocytes
Defined Red Salmon Plaques
Silvery Scales
Extensor Surfaces
Bleeding when Removed
Auspitz Sign
Pruritus
Nail Changes
Histological Finding
Munro Microabscesses
Nucleated Cells in Stratum Corneum
Treatment
Retinoids
Topical Corticosteroids
Tar
Immunosuppressants
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2 mins
Temporal Arteritis
Giant Cell Arteritis
Granulomatous
Old Females
Clinical Features
Polymyalgia Rheumatica
Unilateral Headache
Jaw Claudication
Blindness
Lab Findings
Increased ESR
Normal Serum Creatine Kinase
Treatment
Steroids
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2 mins
Takayasu Arteritis
Pathophysiology
Granulomatous Thickening of Aortic Arch
Pulseless Disease
Asian Females
Signs and Symptoms
Fever
Myalgia
Night Sweats
Arthritis
Ocular Disturbances
Diagnosis
Increased ESR
Different Blood Pressures in Arms
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2 mins
Kawasaki Disease
Pathophysiology
Prevalent in Asian Children
Necrotizing Vasculitis
Signs and Symptoms
Fever
Strawberry-Red Tongue and Mucosa
Lymphadenopathy
Desquamative Skin Rash
Erythema and Edema of the Hands and Feet
Conjunctivitis
Coronary Aneurysm
Treatment
Intravenous Immunoglobulin (IVIG)
Aspirin
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2 mins
Polyarteritis Nodosa
Pathophysiology
Immune Complex-Mediated Transmural Vasculitis
Hepatitis B Association
Fibrinoid Necrosis
Signs and Symptoms
Black Stool (Melena)
Neurologic Dysfunction
Myalgia
Fever
Hypertension
Diagnosis
Vessels / Lesions in Different Stages
Renal Microaneurysms (On Arteriogram)
Spares Pulmonary Arteries (No Pulmonary Involvement)
Treatment
Corticosteroids
Cyclophosphamide
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2 mins
Buerger's Disease
Pathophysiology
Thromboangiitis Obliterans
Segmental Inflammation
Strong Association with Smoking
Signs and Symptoms
Superficial Nodular Phlebitis
Raynaud's Phenomenon
Autoamputation
Treatment
Smoking Cessation
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2 mins
Microscopic Polyangiitis
Pathophysiology
Necrotizing Small Vessel Vasculitis
Signs and Symptoms
Lungs, Kidneys, and Skin Commonly Affected
Palpable Purpura
Rapidly Progressive Cresentic Glomerulonephritis
Diagnosis
Lesions are Same Age
Non-granulomatous
Minimal Nasopharyngeal Involvement
P-ANCA
Treatment
Corticosteroids
Cyclophosphamide
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2 mins
Granulomatosis with Polyangiitis (GPA) Characteristics
Pathophysiology
Small Vessel Vasculitis
Necrotizing
Lungs and Upper Airway
Crescentic Glomerulonephritis
DIAGNOSTICS
c-ANCA
X-ray
Multiple Nodules in Lungs
Treatment
Cyclophosphamide
Corticosteroids
Rituximab
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3 mins
Granulomatosis with Polyangiitis (GPA) Symptoms
Upper Respiratory Tract
Saddle Nose
Perforation of Nasal Septum
Chronic Sinusitis
Otitis Media
Mastoiditis
Lower Respiratory Tract
Cough
Dyspnea
Hemoptysis
Kidney
Rapidly Progressive Glomerulonephritis
Hematuria
RBC Casts
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2 mins
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome)
Pathophysiology
Granulomatous Vasculitis
Signs and Symptoms
Sinusitis
Skin Lesions
Peripheral Neuropathy
Asthma
GI Disease
Heart Disease
Kidney Failure
Diagnosis
Eosinophilia
p-ANCA
Treatment
Corticosteroids
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4 mins
Henoch-Schonlein Purpura
Most Common Childhood Vasculitis
Follows Pharyngeal or Respiratory Infection
Palpable Purpura on Legs and Buttocks
Arthralgia
Intestinal Hemorrhage
Black Stool (Melena)
Associated with IgA Nephropathy
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2 mins
Erythema Nodosum
Pathophysiology
Panniculitis
Painful Inflammation
Subcutaneous Fat
Anterior Shins
Risk Factors
Coccidioidomycosis
Pregnancy or OCP Use
Crohn's Disease
Tuberculosis (TB)
Histoplasmosis
Sarcoidosis
Ulcerative Colitis
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2 mins
Ulcerative Colitis Characteristics
Autoimmune
Mucosal and Submucosal Only
Rectal Involvement
Stepwise Lesions
Friable Pseudopolyp
Crypt Abscess
Loss of Haustra
Lead Pipe Appearance
Bloody Diarrhea
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2 mins
Ulcerative Colitis Associations
Associations
Toxic Megacolon
Colorectal Carcinoma
Anterior Uveitis
Pyoderma Gangrenosum
Ankylosing Spondylitis
Primary Sclerosing Cholangitis
P-ANCA
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1 min
Crohn's Disease Characteristics
Terminal Ileum and Colon
Rectal Sparing
Transmural Inflammation
Granulomas
Skip Lesions
Cobblestone Mucosa
Fissures
Fistulas
Perianal Disease
Creeping Fat
Lymphoid Aggregates
String Sign on Barium Swallow
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2 mins
Crohn's Symptoms and Associations
Diarrhea
Malabsorption
Bile-Wasting
Pigment Gallstones
Oxalate Kidney Stones
Ankylosing Spondylitis
Erythema Nodosum
Uveitis
Migratory Polyarthritis
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2 mins
Crohn's Disease Assessment
Diarrhea
5-6 per day
Cramping Abdominal Pain
Fever
Weight Loss
Malabsorption
Anemia
Complications
Obstruction
Fistulas
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2 mins
Chronic Gastritis
Type A
Type A
Fundus and Body
Autoimmune
Autoantibodies to Parietal Cells
Pernicious Anemia
Achlorhydria
Type B
Type B
Most Common Type
Helicobacter pylori
Antrum
Increased Risk of MALT Lymphoma
Associations
HIV/AIDS
Crohn's Disease
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3 mins
Multiple Sclerosis Features and Mechanisms
Features
More Common in Women
Presents in 20's and 30's
Northern Europeans
Mechanism
Unknown mechanism, genetic factor
HLA-DRB1
Autoimmune Demyelination of CNS
White Matter of Brain and Spinal Cord
T Cell Mediated Inflammation
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2 mins
Multiple Sclerosis Symptoms and Diagnosis
Symptoms
Optic Neuritis
Internuclear Ophthalmoplegia
Scanning Speech
Urinary and Fecal Incontinence
Motor Issues (Intention Tremor, Paresis)
Numbness and Pain
Depression
Relapsing Symptoms
Diagnosis
Gold Standard = Plaques on MRI
Increased CSF Immunoglobulins (IgG)
Oligoclonal Bands
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4 mins
Multiple Sclerosis Interventions
Drug Therapy
Corticosteroids
Interferon Beta
Dimethyl Fumarate (Tecfidera)
Fingolimod (Gilenya)
Mitoxantrone (Novantrone)
Natalizumab (Tysabri)
Considerations
Increase Exercise
Identify Triggers
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2 mins
Celiac Disease Characteristics
Epidemiology
European Descent
HLA-DQ2
HLA-DQ8
Pathophysiology
Autoimmune-mediated Intolerance of Gliadin
Symptoms
Dermatitis Herpetiformis
Weight Loss
Failure to Thrive
Decreased Bone Density
Steatorrhea
Anemia
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2 mins
Celiac Disease Diagnosis and Treatment
Diagnosis
Blunting of Intestinal Villi
Lymphocytes in Lamina Propria
Anti-gliadin Antibodies (IgA)
Anti-endomysial Antibodies (IgA)
Anti-tissue Transglutaminase Antibodies (IgA)
Treatment
Gluten-Free Diet
Corticosteroids
Dapsone
Complications
Intestinal Lymphoma
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2 mins
Addison's Disease
Mechanism
Chronic 1° Adrenal Insufficiency
Atrophy
Destruction
Symptoms
Deficiency In Cortisol And Aldosterone
Skin Hyperpigmentation
Increased POMC
Hypotension
Hyperkalemia
Acidosis
Addisonian Crisis
Diagnosis
ACTH (Cosyntropin) Stimulation Test
Treatment
Exogenous Cortisol
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2 mins
Cold Agglutinin Autoimmune Hemolytic Anemia (C-AIHA)
Pathophysiology
IgM Binds to RBCs at Cold Temperatures (28-31C)
Extravascular Hemolysis
Waldenstrom's Macroglobulinemia
Mycoplasma Pneumoniae
Infectious Mononucleosis
Lymphomas and CLL
Symptoms
Acrocyanosis with Cold Exposure
Blue Fingers and Toes
Diagnosis
Positive Coombs Test
Treatment
Avoid Cold
Rituximab
+/- Fludarabine
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4 mins
Warm Agglutinin Autoimmune Hemolytic Anemia (W-AIHA)
Pathophysiology
Most Common Autoimmune Hemolytic Anemia
IgG Coats RBCs at Warm Temperatures
Fc Portion of IgG Recognized By Macrophages
Extravascular Hemolysis
Medications
Alpha Methyldopa
Chronic Lymphocytic Leukemia (CLL)
Systemic Lupus Erythematosus (SLE)
Diagnosis
Positive Coombs Test
Treatment
Corticosteroids
Splenectomy
Intravenous Immunoglobulin (IVIG)
Rituximab
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3 mins
Antiphospholipid Syndrome
Characteristics
Antiphospholipid Antibodies
Hypercoagulability
Clinical Features
Recurrent Thromboembolisms
Recurrent Miscarriages
Diagnosis
Anticardiolipin Antibodies
Anti-Beta-2-Glycoprotein Antibodies
Lupus Anticoagulant
Management
Anticoagulation
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2 mins
Polymyalgia Rheumatica
General Characteristics
Proximal Muscle Pain
Normal Muscle Strength
Morning Stiffness
Age Over 50
Associated With Temporal Arteritis
Diagnosis
Elevated ESR, CRP
Normal CK
Management
Corticosteroids
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4 mins
Eosinophilic Esophagitis
Characteristics
History of Atopic Disease
Symptoms
Dysphagia
Diagnosis
Endoscopy with Biopsy
Eosinophilic Infiltrate
Unresponsive to GERD Therapy
Interventions
Elimination Diet
Proton Pump Inhibitors (PPIs)
Topical Glucocorticoids
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2 mins
Internuclear Ophthalmoplegia
ETIOLOGY
Multiple Sclerosis
Medial Longitudinal Fasciculus
Mechanism
CN VI Nucleus
CN III Nucleus
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2 mins
Raynaud Phenomenon Characteristics and Presentation
Characteristics
Stress-induced Vasospasm
Primary Type
Primary Raynaud Phenomenon
Idiopathic
Reversible
Symmetric
Secondary Type
Secondary Raynaud Phenomenon
Autoimmune Diseases
Drugs
Severe Pain
Presentation
Ischemic Phase
Hypoxic Phase
Hyperemic Phase
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2 mins
Raynaud Phenomenon Diagnosis and Management
Diagnosis
Nailfold Capillary Microscopy
Complete Blood Count (CBC)
Antibody Testing
Management
Avoid Triggers
Smoking Cessation
Nifedipine
Topical Nitroglycerin
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2 mins
Autoimmune Hepatitis
Chronic Inflammatory Disease
Most Common in Females
Symptoms
Asymptomatic
Fatigue
Nausea
Pruritus
Diagnosis
Antinuclear antibody (ANA)
Anti-smooth Muscle
Anti-Liver/Kidney Microsomal-1 Antibodies
Increased AST/ALT
Histology
Portal and Periportal Lymphoplasmacytic Infiltrate
Treatment
Prednisone
Azathioprine
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2 mins

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