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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
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
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
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
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
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
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
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
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
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
2 mins
Nephritic Syndrome
Inflammatory
Hematuria
RBC casts
Increased Nitrogen in blood
Oliguria
Hypertension
Proteinuria < 3.5 grams per day
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
3 mins
Post Streptococcal Glomerulonephritis
Pathophysiology
Nephritic
Children
Signs and Symptoms
Edema
Cola Colored Urine
Follows Group A Streptococcal pharyngitis or Skin Infection
Diagnosis
Neutrophils
Immunofluorescence (IF) Granular
Lumpy Bumpy Appearance on Immunofluorescence (IF)
Subepithelial Immune Complex Humps
2 mins
Berger's Disease (IgA Nephropathy)
Nephritic
IgA Nephropathy
Immune Complex Deposit in Mesangium
Increased Synthesis of IgA
Most Common Cause of Nephritic Syndrome
Overlapping Features with Henoch–Schönlein Purpura
Presents with Pharyngitis, URI or Acute Gastroenteritis
1 min
Alport Syndrome
Pathophysiology
Nephritic
X-linked
Mutation in Type IV Collagen
Diagnosis
Split Basement Membrane
Basket Weave Appearance
Signs and Symptoms
Ocular Disorders
Deafness
2 mins
Nephrotic Syndrome
Signs and Symptoms
Massive Proteinuria > 3.5g per Day
Edema
Increased risk of infection
Thromboembolism
Hyperlipidemia
Fatty casts
1 min
Membranous Glomerulonephritis
Characteristics
Nephrotic
Diagnosis
LM Thickening of Capillary and Glomerular Basement Membrane (GBM)
EM Spike and Dome Appearance with Subepithelial Deposits
IF Granular
Primary
Phospholipase A2 Receptor Autoantibodies
Secondary
NSAIDS
Gold
Solid Tumors
Hepatitis B Virus (HBV)
Lupus
3 mins
Minimal Change Disease
Pathophysiology
Nephrotic
Most Common in Children
May be Triggered by Recent Infection or Immune Stimulus
Diagnosis
On Electron Microscopy (EM) See Foot Process (Podocyte) Effacement
On Light Microscopy (LM) See Normal Glomeruli
Loss of Negative Charge
Selective Loss of Albumin
Treatment
Responds to Corticosteroids
2 mins
Focal Segmental Glomerulosclerosis
Most Common Cause of Nephrotic Syndrome in Hispanic People and African Americans
Associated with Sickle Cell Disease and HIV
Associated Interferon Therapy and Heroin Abuse
Histopathology
LM Segmental Sclerosis and Hyalinosis
EM Focal Damage of Visceral Epithelial Cells
IF Negative
Signs & Symptoms
Microscopic Hematuria
Nonselective Proteinuria
Considerations
Poor Prognosis
2 mins
Membranoproliferative Glomerulonephritis
Nephritic-Nephrotic Syndrome
Type I
Subendothelial Immune Complexes
Hepatitis C Virus
Granular IF
Tram Tracks Caused by Splitting of the GBM
Ingrowth of Mesangium
Type II
Dense Deposit Disease
C3 Nephritic Factor
2 mins
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