Vasculotides Flashcards
Define Polyarteritis Nodosa
Necrotizing arteritis of medium-sized vessels
What does polyarteritis nodosa commonly involve?
Skin Peripheral nerves Mesenteric vessels Heart Brain Can affect any organ
Etiology of PolyArteritis Nodosa
Idiopathic
Hep B & C
Hairy cell leukemia
Pathogenesis of Polyarteritis Nodosa
Thickening of inflamed vessel wall leads to lumen narrowing
Reduce blood flow & thrombosis
Ischemia to involved organ
Inflammation can also lead to aneurysm formation
Signs & Symptoms of Polyarteritis Nodosa
Fatigue Weight loss Weakness Fever Arthralgias Skin lesions HTN Renal insufficiency Neurologic dysfunction Abdominal pain
Skin Manifestations of Polyarteritis Nodosa
Tender erythematous nodules Purpura Livedo reticularis Ulcers Bullous or vesicular eruption Focal or diffuse & usually on lower extremities
Renal Manifestations of Polyarteritis Nodosa
Most commonly involved Renal insufficiency & HTN Perirenal hematoma Glomerular ischemia but not inflammation or necrosis UA: minimal protein, moderate hematuria
Neurologic Manifestations of Polyarteritis Nodosa
Moto & sensory deficits
Asymmetric neuropathy
CNS involvement
GI Manifestations of Polyarteritis Nodosa
Abdominal pain: post prandial pain, weight loss, bowel infarction with perforation N/V Melena Diarrhea GI bleeding
Cardiovascular Manifestations of Polyarteritis Nodosa
CAD
HF: vasculitis of coronary arteries or uncontrolled HTN due to renal disease
MI uncommon
Musculoskeletal Manifestations of Polyarteritis Nodosa
Myalgias
Muscular weakness
Other Manifestations of Polyarteritis Nodosa
Orchitis
Breast/uterine pain
Ischemic retinopathy
Retinal detachment
Diagnosis of Polyarteritis Nodosa
H&P
Confirm with biopsy or angiography
Labs: CMP, CPK, HBV, HCV, UA, ESR
Treatment of Polyarteritis Nodosa
High dose steroids
Cyclophosphamide
Azathiprine
Methotrexate
Epidemiology of Kawasaki Disease
3-5 year old Asian or Pacific Islander males
Increased in summer & winter
Pathophysiology of Kawasaki Disease
Vasculitis due to the infiltration of vessel walls with mononuclear cells & later IgA secreting plasma cells
Can result in destruction of tunica media & aneurysm formation
Diagnostic Criteria for Kawasaki Disease
Fever >5 days without explanation Bilateral bulbar conjunctival injection Oral mucous membrane changes Peripheral extremity changes Polymorphous rash Cervical lymphadenopathy
Define Incomplete Kawasaki Disease
Only 2 of the criteria are met
Classic Presentation of Kawasaki Disease
Irritability or lethargy Vomiting alone Anorexia Cough or rhinorrhea Diarrhea, vomiting, or abdominal pain
Fever in Kawasaki Disease
Minimally responsive to antipyretics
May be intermittent
>5 days
Conjunctivitis in Kawasaki Disease
Bulbar injection
May spare limbus
Photophobia & anterior uveitis
Mucositis in Kawasaki Disease
Cracked red lips
Strawberry tongue
Mild or not occur at all
Extremity Changes in Kawasaki Disease
Early: erythema of hands & feet
Late: desquamation of hands & feet
Polymorphous Rash in Kawasaki Disease
First days of illness
Perineal erythema & desquamation followed by macular, morbilliform or targeted skin lesion of the trunk & extremities
Lymphadenopathy in Kawasaki Disease
Anterior cervical nodes
May be able to palpate a single large node
Cardiovascular Complications of Kawasaki Disease
Coronary artery aneurysms CHF & decreased EF MI Arrythmias Peripheral arterial occlusion
Evaluation of Kawasaki Disease
Labs: CBC, CMP, ESR, CRP
Echocardiogram
CXR: pulmonary edema
Treatment of Kawasaki Disease
IVIG Aspirin (QID dosing)
Result of IVIG in Kawasaki Disease
Can reduce incidence of coronary aneurysm Anti-inflammatory effect Reduce acute phase reactants, cytokines Augments T cell suppressor activity Resolves fever
Function of Aspirin in Kawasaki Disease
Antipyretic
Anti-inflammatory
Anti-platelet effects
Second Line Therapies for Kawasaki Disease
Methylprenisolone
TNF inhibitors
Another Name for Wegener’s Granulomatosis
Granulomatosis with polyangitis
Triad of Wegener’s Granulomatosis
Necrotizing granulomas of upper respiratory tract
Necrotizing granulomas of lower respiratory tract
Necrotizing glomerulonephritis & thrombosis of capillary loops
What arteries does Wegener’s Granulomatosis affect?
Small arteries
Presentation of Wegener’s Granulomatosis
Nasal congestion Sinusitis Otitis media Mastoiditis Inflammation of the gums Stridor Cough Dyspnea Hemoptysis Fever Malaise Weight loss
Other Symptoms of Wegener’s Granulomatosis
Arthritis of large joints Purpura Dysthesia Renal insufficiency Unilateral proptosis Red eye Eye inflammation Rashes/skin sores Kidney inflammation
Lab Results of Wegener’s Granulomatosis
Mild anemia & leukocytosis Elevated ESR Hematuria Blood cell casts Elevated C-ANCA
Chest CT Results in Wegener’s Granulomatosis
Infiltrates
Nodules
Masses
Cavities
Treatment of Wegener’s Granulomatosis
Corticosteroids
Cyclophosphamide
Rituximab
Henoch-Schonlein Purpura
Palpable purpura
Abdominal pain: GI bleed
Arthritis of lower extremities
Hematuria or proteinuria: glomerulonephritis
Commonly Affected Organs in Henoch-Schonlein Purpura
Skin
Joints
GI tract
Kidneys
Skin Manifestations of Henoch-Schonlein Purpura
Erythematous macular wheals
Ecchymosis
Petechiae
Palpable purpura
Joint Manifestations of Henoch-Schonlein Purpura
Transient or migratory arthritis in large joints
GI Manifestations of Henoch-Schonlein Purpura
N/V Abdominal pain Ileus GI bleed Bowel ischemia Perforation Intussusception
Kidney Manifestations of Henoch-Schonlein Purpura
Hematuria \+ protein Nephritis syndrome Renal insufficiency nephrotic syndrome
Follow Up of Henoch-Schonlein Purpura
UA & BP weekly x 2 months
UA & BP every 1-2 months for 1 year
