Vasculitis Flashcards
HSP classification
Palpable purport with lower limb predominance plus at least one of the following:
- Diffuse abdominal pain
- Biopsy showing typical leukocytoclastic vasculitis OR proliferative glomerulonephritis with predominant IgA deposition
- Arthritis or arthralgia
- Renal involvement (any hematuria and/or proteinuria)
Indications for renal biopsy in HSP
- Impaired GFR
- Severe or persistent proteinuria
- Renal biopsy should be considered in cases of
- Acute kidney injury with worsening renal function as part of a rapidly progressing glomerulonephritis
- Nephrotic Syndrome (heavy proteinuria, hypoalbuminemia, and edema)
- Nephritis Syndrome (impaired GFR, HTN, hematuria/proteinuria)
Principle lesion found in HSP nephritis
Endocapillary proliferation with an increase in endothelial and mesangial cells
- IgA, fibrin, C3 and properdim in most involved glomeruli
- Peripheral capillary loops involved in more severe cases
Poor prognostic factors in HSP
- Mixed nephrotic nephritis syndrome within the first 6 months
- Decreased factor XIII activity
- HTN
- Renal failure at onset
- Increased number of glomeruli with crescents
- TI disease or macrophage infiltration
When do GI and renal manifestations typically occur in HSP
GI occur usually within a week
Renal usually develops within 6-8 weeks of rash but should monitor for 6 months
Lab findings in HSP
NORMAL platelet count Moderate leukocytosis Normochromic anemia May have positive stool guiac ANA/ RF negative UA abnormalities Hypoalbuminema with proteinuria Complements usually normal Ancas negative
Most common season for HSP
Winter
Classification criteria for hypersensitivity vasculitis/serum sickness
Need 3/6
- Age at onset >16
- Medication at disease onset
- Palpable purpura not related to thrombocytopenia
- Maculopapular rash
- Characteristic biopsy findings
Pathology of hypersensitivity
vasculitis
- Cellular infiltrate contains a large number of neutrophils and eosinophils
- Granulocytes present in perivascular or extravascular location
Labs associated with Hypersensitivity vasculitis
- leukocytosis
- Eosinophilia
- ESR is NORMAL
Classification criteria for PAN
1. Evidence of necrotizing vasculitis in medium or small arteries or angiographic abnormality showing aneursym, stenosis, or occlusion of a medium or small sized artery (histo or angio mandatory) plus at least 1 A. Skin involvement - Livedo - Skin nodules - Infarcts
B. Myalgia or muscle tenderness C. HTN - Systolic/diastolic blood pressure >95th percentile for height D. Peripheral neuropathy - Sensory - Motor mononeuritis multiplex E. Renal involvement - Proteinuria - Hematuria - Impaired renal function
Viral/bacterial trigger for PAN
Hepatitis B + Strep
Genetic mutation that may predispose to PAN
MEFV
Typical labs for PAN
Leukocytosis
Thrombosis
ESR/CRP
UA –> protein, hematuria
ANA and ANCA typically negative
Characteristic histopath of PAN
Fibrinoid necrosis of the walls of medium or small arteries with a marked inflammatory response within or surrounding the vessel wall
- Lesions tend to be focal and segmental
What increases risk of relapse in PAN?
Gi involvement
Symptoms of cutaneous PAN
Fever, nodular, painful non purpuric lesions with or without livedo racemosa, occurring predominantly in the lower extremities and with NO systemic involvement except for myalgias, arthralgia, and nonerosive arthritis
Skin biopsy of cPAN
necrotizing, non granulomatous small and medium sized vasculitis
Bacterial cause for cPAN
strep infection
Diagnostic Criteria for Kawasaki
Fever for more than 5 days plus 4/5 following signs CRASH Conjunctivitis Rash Adenopathy Strawberry tongue Hand and feet swelling
Acute febrile phase of KD
- Lasts 10-14 days
- Onset of fever is abrupt
- Irritability, vomiting decreased PO, diarrhea, abd pain, cough, rhinnorhea, weakness, arthralgia, arthritis, perineal desquamation in days leading up to presentation
- Over next 3-4 days, cervical LAD, conjunctivitis, changes in oral mucosa, pleomorphic rash, erythema and edema in hands and feet
- No particular order
- If untreated, clinical signs subside after about 12 days
- Myocarditis, pericarditis, abd pain, ascites and hydrops of gallbladder may occur during this phase
Subacute phase of KD
- 2-4 weeks
- May be entirely asymptomatic if given IVIG
- Desquamation of skin (digit) may be only clinically apparent residual feature
- May develop arthritis
- CAA most commonly first develop during this phase
- Irritability resolves
- Phase ends with return to normal of platelet count and ESR
Convalescent phase
- Most children asymptomatic
- Beau lines (horizontal ridging of nails) may appear
- Vessels undergo healing, remodeling and scarring
IVIG resistance in KD
- Higher CRP, LDH, bilirubin
- Hyponatremia
- ELEvated LFTs
- high percentage of bands
- platelets less than 300000
- SHort duration between fever and diagnosis
- <12 months at onset or older than typical
Incomplete KD
Seen more frequently in younger infants and older children
- Fever greater than or equal to 5 and 2 or 3 clinical criteria
- Check CRP and ESR >3/40
- Albumin <3
- Anemia for age
- Elevated ALT
- Platelets after 7 days >450k
- WBC >15
- Urine WBC >10
Pathology of KD coronaries
Systemic necrotizing vasculitis with fibrinoid necrosis
- Begins with neutrophilic vasculitis (saccular)
- Chronic vasculitis (lymphocytes, eosinophils, macrophages, IgA secretign plasma cells)– Fusiform anerurysms
- Smooth muscle cells may be converted to myofibroblasts
Markers for severe disease
significant neutropenia
thrombocytopenia
Infantile polyarteritis nodosa
onset before age 2, most commonly affects coronaries
- frequently presents with heart failure, may also have renal, GI, CNS manifestations
What is considered an abnormal coronary?
if age less than 5, >3, if 5 and up, >4
Aneursym if the diameter is 1.5x the size of the adajcent vessel
Signs of MAS in KD
Thrombocytopenia, anemia and modest elevation of the ESR
Small Aneurysm
Z score <= 2.5 to <5 (or less than 4mm)
Medium Aneurysm
Z score 5 to <10 (4-8mm)
