Pathophysiology of Vasculitis- Vanguri Flashcards
What histological features indicate that the vasculitis injury is in the acute phase?
- Presence of inflammatory cells
- Fibrinoid Necrosis*
Fibrinoid necrosis is when endothelial cells are damaged, allowing fibrin to seep in from under the intima and into the media.
The resolution of fibrinoid necrosis and the absence of inflammation indicates that we are at which phase of the vasculitis?
-About 1-2 weeks after the vasculitic injury.
The presence of dense collagenous tissue indicates what phase of the vasculitis?
What is the clinical significance?
This is the healing stage.
The nice elastic lamina of the vascular wall is no longer, it has been replaced by collagen scar. As such, the vessel will not be able to accommodate the fluctuating pressures of diastole and systole- thereby, promoting more vasculitic injury.
Describe Good Pasture’s histological and IF presentation, as well as the pathology.
- Anti-GBM disease
- Auto-antibodies against Type IV collagen
- Type II hypersensitivity
- Linear IF along basement membrane
- Cresenteric Glomerulonephritis
- Pulmonary Hemorrhage
Signs: Hematuria and Hemoptysis
Discuss the differential for IgA predominant complex deposits in the glomerulus
-Recall, Immune complex deposition shows a granular pattern on IF.
Differentials:
- IgA Nephropathy
- Young Patient
- Microscopic Hematuria after URI
- NO Crescents - Henoch Schonlein Purpura
- Kids
- Severe Purpura
- Renal Failure
- Crescenteric Glomerulonephritis
Which immune complexes are the most common in the glomerulopathies?
- IgG Immune Complexes
- Present in the case of many infections or autoimmune diseases.
SLE would show:
-IgG, IgA, IgM, C3, C1q
Infection would show:
-IgG and C3
Discuss crescentic glomerulonephritis in the setting of immune complex-negative immunofluorescence. What are the differentials?
- Termed Pauci-immune
- Associated with ANCA
Differentials:
- Wegener’s
- Churg Strauss
- Microscopic Polyangiitis
Discuss the difference between Wegener’s Polyangiitis and Goodpasteur’s
Wegener’s:
- Pauci-immune, thus, no GBM IF staining.
- -Linear IgG staining of GBM in Goodpasteur’s
- Widespread vascular and organ involvement
- -Goodpasteur’s limited to kidneys and lungs
Note: Granulomatous inflammation not always seen in Wegener’s; however, we may see Giant cells.
Describe Churg Strauss serology and Histology
- p-ANCA seen in 60% of cases
- no ANCA seen in 30%
- Eosinophilic rich infiltrate involving small vessels
- Respiratory involvement is common
- Patient with Hx of Asthma or atopy.
Discuss the large Vessel vasculitides
- Takayasu Arteritis
- Giant Cell Arteritis
Takayasu (aka Pulseless disease)
- Afflicts women in 20s and 30s
- Vasculitis of aortic arch and its branches
- Results in a measured low BP and diminished pulses
- Granulomatous infiltrates on histology
Giant Cell Arteritis
- Afflicts Elderly
- Temporal artery inflammation
- Macrophage giant cells on histology
- Unilateral headache –> can progress to Blindnes
- Rx: Anti-inflammatories as empiric treatment
Discuss the main Medium vessel arteritis conditions
- Kawasaki Disease
- Polyarteritis Nodosa
Kawasaki Disease (aka Mucocutaneous Lymph node Syndrome)
- Children
- Oral and Conjunctival lesions
- Lymphadenopathy (Cervical)
- Anti-endothelial antibodies (leads to thrombosis and MI)
Polyarteritis Nodosa
- Young adults
- Episodic Vasculitis (acute, healing, healed phases all present)
- Immune complex mediated (assoc with Hep B)
- Vague symptoms
- Does not affect capillaries thus NO Glomerulonephritis
