Summary Slides and Pics Systemic Disease I Flashcards
Granulomatosis with polyangiitis
- aka?
- Systems affected
- IF?
- KEY LABS
aka Wegner’s Granulomatosis Systems Affected:
• Sinopulmonary Renal Disease
IF:
• Pauci-Immune Glomerulonephritis (non-specific, weak staining)
KEY LABS:
• C-ANCA (PR3-ANCA) positive
Thrombotic Microangiopathy
• 2 main types?
- HUS (Hemolytic Uremic Syndrome)
- TTP (thrombotic thombocytopenia purpura)
Hemolytic Uremic Syndrome
• Triad?
- Hemolytic Anemia (Schistocytes on Blood Smear)
- Renal Dysfunction
- Thrombocytopenia
Thrombotic Thombocytopenic Purpura
• how is it different than HUS?
Fever and Neurologic Dysfunction = major differentiators
**Neurologic Dysfunction = seizures***
Systemic Lupus Erythematous
• Survival with Class IV disease
Poor Renal Survival Associated with diffuse proliferative Disease
Scleroderma (systemic sclerosis)
• KEY SIGNS
• New onset of Accerlated Arterial HTN and/or rapidly progressive oliguric renal failure
What is shown in the left and right images here?
Focal Segmental Arteritis
Transmural and Necrotizing inflammation of Medium sized vessel = PAN associated
What disease is this lesion indicative of?
• possible etiologies?
• significance of ANCA serologic marker?
Disease:
• polyarteritis nodosa
Possible Etiologies:
• Hepatitis C associated
Significance of ANCA:
• NO ANCA IN PAN
What Type of lesion is indicated here?
• why is this associated more with PAN than other diseases?
Lesion:
• INFARCT in the kidney is shown here
MEDIUM vessel vasculties then to produce KIDNEY infarcts more so than cresent formation like is seen in Small Vessel vasculitis
What disease most closely correlates with the pathology shown here?
• what is indicated by the white, red, and black arrows?
WHITE:
• Infarcts are seen
BLACK:
• Aneurysms
RED:
• Ruptured aneurysm
What does immunofluorescence in each of these images show specificity for?
- Left: C-ANCA - this was done in EtOH so density of IF stays in the cytoplasm and appears GRANULAR
- Right: P-ANCA - this was done in EtOH so density of IF stays in Near the nucleus and is more SMOOTH in appearance
What process is happening here?
• what disease is known to cause this process in a branch of the renal artery?
• what layer of this artery is being damage?
Wegner’s (Granulomatois with Polyangiitis) is a necrotizing granulomatous inflammation
Artery layer = MEDIA is damaged
What disease process is shown here?
- what c-ANCA associated disease likely caused this?
- what treatment is used for this disease?
Cresent formation has compressed out the glomerulus causing Granulomatosis with Polyangiitis.
• CYCLOphosphamide - used for immunosuppression in Wegner’s
This implys that Wegner’s has the Potential to be a RPGN, (and so can lupus)
If a patient has Renal Dysfunction and thrombocytopenia, what group of disorders should come to mind?
Thrombocytopenic Thombotic Purpura
HUS, aHUS, and other Microangiopathies
What is shown here?
• associated diseases?
Thrombotic Microangiopathies may cause thrombosis in small capillaries as shown here
• Note the Thrombi in the right center in this glomerulus
Disease: TTP, HUS
What disease other than malignant HTN would cause this appearance on staining?
See here is ONION skinning appearance that tends to appear in thrombotic microangiopathies (TTP, HUS, or DIC)
Grade and name the two forms of lupus shown below.
Left:
• Grade III - FOCAL lupus Glomerulonephritis
Right:
• Grade IV - DIFFUSE lupus GN, it its to this point is it likely also GLOBAL
What is shown here?
• what are the arrows pointing to?
LUPUS - Grade IV with Wire Loops
• top arrow points to Hyaline Capillary Thrombosis
• Bottom arrow points to WIRE LOOP
What are the key features of the lupus GN shown here?
There is Cresent formation and XS cellularity
What conditions that we know of cause this appearance in kidney vessels?
Scleroderma - would most likely cause this in an ARCUATE artery
Thombotic Microangiopathies
Malignant HTN
