The Kidney in Systemic Disease I Flashcards
(51 cards)
What are the 4 vascular injury syndromes that cause kidney problems?
- ANCA-associated disease
- Thrombotic Microangiopathy
- Lupus nephritis
- Scleroderma
Other Systemic Diseases in the Kidney • Diabetic Nephropathy • Sickle Cell Nephropathy • Amyloidosis • Light Chain Disease
Other Systemic Diseases in the Kidney • Diabetic Nephropathy • Sickle Cell Nephropathy • Amyloidosis • Light Chain Disease
What are 2 viral associated systemic diseases that affect the kidneys?
- HIV-associated nephropathy
* Cryoglobulinemia
What are two general grouping of vascular disorders that cause kidney damage?
These Diseases either cause:
1. Inflammation of blood vessels as seen in vasculitides
OR
- Loss of thromboresistance as seen in the thrombotic microangiopathies
What should you be worried about when you see cresent formation on a kidney biopsy?
Rapidly Progressive Glomerulonephritis
What is the major Kidney complication of Medium Vessel Disease?
• other factors that may cause reduced kidney function?
• are these people typically ANCA positive?
RENAL INFARCTS
- Distal glomerular ischemia may cause decline in GFR
- NOT associated with glomerular inflammation with RBC casts
NO - these people typically do NOT have ANCA
What small is the major Kidney complication of Small vessel kidney diseases?
• other factors that may cause reduced kidney function?
• are these people typically ANCA positive?
Focal necrotizing lesion with CRESCENT FORMATION
- ACTIVE urinary sediment and RAPID progression of kidney failure
- YES - these people are typically ANCA postitive
Polyarteritis Nodosa (PAN) • vessels affected • ANCA presence? • Epidemiology? • Cause?
Vessels:
• PAN = MEDIUM VESSEL disease (more likely to cause renal infarct)
**ANCA is NEGATIVE in PAN
Epidemiology:
• Mostly Middle-aged or OLDER adults - peak incidence = 60s
Cause:
• Usually Ideopathic, but associated with Hepititis C.
What are some symptoms associated with PAN?
Symptoms = Systemic (b/c its a systemic disease that just happens to affect the kidneys)
• Fatigue, Weight Loss, Weakness, Fever, Arthralgias
May also see:
• Skin lesions, HTN, renal insufficiency, neurologic dysfunction, abdominal pain
PAN
• H and E descriptors?**
• Age of Lesions?
PAN H and E:
• Segmental Transmural Necrotizing Vasculitis
(so it involves only parts of the artery, but affects the full vessel circumference via fibrosis)
Age of Lesions:
• New and Old lesions may exist in the same vessel
What does Pauci-immune glomerulonephritis refer to?
• what is pauci-immune stain often associated with?
Pauci-immune GN => negative immunofluorescence studies often in the setting of CRESCENTRIC Glomerulonephritis.
Association:
• Pauci-immune GN - often associated with ANCAs (anti neutrophil antibodies)
Why is infarction associated with Polyarteritis Nodosa?
Medium Vessel Vasculites are often associated with INFARCTION
caused by: Medial and Intimal proliferation leads to arterial narrowing with Subsequent ischemia and infarction
What do you see grossly in kidneys on autopsy of someone with PAN?
- Large Aneurysms filled with clotted blood
2. Infarcts (in periphery where larger vessels are located)
What how do Kidney Aneurysms in PAN differ from true aneurysms?
• In PAN thse form as a result of erosion of inflammatory cells through the vessel wall into the adjacent parenchyma
In reality this is just erosion through a vessel by an inflammatory process
What are the two distinct kinds of Antineutrophil Cytoplasmic Antibodies?
ANCAs - 2 types
- C-ANCA aka PR3-ANCA
- P-ANCA aka MPO-ANCA
Compare and contrast the following for C-ANCA and P-ANCA:
• Location of Reactivity
• Structure Specificity
• Significance of a Positive test
C-ANCA:
Location - cytoplasmic reactivity
Structure Specificy- Proteinase 3 (serine protease)
Significance of + test: WEGENER’s GRANULOMATOSIS (95% specificity) aka granulomatosis with polyangiitis
P-ANCA:
Location - perinuclear reactivity
Structure Specificity - Lysosomal Peroxidase
Significance of + test: Not that specific
What is the appearance of C-ANCA and P-ANCA in EtOH fixed neutrophils?
C-ANCA:
• Fine Granular Appearance in the cytoplasm
P-ANCA:
• smoother appearance - perinuclear
What happens when ANCAs bind to neutrophils?
- Neutrophil (PMN activation)
* Increased contact and adhesion with endothelial cells and vascular structures
Between Goodpastures and Wegner’s, which is more associated with respiratory problems?
Goodpastures:
• More Renal
Wegner’s (granulomatosis with polyangiitis)
• more Respiratory
Granulomatosis with Polyangiitis
• Vessels affected
• Tissues mostly affected
• Markers?
Vessels - SMALL vessel disorder
Sinopulmonary Renal Syndrome (affects lungs, nose, and kidneys)
markers:
• WeCner’s - C-ANCA (PR3-ANCA) - very sensitive AND specific marker
T or F: you may see patients that have Wegner’s but are ANCA negative
True, this is the case of patients that have been treated with steroids prior to having their biopsy done
some pts. may also be positive for P-ANCA
What is Azotemia?
• does this go with Wegners?
Azotemia = high levels of nitrogen compounds in the blood e.g. Creatinine and Urea
What are you looking for on the H and E of someone’s lung biopsy with Wegner’s?
Overall:
• GEOGRAPHIC NECROSIS with loss of alveoli
Vessels:
• NECROSIS of small to medium-sized vessels (capillaries, venules, arterioles, and arteries)
Cells:
• Giant Cells and lots of infiltration of Neutrophils
T or F: the severity of necrotizing glomerulonephritis in Wegner’s correlates to the severity of clinical presentation.
True,
NOTE:
• this ranges from mild focal and segmental Glomerulonephritis in pts. with asymptomatic hematuria and normal to near-normal renal function.
• all the way to diffuse necrotizing and crescentric glomerulonephritis in pts. with acute kidney injury
