302 Multisystem diseases Flashcards
What are pulmonary renal syndromes?
A life threatening condition causing renal failure with associated respiratory impairment and manifests in the form of rapidly progressive glomerulonephritis with diffuse alveolar hemorrhage (DAH)
What are some causes of Pulmonary renal syndromes?
ANCA associated vasculitis (60%)
Eg. Wegeners, Microscopic polyangitis, Churg Strauss syndrome
Goodpastures Syndrome (20%)
Others: SLE, uremic lung, pneumonia with infectious GN
What is ANCA associated vasculitis?
ANCA: Anti-Neutrophil Cytoplasmic Antibody
ANCA associated vasculitis (AAV) is an umbrella term for a group of multi-system autoimmune small vessel vasculitides
What is Wegeners disease?
An disorder that causes inflammation of the blood vessels in your nose, sinuses, throat, lungs and kidneys
A type of ANCA associated vasculitis
What is Microscopic polyangitis?
An idiopathic autoimmune disease characterized by a systemic vasculitis that predominantly affects the smaller blood vessels
A type of ANCA associated vasculitis
What is Churg Strauss syndrome?
A disorder marked by blood vessel inflammation
A type of ANCA associated vasculitis
What is Goodpasture’s syndrome?
AKA anti-glomerular basement membrane (anti-GBM) disease
It’s an autoimmune disease that affects both the kidneys and lungs by the formation of autoantibodies that attack their basement membranes
What are the manifestations of cutaneous vasculitis?
Palpable purpura, petechiae, urticaria, ulcers, livedo reticularis, and nodules
What are Petechiae?
Pinpoint non-blanching spots that measure less than 2 mm in size and affect the skin and mucous membranes
What is livedo reticularis?
A cutaneous physical sign characterized by transient or persistent, blotchy, reddish-blue to purple, net-like cyanotic pattern
It’s a manifestation of cutaneous blood flow disturbance
What is the treatment for small vessel vasculitis?
Aggressive immunosuppressive therapy using steroids, cyclophosophamide and possibly plasmapharesis
Which features diagnose SLE in kidney disorder?
Proteinuria, haematuria, and/or elevated creatinine
Name some pathogenic auto-antibodies in SLE?
Anti-dsDNA
Nucleosomes
RO
La
Sm
NMDA
Phospholipids
Alpha-actinin
C1q
Describe the classes of lupus nephritis
Class 1: minimal mesangial lupus nephritis
Class 2: mesangial lupus nephritis (mesangial hypercellarity with mesangial immune deposits on immunofluorscence)
Class 3: focal proliferative lupus nephritis affecting <50% of glomeruli
Class 4: diffuse proliferative lupus nephritis affecting >50% of glomeruli
Class 5: membranous lupus nephritis (thickening of capillary walls and global immune deposits)
Class 6: advanced sclerosis lupus nephritis (>90% glomeruli globally sclerosed)
What is cardiorenal syndrome?
Any acute or chronic problem in the heart or kidneys that could result in an acute or chronic problem of the other
What is the pathogenesis of kidney disease in chronic heart failure?
There is increase in the nuerohormonal vasoconstrictors in CHF leading to afferent arteriolar vasoconstriction causing Na and K retention and water retention
There is increase in the hormonal vasodilators as ANP and renal prostaglandins
The nuerohormonal changes lead to decrease in renal blood flow and GFR and may give a picture of pre- renal azotaemia or lead to worsening of a pre - existing renal impairment to the point that dialysis may be required
What is a hepatorenal syndrome?
Progressive oliguric renal failure either insidious or rapid
Usually occur in hospitalized patients
May be precipitated by bleeding aggressive diuresis or abdominal paracentesis
Functional renal failure with very low Na
Should differentiated from ATN and pre renal states
What is paracentesis?
The perforation of something like a cyst
What is type 1 hepatorenal syndrome?
Rapid progression (<2 weeks)
Trigger: alcohol or sepsis
If untreated, mortality within 2 weeks
What is type 2 hepatorenal syndrome?
Slow progression
Presents with diuretic resistant ascites
Better prognosis than type 1
What is the criteria for diagnosis of a hepatorenal syndrome?
Cirrhosis with ascites
Serum creatinine >133
Absence of hypovolaemia
How can the liver cirrhosis cause kidney disease?
Liver disease will cause retention of Na to the point where the urine in Na free
Very low Na in urine is a marker of disease severity and indicates poor response to diuretics
What is the treatment for hepatorenal syndrome?
Search for correctable causes
Na+ and water restriction
Dialysis is not effective unless to support and candidate for transplant
Name some disease associated with glomerular diseases?
Bacteria of all types
HIV
Hepatitis C and B
Erythrovirus (parvovirus)
What is FSGS?
Focal Segmental glomerulosclerosis
A type of glomerular disease and describes scarring (sclerosis) in your kidney
Which tumours are associated with glomerular disease?
Lymphoma
Leukaemia
Myeloma
Carcinoma
