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Flashcards in The Kidneys in Systemic Disease Deck (39)
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1
Q

The kidney is a particular target for many systemic diseases, why?

A

The kidney receives more blood flow per unit volume than any other organ in the body

The glomeruli may filter proteins which are then reabsorbed by the tubule epithelial cells

The glomeruli may trap proteins or immune complexes

The kidney metabolises or excretes certain drugs, which may be toxic to the kidney

2
Q

what are systemic diseases that affect the kidneys?

A

Diabetes mellitus

Cardiovascular disease - Cardiac failure, Atheroembolism, Hypertension, Atherosclerosis

Infection - Sepsis, Post-infectious GN, Infective endocarditis

Inflammation in blood vessels - SLE, Vasculitis, Scleroderma and other connective tissue diseases, Cryoglobulinaemia

HUS / TTP

Myeloma

Amyloidosis

Drugs - Aminoglycosides, NSAIDs, ACE inhibitors, Radiocontrast, Penicillamine, gold

3
Q

what is shown here?

A

Diabetic Nephropathy

4
Q

Describe the Natural history of diabetic nephropathy - Development of proteinuria and decline in GFR?

A
  1. Silent sub-clinical phase, Hyperfiltration, Increased GFR
  2. Microalbuminuria [20 - 200ug/d]
  3. Clinical nephropathy [proteinuria > 0.5g/d]
  4. Established renal failure
5
Q

what is the long term risk of diabetic nephropathy in type 1 and type 2 patients?

A
  • 4% with Type 1 DM will develop nephropathy within 10 years
  • 25% with Type 1 DM will develop nephropathy within 25 years
  • 10% with Type 2 DM will have nephropathy by 5 years
  • 30% with Type 2 DM will have nephropathy by 20 years
  • 30% of those with diabetic nephropathy will progress to ESRF
  • Substantial associated increase in mortality
6
Q

What is the commonest single cause of ESRF?

A

diabetes

7
Q

Is the incidence of ESRD due to Diabetes increasing or decreasing?

A

increasing

8
Q

Diabetic nephropathy summary:

  • Diabetic nephropathy develops over many _____
  • Type I and Type II patients are ______ at risk
  • Increasing _________ is usually associated with declining GFR
  • Diabetic nephropathy is the single commonest cause of ____ leading to the need for ______ or transplantation
A

years

equally

proteinuria

ESRF

dialysis

9
Q

what is the classification of chronic kidney disease based on?

A

Classification based on kidney function - Glomerular Filtration Rate ( GFR )

10
Q

chronic kidney disease has a relationship with ____________ disease

A

cardiovascular

11
Q

what other things are common causes of renal failure in older patients?

A

Reno-vascular Disease

Atheroembolic Disease

12
Q

Systemic diseases – Case history

71 year old female

3/52 fever, anorexia, malaise, rigors

On examination temp 37.5-38 oC

BP 130/80

No cardiac murmurs

No organomegaly

Invesitgations:

Hb 101 g/l

WCC 15.4 x 109/l

Plat 608 x 109/l

ESR 105 mm/h

Na 134 mmol/l

K 3.6 mmol/l

Creatinine 195 umol/l

Urea 7.3 mmol/l

Albumin 28 g/l

Alk phos 360 u/l

Sigmoidoscopy Normal

Ba enema Normal

ERCP Normal

CT thorax/abdo Small pleural effusions

what is the intial management?

A

Treatment with antibiotics

However, no improvement, remained pyrexial

invesitgations/course:

Urinalysis - blood +++ , protein ++

pANCA positive, raised anti MPO antibodies

Renal biopsy - Focal necrotising GN

Diagnosis - Microscopic polyarteritis

Treatment - Cyclophosphamide, prednisolone

Outcome - Creatinine 120 umol/l after 3/12

13
Q

what is vasculitis?

A

Inflammatory reaction in the wall of any blood vessel

Defined by size of vessel involved

Can affect single or multiple organs

Wide spectrum of clinical presentations

14
Q

Nomenclature of vasculitis - Aorta/large artery

A

Takayasu arteritis

Giant cell arteritis

15
Q

Nomenclature of vasculitis - medium artery

A

Polyarteritis nodosa

Kawasaki disease

16
Q

Nomenclature of vasculitis - Small vessel

A

Wegener’s granulomatosis

Microscopic polyarteritis

Churg-Strauss syndrome

17
Q

What is Wegener’s granulomatosis and who does it occur in?

A

Granulomatous inflammation in respiratory tract

Focal necrotising glomerulonephritis with crescents

Slightly more common in males

Affects all age groups, most common 40-60 y

haracterized by inflammation in various tissues, including blood vessels (vasculitis), but primarily parts of the respiratory tract and the kidneys

18
Q

Where does Wegener’s granulomatosis and what is its effects?

A

Upper respiratory tract - Epistaxis, nasal deformity, sinusitis, deafness

Lower respiratory tract - Cough, dyspnoea, haemoptysis; Pulmonary haemorrhage

Kidney - glomerulonephritis

Joints - arthralgia, myalgia

Eyes - scleritis

Heart - pericarditis (inflammation of the pericardium)

Systemic - fever, weight loss, vasculitic skin rash

19
Q

what is Microscopic polyarteritis?

A

Similar clinical spectrum to Wegener’s granulomatosis

Can present with systemic disease, renal and pulmonary involvement

More commonly renal limited disease

a form of necrotizing small vessel vasculitis that most often affects venules, capillaries, arterioles, and small arteries, although it occasionally involves medium-sized arteries

20
Q

how is a diagnosis in vasculitis done?

A

Urine - Blood/protein

Renal function - Raised urea/creatinine

Biochemistry - Raised alk phos, CRP, low albumin

Haematology - Anaemia, thrombocytosis, leukocytosis

Immunology - Hyperglobulinaemia, Positive ANCA

Renal biopsy

21
Q

what is ANCA?

A

Antineutrophil cytoplasmic antibodies (ANCA) are autoantibodies directed against antigens found in the cytoplasmic granules of neutrophils and monocytes. ANCA testing is usually performed to help diagnose or exclude Wegener’s granulomatosis and microscopic polyangiitis

22
Q

what is Infective endocarditis?

A

infection of the endocardium

23
Q

what causes infective endocarditis?

A

Endocarditis: a result of bacterial (or fungal) infection on cardiac valves

Typical infections:

  • Staphylococcus aureus
  • Viridans streptococci
  • Enterococci
24
Q

How does infective endocrditis affect the kidneys?

A

Leads to glomerulonephritis ± small vessel vasculitis due to immune complex formation

25
Q

Glomerulonephritis in infective endocarditis - renal involvement is suggested by what?

A

Abnormal urea/creatinine

Haematuria, red cell casts

Reduced complement levels

Renal disease should recover when underlying infection treated

26
Q

what is a multiple myeloma?

A

A monoclonal proliferation of plasma cells producing an excess of immunoglobulins and light chains

a cancer that forms in a type of white blood cell called a plasma cell

27
Q

who is multiple myeloma common in?

A

common in eldery

28
Q

what are the clinical features of a multiple myeloma?

A

Markedly elevated ESR

Anaemia

Weight loss

Fractures

–nfections

Back Pain/Cord compression

29
Q

how is a multiple myeloma diagnosed?

A

Bone marrow aspirate >10% clonal plasma cells

Serum paraprotein ± immunoparesis

Urinary Bence-Jones protein (BJP)

Skeletal survey - lytic lesions

30
Q

how is renal failure present in a myeloma?

A

Cast nephropathy - ‘myeloma kidney’

Light chain nephropathy

Amyloidosis

Hypercalcaemia

Hyperuricaemia

31
Q

What is shown here?

A

Cast nephropathy

is the formation of plugs (urinary casts) in the kidney tubules from free immunoglobulin light chains leading to kidney failure in the context of multiple myeloma. It is the most common cause of kidney injury in myeloma

32
Q

What is shown here?

A

Light chain disease: TBM Ig deposition

Light chain deposition disease (LCDD) is a rare blood cell disease which is characterized by deposition of fragments of infection-fighting immunoglobulins, called light chains (LCs), in the body. LCs are normally cleared by the kidneys, but in LCDD, these light chain deposits damage organs and cause disease

33
Q

What is shown here?

A

Amyloid

a rare disease that occurs when an abnormal protein, called amyloid, builds up in your organs and interferes with their normal function

34
Q

When to suspect a systemic disease with renal involvement?

Is this common and what may be seen in the histry

A

Relatively rare but frequently missed

Outcome depends on prompt diagnosis and treatment

History: Fever, malaise, weight loss, arthralgia, myalgia, skin rash (vasculitic), gritty eyes, breathlessness, haemoptysis, epistaxis, haematuria, oedema

35
Q

When to suspect a systemic disease with renal involvement?

what ssigns would you suspect on the hands, face and skin?

A

Hands - Splinter haemorrhages, purpura, Raynaud’s

Face - Scleritis, uveitis, nasal cartilage deformity, retinal vasculitis, hypertensive retinopathy

Skin - Vasculitic rash, scleroderma

36
Q

When to suspect a systemic disease with renal involvement?

what CVS, chest, locomotor and CNS signs would you suspect?

A

CVS - Hypertension, murmur

Chest - Crepitations, haemoptysis

Locomotor - Joint swelling, tenderness

CNS - Stroke, encephalopathy

37
Q

When to suspect a systemic disease with renal involvement?

what initial investigations would you do?

A

Urine:

  • Blood/protein on urinalysis
  • Microscopy - red cell casts

Blood:

  • Elevated urea/creatinine
  • Raised CRP
  • Thrombocytosis, anaemia
  • Raised alkaline phosphatase
38
Q

what furthe rinvestigations may be done?

A

Blood:

ANCA (anti-MPO/anti-PR3 antibodies)

ANA, dsDNA antibodies

Complement levels C3, C4

Blood cultures

Radiology:

CXR

USS abdomen

Renal size

CT thorax

Pulmonary granulomas, interstitital disease

Echocardiography

Biopsy:

Kidney

Nasal mucosa

Lung

Skin

39
Q

In conclusion:

Systemic diseases are a rare but potentially _______ cause of renal disease

Need to consider when systemic symptoms±signs are present with abnormal urinalysis ± impaired renal function

Early treatment _________ renal function

A

treatable

preserves