Multisystem Disease Flashcards
What can sarcoidosis affect?
Commonest = lungs
Can also affect eyes, skin, renal or CNS symptoms in absence of respiratory symptoms
How can you recognise renal involvement in a multisystem disease?
Blood and protein detected by urine dipstick
Rise in serum creatinine
How can you use urine dipstick to differentiate between pre-renal, renal and post-renal causes of AKI?
Pre-renal = usually no blood or protein seen. Specific gravity may be high if kidneys are preserving water.
Renal = Protein: Positive (especially in glomerulonephritis).
Blood: Positive (hematuria is common in glomerulonephritis and interstitial nephritis).
Leukocytes: Positive (indicative of interstitial nephritis or infection). Specific Gravity: Typically low or normal, reflecting the kidneys’ inability to concentrate urine properly.
Post-renal = Protein: Trace to mild (due to back pressure).
Blood: Positive (hematuria can occur due to obstruction).
Leukocytes: Can be positive if there is a concurrent infection.
Nitrites: Can be positive if there is a concurrent infection. Specific Gravity: Variable, often normal.
What percentage of AKIs are caused by intrinsic renal causes?
Rare - <10% of all AKIs
What is pulmonary renal syndrome?
A clinical syndrome which is characterised by the presence of both diffuse alveolar haemorrhage and glomerulonephritis. It encompasses a group of diseases with distinctive clinical and radiological manifestations, as well as different pathophysiological processes.
When looking at pulmonary renal syndrome - what is the biggest distinction to make when trying to work out which disease is causing it?
Whether there is ANCA Abs or not
What are ANCA Abs?
Antineutrophilic Cytoplasmic Antibodies - autoantibodies - often formed after infection. They attack Ns and monocytes. Often involved with vasculitis.
What is Goodpasture’s Syndrome?
AntiGBM (glomerular basement membrane) ABs attack both glomerulus and pulmonary basement membranes -> glomerulonephritis and pulmonary haemorrhage. Often presents with acute kidney failure + coughing up blood.
What is vasculitis?
Inflammation of blood vessels - divided into large, medium and small - disease is classified by the size of the vessels affected.
What often causes large vessel vasculitis?
Giant cell arteritis
Which disease can cause medium vessel vasculitis?
Kawasaki disease
What can cause small vessel vasculitis?
ANCA associated vasculitis
What are the clinical manifestations of vasculitis?
How much protein needs to be in the urine for nephrotic syndrome?
More than 3.5g per day
How can vasculitis affect the kidney?
Can range from asymptomatic microscopic haematuria to rapidly progressing glomerulonephritis requiring dialysis
What shape is inflammation in the glomerulus in vasculitis?
Crescent shaped
What is the Rx for small vessel vasculitis?
Immunosuppression - steroids + cyclophosphamide
Maintenance - azathioprine or mycophenolate mofetil
Refractory/relapsing - rituximab
What often trigger’s Goodpastures disease?
Infection - causes Abs to be created which then attach to basement membranes
What are the signs of Goodpastures?
Cough
Dyspnoea
Haemoptysis
Glomerulonephritis
Which antibody is unique to SLE?
Anti-double stranded DNA Abs
Which antibodies will be positive in SLE?
Anti-nuclear antibodies
Anti-double stranded DNA abs
What is the normal course of SLE?
Is a relapsing remitting pattern of flares
What is the pathophysiology of SLE?
Anti-nuclear ABs - attack the cells own nucleus - generating an inflammatory response.
What can SLE present with?
Fatigue
Weight loss
Arthralgia
Myalgia
Fever
Photosensitive malar rash
Lymphadenopathy
Splenomegaly
SOB
Pleuritic chest pain
Mouth ulcers
Hair loss
Raynauds
