QuestionSBA
QuestionChoice
Whilst hypertension requires aggressive treatment in IgA nephropathy, 5mg is a high starting dose as patients are usually started at 1.25-2.5mg and then gradually increased at two weekly intervals
32570
c
Commence ramipril 5mg once daily
QuestionChoice
High blood pressure should be treated aggressively in patients with IgA nephropathy, and the antihypertensive medication of choice is an angiotensin-converting enzyme inhibitor (ACEi) such as ramipril. They protect kidney function and may even be beneficial with normal blood pressure
32568
a
Commence ramipril 1.25mg once daily
QuestionChoice
A loop diuretic is not the first line diuretic in this instance and may lead to electrolyte imbalance
32569
b
Commence furosemide 20mg once daily
QuestionChoice
The National Institute of Clinical Excellence (NICE) states that calcium channel blocker is first line antihypertensive medication for those over the age of 55 or of African or Afro-Caribbean family origin. As this patient is young an ACEi is therefore first line
32572
e
Commence amlodipine 5mg once daily
QuestionChoice
There is some role for steroids in the treatment of IgA nephropathy, however this does not address his hypertension
32571
d
Commence prednisolone 5mg once daily
QuestionComment
pretty sure ramipril is nephrotoxic?

22755
QuestionComment
jAK spend time on the wards

23370
User
subscriber
Intravenous Prone
QuestionComment
good for GN bad for CKD as i understand it bcs it lowers pressure in glomerulus
25599
User
subscriber
NICU Serotonin
QuestionComment
In the acute setting stop, long term these are renoprotective
28822
User
subscriber
Edema Dominant
QuestionComment
bad for aki,good for ckd, 
43021
User
subscriber
Ale
User
subscriber
JAK Yeast
QuestionComment
if someone's mad at me for not knowing this as an F1 then that's their f problem
26275
QuestionComment
you'd know this as an F1 if you spent more time on the wards mate
27066
User
subscriber
Bradykinin Botox
User
subscriber
lil' aneurysm
QuestionComment
wish I could just download the BNF to my brain
44904
User
subscriber
Serpiginous Defibrillator
Concept
Chapter
# Summary

IgA nephropathy, also known as Berger disease, is the most common primary glomerulonephritis. It occurs due to deposition of immune complexes made up of immunoglobulin A (IgA) in the mesangium, which trigger glomerular inflammation. Patients typically present with macroscopic haematuria in the days following development of an upper respiratory tract infection. A significant proportion are asymptomatic and are diagnosed following an incidental finding of microscopic haematuria and proteinuria. Initial investigations include a urine dip and microscopy, as well as bloods for renal function. The gold standard diagnostic test is a renal biopsy. Management involves supportive care with blood pressure control, consideration of sodium-glucose cotransporter 2 (SGLT2) inhibitors for patients with significant proteinuria, followed by consideration of immunosuppression (e.g. steroids or an alternative such as mycophenolate mofetil). For most patients IgA nephropathy is a benign disease however approximately 40% progress to chronic renal disease.

# Definition

IgA nephropathy is a type of glomerulonephritis that occurs due to deposition of IgA in the mesangium of the glomerulus. It may lead to progressive renal impairment and chronic kidney disease. 

# Epidemiology

- IgA nephropathy is the commonest primary glomerulonephritis, with a worldwide incidence of 2.5 cases per 100,000 people per year
- However, there is wide geographic variation in prevalence; it is rare in African countries with the highest rates seen in Eastern and Pacific Asian countries
- Median age at diagnosis is approximately 40 years old
- In European and American patients, men are more commonly affected (there is an even gender split in other countries)

# Aetiology

- There is excessive generation of IgA1, often triggered by mucosal infection (e.g. upper respiratory tract or gastrointestinal)
- There is no evidence that there is a specific infectious agent responsible
- The IgA forms immune complexes that deposit in the glomerular mesangium
- This immune complexes trigger glomerular inflammation, with mesangial cell proliferation, complement activation and recruitment of macrophages, monocytes and T cells
- A small percentage of cases have a familial form of IgA nephropathy; the majority of cases are sporadic

# Signs and Symptoms

- Many patients are asymptomatic and are identified due to an incidental finding of microscopic haematuria or proteinuria (usually mild)
- Most commonly, patients present with visible haematuria
- This usually occurs 12-72 hours after an upper respiratory tract or gastrointestinal infection
- It is typically self-limiting within a few days
- Haematuria typically recurs with subsequent infections
- Loin pain (either unilateral or bilateral) may accompany episodes of haematuria

Signs include:

- Hypertension
- Oedema and frothy urine in patients presenting with nephrotic syndrome (approximately 5% of cases)

# Differential Diagnosis

- **Secondary IgA nephropathy** may occur due to a variety of other diseases that can cause renal IgA deposition - this is important as treatment in these cases should be directed at the underlying cause rather than consideration of immunosuppression
- Liver cirrhosis causes reduced clearance of IgA complexes
- Coeliac disease is another association
- HIV is associated with higher serum IgA levels
- Other infections such as hepatitis B are also associated with IgA nephropathy
- **Post-streptococcal glomerulonephritis** also presents with visible haematuria after an infection, specifically beta-haemolytic streptococci (e.g. tonsillitis secondary to Streptococcus pyogenes) - typically this is 1-3 weeks after the infection rather than a couple of days in IgA nephropathy
- **Henoch Schonlein Purpura** is a disease related to IgA nephropathy that usually occurs in children, which presents with a purpuric rash, arthralgia, haematuria and renal involvement (also with glomerular IgA deposits)
- **Lupus nephritis** may involve IgA mesangial deposition as well as other immunoglobulins and complement - this is referred to as a "full house" on renal biopsy when all of IgA, IgG, IgM, C3 and C1Q are positive

# Investigations

**Bedside tests:**

- **Urine dip** looking for blood and protein
- **Urine microscopy and culture** to rule out infection and look for dysmorphic red blood cells and casts
- **24 hour urinary protein** or a **protein:creatinine ratio** to quantify proteinuria - this is usually mild

**Blood tests:**

- **U&Es** to assess renal function
- **Immunoglobulins** may show raised serum IgA1 levels in up to 50% of patients (however this does not correlate with disease severity)
- **Complement** is usually normal
- **Liver function tests** to rule out cirrhosis
- **Full blood count** looking for anaemia and to check platelets prior to biopsy
- **Clotting screen** to screen for coagulopathy prior to biopsy

**Imaging tests:**

- **Renal ultrasound** to check kidney size - this may be normal or kidneys may be small in chronic kidney disease

**Special tests:**

- **Renal biopsy** is the diagnostic test and shows diffuse mesangial IgA immune complex deposits

# Management

**Conservative management:**

- All patients should be referred to secondary care services for confirmation of the diagnosis and ongoing management
- Lifestyle changes including regular exercise, maintaining a healthy diet and weight and smoking cessation
- Dietary salt should be restricted

**Medical management:**

- Control blood pressure with a target of < 120-130/80
- ACE inhibitors or angiotensin II receptor blockers (ARBs) are first-line for hypertension and may also be used for proteinuria > 0.5 g/day
- SGLT2 inhibitors can be added if patients with persistent haematuria despite an ACE inhibitor or ARB
- Clinical trial enrollment should be considered for patients with high risk of progression
- The next step is immunosuppression - options vary with patient ethnicity
- Hydroxychloroquine and mycophenolate mofetil are options in Chinese patients
- Systemic steroids may be required in some cases e.g. nephrotic syndrome or rapidly progressive glomerulonephritis

**Surgical management:**

- Patients with end-stage renal disease require renal replacement therapy with dialysis or transplant
- However, IgA nephropathy often recurs after transplant (up to 30% of cases)

# Prognosis

- Disease severity varies significantly
- Most patients experience persistent microscopic haematuria, although episodes of macroscopic haematuria typically become less frequent with time
- Less than 10% of patients experience complete resolution of haematuria and proteinuria
- Within 25 years of diagnosis, approximately 30% of patients progress to end-stage renal disease
- Risk factors for renal failure include impaired renal function at diagnosis, significant proteinuria and hypertension

# References

[UK Kidney Association - IgA Nephropathy](https://ukkidney.org/rare-renal/clinician/iga-nephropathy)

[Patient UK - IgA Nephropathy](https://patient.info/doctor/iga-nephropathy-bergers-disease-pro)

[IgA nephropathy in adults - treatment standard](https://academic.oup.com/ndt/article/38/11/2464/7221084)

[IgA Nephropathy: Core Curriculum in Nephrology](https://www.ajkd.org/article/S0272-6386(21)00598-9/fulltext)
305
309
IgA nephropathy
Topic
33
Nephrology
Video
Concept
309
IgA nephropathy
590
erivGUb
images/videos/nephrology.png
Quesmed Tutorial: Paediatric Nephrology
6514
Ramipril, an ACE inhibitor, is used in managing IgA nephropathy by reducing proteinuria and slowing disease progression through its effects on lowering blood pressure and decreasing intraglomerular pressure.
A 31 year old male presents to the general practice with rose coloured urine and fatigue. He reports that he had a recent upper respiratory tract infection, but otherwise has no past medical history of note. He undergoes a renal biopsy and is diagnosed with IgA nephropathy. During his follow up he is found to have hypertension on serial blood pressure measurements.

What single medication ought to be started in the first instance?
a