A 47-year-old lady presented to the Emergency Department with a three-day history of rapid deterioration. She complained of feeling unwell for the last three days with extreme fatigue and reduced appetite, followed by a gradual but rapid onset of swelling in both her legs. She complained further of changes in her urine over the last few days, stating that it appeared frothy and over the last day noticed the presence of blood in her urine, but she denied the presence of urinary frequency, urgency or dysuria. She complained of feeling short of breath on exertion but not at rest and did not suffer from chest pain. She had a past medical history comprising SLE which was diagnosed 12 years ago and hypertension for which she was prescribed ramipril 10mg OD, azathioprine 50mg OD, methotrexate 20mg once per week and folic acid 5mg once per week.
Examination revealed the presence of an unwell lady with a blood pressure of 166/86 mmHg, heart rate of 102, a temperature of 37.1 Celsius and respiratory rate of 22/min. Examination of her cardiovascular system revealed the presence of gross bilateral pitting oedema in her lower limbs to the level of her mid shins, with a JVP of 3cm and normal heart and breath sounds. Examination of her gastrointestinal system was unremarkable.
Initial investigations reveal the following results. Results from DMARD monitoring bloods 2 months ago are shown in brackets.
Hb 122 g/l (126)
Platelets 242 * 109/l (248)
WBC 7.8 * 109/l (8.2)
ESR 26 mm/hr
Na+ 131 mmol/l (138)
K+ 6.2 mmol/l (5.2)
Urea 22.2 mmol/l (6.4)
Creatinine 268 µmol/l (77)
CRP 42 mg/l
Bilirubin 18 µmol/l (18)
ALP 76 u/l (82)
ALT 72 u/l (21)
Total protein 52 g/l (78)
Albumin 18 g/l (39)
Glucose 8.2 mmol/l
Chest x-ray: normal heart size and lung fields
ECG: sinus tachycardia 108bpm no acute changes
Urinalysis: blood ++, protein ++++, leuc trace, nit negative, gluc negative, ketone trace
What is the most likely diagnosis?
Berger’s nephropathy
Membraneous glomerulonephritis
Focal segmental glomerulosclerosis
Rapidly progressive glomerulonephritis
Mesangiocapillary glomerulonephritis
This lady has presented with acute nephrotic syndrome and (presumably) acute severe kidney failure. Options 2,3 and 5 are all associated with SLE and can cause a nephrotic syndrome; indeed membranous glomerulonephritis is the most common cause of nephrotic syndrome in adults. They are all associated with end stage renal failure to some degree, though this tends to be a chronic gradual decline in renal function. RPGN, also known as crescentic glomerulonephritis, also associated with focal segmental glomerulosclerosis, can present with acute nephritic or nephrotic syndrome and is associated with a fulminant renal failure. Early recognition is essential to delay renal further renal damage; this lady will require aggressive immunosuppressive treatment.