Previously called chronic pyelonephritis, reflux nephropathy is a form of interstitial renal disease that may be the cause of around 10 % of the CKD in UK patients who require dialysis. The aetiology is thought to be vesico-ureteric reflux (VUR) combined with ascending infection in early life, leading to renal inflammation with cortical scars and calyceal clubbing. Historically the diagnosis was made by IVP, showing a triad of small irregular kidneys with cortical scars and calyceal clubbing. Nowadays, ultrasound will pick up major anatomical abnormalities but early scars are best detected by DMSA isotope scan. Those with hypertension, proteinuria and bilateral disease are more likely to progress to ESRD than those without these risk factors and whose serum creatinine is normal. In adulthood treatment is conservative with prompt management of underlying symptomatic infection.

Renal disease is common in SLE with up to 75 % of all those with SLE developing some evidence of renal involvement during their lifetime. Six distinct pathological entities are described ranging from minimal evidence of immune deposition to more active focal or diffuse disease (+/− crescentic lesions) or advanced sclerosis. The clinical presentation of lupus nephritis invariably involves proteinuria, which may be in the nephrotic range. Other presentations include haematuria, hypertension and deteriorating renal function including RPGN. Signs of renal involvement with evidence of a high anti-DNA and low complement titre (C3/C4) are suggestive of active lupus nephritis, although a renal biopsy is required to achieve a diagnosis and plan management. Management of active disease is largely based on immunosuppression with steroids, mycophenolate +/− cyclophosphamide.

Amyloidosis is a complex group of acquired or inherited conditions characterised by extracellular deposition of an abnormal protein (amyloid fibrils) within various body tissues. Acquired amyloidosis, specifically AA (secondary) and AL (primary) amyloidosis, commonly presents with renal disease. Proteinuria is common, renal impairment less so and tubular dysfunction rare. AA amyloidosis is associated with chronic inflammatory disorders such as bronchiectasis, osteitis and chronic tuberculosis. Treatment is directed at the underlying inflammation, supplemented by best supportive CKD care. Renal prognosis is poor with patients often developing ESRF. However, in those who receive a transplant, graft survival is similar to the general population. AL amyloidosis is a plasma cell disorder, similar to myeloma, which results in an excess of abnormal immunoglobulin light chains. Chemotherapy is used to lower the light chain burden.

Medullary sponge kidney is usually a benign condition, characterised by malformed, dilated collecting ducts which result in the formation of medullary cysts of various sizes. It is thought to be a developmental abnormality, and may cluster in families. For most this condition remains asymptomatic with preserved excretory renal function. However recurrent haematuria, urinary tract infections or renal stone disease may be a presenting feature in some and, if complicated, can lead to deterioration in renal function. Plain x-ray and ultrasound often suggests nephrocalcinosis but both may be falsely negative. Intravenous pyelogram (IVP) was always the investigation of choice, showing a radial, linear or “brush like” pattern in the papillae with contrast medium in the ectatic collecting ducts seen as a “bouquet of flowers”. Nowadays CT urography may be preferred. Therapy is aimed at managing complications such as urinary tract infection or stone disease.

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