Table 1:

Classification of ADPKD patients by imaging

Class 1: TypicalBilateral and diffuse distribution, with mild, moderate, or severe replacement of kidney tissue by cysts, where all cysts contribute similarly to TKV.
Measure TKV (Could be done by nephrologist or radiologist in few minutes) http://www.mayo.edu/research/documents/pkd-center-adpkd-classification/doc-20094754
Estimated eGFR slope (ml/min/1.73m2 per year)Disease outcome and recommendation
MaleFemale
 Class 1 A−0.230.03Likely will not reach ESRD; reassure patient
 Class 1 B−1.33−1.13Slowly progressing disease; monitor patient
 Class 1 C−2.63−2.43Rapidly progressing disease; propose treatment
 Class 1 D−3.48−3.29Rapidly progressing disease; propose treatment
 Class 1 E−4.78−4.58Rapidly progressing disease; propose treatment
Class 2: Atypical
 Class 2 A:UnilateralDiffuse cystic involvement of one kidney causing marked kidney enlargement with a normal contralateral kidney defined by a normal kidney volume (<275 ml in men; <244 ml in women) and having no or only 1–2 cysts
SegmentalCystic disease involving only one pole of one or both kidneys and sparing the remaining kidney tissue
AsymmetricDiffuse cystic involvement of one kidney causing marked kidney enlargement with mild segmental or minimal diffuse involvement of the contralateral kidney defined by a small number of cysts (>2 but <10) and volume accounting for <30% of TKV
LopsidedBilateral distribution of kidneycysts with mild replacement of kidney tissue with atypical cysts where ≤5 cysts account for ≥50% TKV (the largest cyst diameter is used to estimate individual cyst volume)
 Class 2 B:Bilateral presentation with acquired unilateral atrophyDiffuse cystic involvement of one kidney causing moderate to severe kidney enlargement with contralateral acquired atrophy.
Bilateral presentation with bilateral kidney atrophyImpaired kidney function (serum creatinine≥1.5 mg/dl) without significant enlargement of the kidneys, defined by an average length <14.5 cm, and replacement of kidney tissue by cysts with atrophy of the parenchyma.