Illustration depicting ICD-10 codes for different types of Polycystic Kidney Disease.

Polycystic Kidney Disease: ICD-10 Codes and Diagnosis Insights

A hereditary condition known as polycystic kidney disease (PKD) is typified by the development of many kidney cysts. Kidney failure may result from these cysts eventually replacing a large portion of the kidneys’ natural structure. Accurate medical records and appropriate billing depend on the diagnosis and proper PKD coding. We shall examine the specifics of PKD and its associated ICD-10 codes in this post.

Understanding Polycystic Kidney Disease

PKD is one of the most common genetic disorders affecting the kidneys, with autosomal dominant and autosomal recessive forms. Autosomal dominant PKD (ADPKD) is more prevalent, typically presenting in adulthood and affecting about 1 in 500 individuals worldwide. Autosomal recessive PKD (ARPKD), on the other hand, is rarer and tends to manifest in infancy or early childhood.

ICD-10 Codes for Polycystic Kidney Disease

ICD-10 codes are alphanumeric codes used to classify diseases and medical conditions for billing, medical records, and statistical purposes. The correct coding of PKD ensures accurate documentation and facilitates proper healthcare management. Here are the relevant ICD-10 codes for PKD:

  1. Autosomal Dominant Polycystic Kidney Disease (ADPKD):
    • ICD-10 Code: Q61.2
    • Description: This code specifically denotes ADPKD, the most common form of PKD. It encompasses various manifestations and complications associated with the disease.
  2. Autosomal Recessive Polycystic Kidney Disease (ARPKD):
    • ICD-10 Code: Q61.3
    • Description: ARPKD is represented by this code, indicating the rarer form of PKD that typically presents in infancy or early childhood. It encompasses the distinct clinical features of ARPKD.
  3. Other Specified Polycystic Kidney Disease:
    • ICD-10 Code: Q61.8
    • Description: This code is used when PKD does not fit into the categories of ADPKD or ARPKD, or when there are other specific presentations or circumstances related to the disease.
  4. Polycystic Kidney Disease, Unspecified:
    • ICD-10 Code: Q61.9
    • Description: When the type or specific details of PKD are not documented or known, this code is utilized for coding purposes. It encompasses cases where the diagnosis is confirmed as PKD, but further specification is unavailable.

Clinical Manifestations and Complications

PKD is not merely characterized by the presence of kidney cysts; it can also involve various clinical manifestations and complications that impact patient management and prognosis. Some of the common clinical features and complications of PKD include:

  1. Hypertension (ICD-10 Code: I10): Hypertension is a common complication of PKD and can contribute to the progression of kidney damage and cardiovascular disease.
  2. Chronic Kidney Disease (ICD-10 Code: N18): Progressive loss of kidney function in PKD can lead to chronic kidney disease (CKD), requiring monitoring and management to slow disease progression.
  3. Renal Cysts (ICD-10 Code: N28.1): The presence of renal cysts is a hallmark of PKD, contributing to kidney enlargement and dysfunction.
  4. Hematuria (ICD-10 Code: R31.9): Blood in the urine (hematuria) can occur in PKD due to cyst rupture, kidney stones, or other factors, necessitating evaluation and management.
  5. Urinary Tract Infections (ICD-10 Code: N39.0): PKD patients are at an increased risk of urinary tract infections due to urinary stasis, cyst compression, or structural abnormalities in the urinary tract.

Management and Treatment

The management of PKD focuses on controlling symptoms, slowing disease progression, and managing complications. Treatment strategies may include:

  1. Blood Pressure Control: Antihypertensive medications are commonly prescribed to manage hypertension and reduce the risk of cardiovascular complications.
  2. Pain Management: Analgesics may be used to alleviate abdominal or flank pain associated with enlarged kidneys or cyst complications.
  3. Monitoring and Surveillance: Regular monitoring of kidney function, blood pressure, and imaging studies is essential to assess disease progression and detect complications early.
  4. Lifestyle Modifications: Dietary modifications, such as reducing sodium intake, may help manage hypertension and slow the progression of kidney disease.
  5. Dialysis and Transplantation: In advanced stages of PKD with kidney failure, dialysis or kidney transplantation may be necessary to replace lost kidney function.


Polycystic Kidney Disease is a complex genetic disorder characterized by the growth of cysts in the kidneys, leading to progressive kidney damage and various complications. Proper coding of PKD using ICD-10 codes is crucial for accurate medical documentation, billing, and statistical analysis. Understanding the specific codes and clinical manifestations associated with PKD facilitates effective management and care coordination for affected individuals. Early diagnosis, comprehensive management, and ongoing surveillance are essential for optimizing outcomes in patients with PKD.

Wasiur Rehman is fueled by a deep passion for advancing innovation in healthcare and medical research. He possesses a Bachelor's degree in Computer Science Engineering and has dedicated approximately two years to his role as a research analyst and SEO content writer. Currently, he is a valuable member of the DiseaseInfoHub team, serving as a content and research guide.

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