Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired disease of the blood. Evidence suggests that PNH may increase the chances of developing chronic kidney disease (CKD).

PNH is characterized by blood clots (thrombosis), destruction of red blood cells, and impaired bone marrow function. But it can also impair renal (kidney) function.

People living with PNH may have an increased risk of developing CKD compared to the general population. If you are living with PNH, you may be able to take steps to reduce your risk of CKD before it develops.

PNH is an acquired blood disease characterized by red blood cell destruction, impaired bone marrow function, blood clots, impaired renal function, and arterial and pulmonary hypertension.

The potentially fatal disease affects an estimated 15.9 people out of 1 million. The total number of people living with PNH may actually be higher due to underreporting and missed diagnoses.

Many of the symptoms associated with PNH are non-specific, such as fatigue and shortness of breath. And in some cases, you may not experience any symptoms at all.

PNH occurs due to an acquired mutation on X-linked phosphatidylinositol glycan class A (PIG-A) gene. The mutation causes a deficiency in the glycosylphosphatidylinositol (GPI) protein.

The GPI protein helps anchor other proteins to red blood cells. Of particular concern in PNH are proteins CD55 and CD59. When these cannot attach to red blood cells, it leads to a perpetual state of hemolysis — the destruction of red blood cells.

Slow damage to the kidneys over the course of months or years characterizes chronic kidney disease (CKD). As damage worsens, your kidneys can no longer filter blood as well as they used to. Kidney damage may also interfere with other important kidney functions.

Over time, CKD continues to damage the kidneys. This can lead to renal (kidney) failure. If kidney failure occurs, you will need either a kidney transplant or dialysis.

Several factors can increase your risk of developing CKD. Some of the most common risk factors include heart disease, high blood pressure, diabetes, and a family history of kidney disease.

You can take steps to help both prevent CKD and also slow the progression of kidney damage.

Some evidence suggests that if you’re living with PNH, you’re at a 6 times greater risk of developing CKD compared to the non-affected population.

A 2019 study found that over half of its study population of people living with PNH developed kidney disease. The researchers suggested that healthcare professionals should monitor people living with PNH’s kidney function regularly.

Another study from 2017 found that 45% of the people participating in the study developed renal failure. Based on these findings, the researchers recommended treatment with eculizumab in people with PNH to help improve renal function and prevent kidney failure.

Some evidence also suggests that between 8–18% of PNH-related deaths occur due to renal failure.

Some experts suggest that the buildup of iron in the kidneys due to hemolysis can contribute to acute kidney injury.

A case study from 2023 notes that PNH can cause renal dysfunction in all parts of the kidney. The study noted that microlesions and damage to the kidneys contribute to CKD in people living with PNH.

PNH increases your risk of developing CKD, but you may be able to help prevent kidney damage and reduce your risk.

The National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) recommends people at risk for developing CKD make some of the following modifications to their daily lives:

  • Limit alcohol intake.
  • Stop or never start smoking.
  • Get between 7 and 8 hours of sleep each night.
  • Maintain a moderate weight.
  • Choose nutritious foods like fresh or frozen vegetables, fresh fruits, whole grains, and low fat or fat-free dairy products.
  • Manage comorbid conditions, such as diabetes, heart disease, or high blood pressure.
  • Take steps to manage stress.

Specific to PNH, you should follow healthcare professional instructions on treatment for PNH. Modern treatments, such as eculizumab, help to prevent red blood cell destruction, which can help prevent damage to your kidneys.

The therapy is considered lifesaving and can help you live a normal life span by reducing the risk of blood clots and other complications, such as CKD.

If you do develop kidney failure or damage to your kidneys, you may need dialysis. This treatment helps to perform the filtering function of your kidneys.

You may want to speak with a nephrologist about possible kidney involvement if you are living with PNH. They may help provide more specific tips for your individual situation to help reduce your risk of CKD.

Other healthcare professionals you may want to speak to include:

  • Dietitian: This person can help you develop a meal plan and strategy to make a balanced diet.
  • Primary care professional: A primary care professional can help manage medications and treatment for high blood pressure and control cholesterol.
  • Endocrinologist: If you have diabetes, this specialist can help manage treatment for diabetes and monitor medications.

PNH may increase your risk of developing CKD and failure. If you’re living with other risk factors for CKD, you may want to discuss managing your risk of CKD with healthcare professionals who can help manage blood glucose levels, cholesterol, and high blood pressure.

You can take several steps to prevent and manage CKD. They include lifestyle changes, such as eating a balanced diet, getting enough sleep, and avoiding drinking in excess.

Managing PNH with medication can also reduce your risk of CKD. If CKD does develop, you may need additional treatments, such as dialysis.