Pneumocystis Pneumonia

Pneumocystis pneumonia (PCP or PJP) is a lung infection you get from the fungus Pneumocystis jirovecii. It almost exclusively affects people with weakened (compromised) immune systems, including people who have:

• HIV/AIDS
• Certain types of cancer (usually lymphomas and leukemias)
• Received a stem cell, organ or bone marrow transplant
• Chronic lung conditions
• Autoimmune disorders
• Used high-dose corticosteroids over a long period of time

If you think you have Pneumocystis pneumonia and have a compromised immune system, seek immediate medical attention. PCP can be fatal if left untreated.

Pneumocystis jirovecii pneumonia used to be called Pneumocystis carinii pneumonia, which is why it’s abbreviated PCP. While it can cause serious illness in people with compromised immune systems, it’s rare in people with healthy immune systems.

What are the signs and symptoms of Pneumocystis pneumonia?

Symptoms of Pneumocystis pneumonia include:

• Fever
• Chills
• Cough
• Shortness of breath (dyspnea)
• Headache
• Fatigue
• Feeling of chest tightness or chest pain with breathing
• Bluish skin, lips or nails (cyanosis)

In most people, symptoms come on quickly, over only a few days. In people with HIV, symptoms can come on slowly, over a few weeks.

What causes Pneumocystis pneumonia?

The fungus Pneumocystis jirovecii (P. jirovecii) causes PCP. Studies suggest that — unlike many types of fungi — P. jirovecii only lives in humans, rather than in the environment around us.

How do you get Pneumocystis pneumonia?

You get Pneumocystis pneumonia from other people. P. jirovecii is transmitted from one person to another through the air when someone coughs, sneezes or talks. Most of us are exposed to P. jirovecii in our lives — usually before age 4 — but don’t have symptoms or have mild symptoms. You can spread PCP to people who have compromised immune systems, even if you have no symptoms.

What are the risk factors?

Most people who get PCP have a weakened immune system. Specifically, it most commonly affects people who have issues with T cell function. T cells are a specialized part of your immune system that help you recognize and fight infections.

Health conditions that put you at a higher risk for PCP include:

• HIV
• Cancer, especially blood cancers like leukemia and lymphoma
• Receiving a stem cell, solid organ or bone marrow transplant
• Severe combined immunodeficiency (SCID)

About 30-40% of PCP cases are in people with HIV. It’s one of the most common AIDS-defining illnesses. Your risk is higher if you don’t take medications to manage HIV (antiretroviral therapy).

How is Pneumocystis pneumonia diagnosed?

A healthcare provider will diagnose PCP by looking for signs of P. jirovecii in your mucus or fluids from your lungs. To get a sample to test, they might use:

• A sputum test: You cough and then spit into a container to get a sample.
• Bronchoalveolar lavage: During a bronchoscopy procedure, your provider uses a solution to “wash” your airways and get a fluid sample.

Your provider can also use a chest X-ray or CT scan to take pictures of your lungs and look for signs of pneumonia.

How is Pneumocystis pneumonia treated?

Healthcare providers treat Pneumocystis pneumonia with high doses of the antibiotic trimethoprim-sulfamethoxazole (TMP-SMX). Antibiotics are effective in treating certain fungal infections. Other treatments include:

• Atovaquone
• Pentamidine
• Dapsone
• Clindamycin
• Primaquine

You might need a ventilator to help you breathe. If your lungs are working but you’re not getting enough oxygen, your provider might give you extra oxygen through a tube in your nose or mask on your face. Your provider may also give you corticosteroids to decrease inflammation in your lungs.

When should I see my healthcare provider?

If you have HIV or another condition that weakens your immune system, talk to your provider about preventing infections. See a provider right away if you have a weakened immune system and you have symptoms of PCP or any other infection.

When should I go to the ER?

Go to the emergency room if you have symptoms of a severe illness, including:

• High fever (over 103 degrees Fahrenheit/40 degrees Celsius)
• Difficulty breathing
• Persistent cough
• Chest pain
• Bluish skin, lips or nails
• Confusion
• Seizures

If you have a weakened immune system, ask your provider if you should go to the ER sooner than what’s listed here. You may need emergency care right away or with less severe symptoms.

Can you recover from Pneumocystis pneumonia?

Pneumocystis pneumonia can be cured with medication, but you need to start treatment as soon as possible. Because only people with weakened immune systems get sick with PCP, it can quickly make you seriously ill. Your provider may start treatment before all of your test results come back. Pneumocystis pneumonia is almost always fatal if left untreated.

How long does it take to recover?

How long it takes to recover from PCP depends on how severe your case is. You may need to be treated in the hospital. You’ll need to take medication for three weeks, but it may take longer to feel completely better.

What is the survival rate of Pneumocystis pneumonia?

It’s hard for experts to estimate a Pneumocystis pneumonia survival rate. In general, mortality rates for PCP are higher in people with compromised immune systems who don’t have HIV than those with HIV.

Can Pneumocystis pneumonia be prevented?

Since most people don’t have symptoms of a Pneumocystis infection, it can be hard to avoid being exposed. Having good handwashing habits, avoiding other people when you’re sick, and covering your mouth and nose when you cough or sneeze can help prevent the spread of infectious diseases.

If you smoke, your provider will recommend that you quit. Smoking can increase the risk of Pneumocystis infection and make it more likely you’ll get severely sick.

Your healthcare provider might prescribe medication to help prevent a Pneumocystis infection if you:

• Have HIV/AIDS and a CD4 count of 200 or fewer cells per cubic millimeter
• Regularly take medications that suppress your immune system (for example, if you take high doses of daily corticosteroids for a month or longer)
• Have had an organ or stem cell transplant within the past year
• Have another condition that compromises your immune system, including SCID, hypogammaglobulinemia and graft vs. host disease
• Have had Pneumocystis pneumonia before

Providers prescribe the same medications to both prevent and treat PCP.

What is the difference between pneumonia and Pneumocystis pneumonia?

Anyone can get the most common kinds of pneumonia from viruses, bacteria and fungi. But only the fungus Pneumocystis jirovecii can cause Pneumocystis pneumonia. Most people who get Pneumocystis pneumonia have compromised immune systems.