Autoimmune and Chronic Neutropenia

Jeannette | Posted on March 17, 2023 
autoimmune neutropenia

Autoimmune and chronic neutropenia are rare disorders that make it difficult for the body to fight against potentially lethal infections. Neutropenia occurs when the body’s neutrophil count falls below 1500 per microliter.

The number of neutrophils decreases due to increased neutrophil destruction in the periphery by antibodies resulting from autoimmune illnesses. These autoantibodies target a cell membrane antigen located on IgG Fc receptors. There are two types of autoimmune neutropenia: primary and secondary. Primary autoimmune disease is common in children, whereas secondary autoimmune disease commonly occurs in adults with other autoimmune diseases or blood cancers.

Depending on how severe the illness is and whether any underlying infections are present, the signs and symptoms of autoimmune neutropenia vary from person to person. Some people might not have symptoms, while others might have mouth ulcers, fever, weariness, and repeated infections.

This post discusses the low neutrophils autoimmune disease and autoimmune neutropenia treatment and management strategies.

Autoimmune Neutropenia

Neutropenia is characterized by a low number of neutrophils (type of white blood cells). It may result from various causes, such as decreased production, endothelium, tissue sequestration, or enhanced peripheral breakdown.

The immune system incorrectly recognizes neutrophils as foreign or aberrant and then destroys these cells due to this misrecognition. The immune system produces autoantibodies directed against neutrophils, and their presence can be observed in the blood. These autoantibodies bind to the neutrophils and mark them for elimination by the complement system or other immune cells. The elimination of neutrophils reduces the total number of this type of cell in the bloodstream.

Since neutrophils are an important part of the body’s defense mechanism against bacterial and fungal infections, people with neutropenia are more likely to contract infections than healthy ones.

Chronic neutropenia is defined as autoimmune neutropenia lasting more than three months and having no identifiable cause. In addition to idiopathic reasons, various autoimmune diseases can result in chronic neutropenia.

Based on its origin and pathophysiology, it is categorized as either primary or secondary. In children, the predominant form of autoimmune neutropenia is characterized by a hematologic abnormality (issues with blood cells) and hypoplasia (underdevelopment) of the bone marrow.

The manifestations of secondary autoimmune neutropenia in adults are autoimmune illnesses, primary immunological deficits, infections, hematologic malignancies, and drug exposure. It is also prevalent among individuals recently undergoing transplants and those with specific neurological diseases.

Depending on the severity of the autoimmune disease, the clinical manifestations might range from asymptomatic to life-threatening. Children are at risk for repeated infections, hematologic malignancies, and mental disorders.

Conditions Associated with Low Neutrophils, Autoimmune Disease

Autoimmune disorders develop when the body’s immune system mistakes its tissues for external intruders and attacks them. Some autoimmune diseases associated with low neutrophils include:

  • Lupus: It is a chronic autoimmune disease that affects the skin, joints, and kidneys.
  • Rheumatoid arthritis: It is a joint-affecting, chronic inflammatory illness that causes pain, swelling, and stiffness.
  • Felty’s syndrome: It is the serious consequence of rheumatoid arthritis characterized by low neutrophils, an enlarged spleen, and inflammatory nodules.
  • Sjogren’s syndrome: It is an autoimmune condition that predominantly affects glands that produce tears and saliva, resulting in dry eyes and mouth.
  • Myelodysplastic syndrome: It is a set of bone marrow illnesses that can cause neutropenia and low blood cell counts.

If you have low neutrophil or autoimmune disease, consult your healthcare practitioner to manage your condition and prevent infections.

Autoimmune Specialist

Diagnosing Low Neutrophils, Autoimmune Disease

A physical examination, blood tests, and medical history review are required for diagnosis.

  • Medical History: Your physician will inquire about your symptoms and medical history, including any previous infections or autoimmune conditions. Also, they may inquire about any medications you are on, as certain drugs can cause neutropenia.
  • Physical Examination: Your physician will conduct a physical exam to look for symptoms of infection or other health problems. Doctors may also examine your lymph nodes and spleen, as autoimmune illnesses can damage these organs.
  • Blood tests: Tests determine the number of blood cell types in the body, including neutrophils. If you suffer from autoimmune neutropenia, your neutrophil count will be below average. Also, your physician may order additional blood tests to look for symptoms of infection or inflammation.
  • Bone marrow biopsy: In certain instances, your physician may recommend a biopsy. It checks for abnormalities in blood cell formation by obtaining a bone marrow sample.

If your doctor diagnoses you with autoimmune neutropenia, they may recommend you to a hematologist or a specialist in blood diseases. Together, you can identify the underlying cause of your disease and develop a suitable treatment plan.

Autoimmune Neutropenia Treatment

The treatment depends on the underlying condition and the pathophysiologic manifestation of the disease. It aims to reduce the likelihood of infection. Following are some common autoimmune neutropenia treatment options:

  • Medication: Your doctor may prescribe corticosteroids or immunosuppressants if your neutrophil count is low and you are at risk for infection. These drugs can help suppress the immune system by decreasing the development of antibodies that kill neutrophils.
  • Growth factors: Administering growth factors like granulocyte colony-stimulating factor (G-CSF) or granulocyte-macrophage colony-stimulating factor is another treatment option. These factors increase neutrophil production in the bone marrow.
  • IVIG: If other therapies fail, your physician may give intravenous immunoglobulin (IVIG). This treatment entails obtaining an antibody transfusion from healthy donors. These antibodies can prevent infections and lessen the likelihood of consequences.

In addition to medicinal treatments, autoimmune neutropenia can be managed with lifestyle modifications. They include practicing good hygiene, avoiding contact with sick individuals, consuming a nutritious diet, obtaining sufficient rest, and avoiding activities that raise the risk of infection. Close monitoring of any infections is also necessary for management. Seeing your physician develop the optimal treatment approach for your unique condition is essential.

See Also: The Diagnosis and Treatment of Autoimmune Encephalitis


Chronic or autoimmune neutropenia can significantly impact an individual’s immune system and increase the risk of infection. Various factors, like medications, underlying autoimmune diseases, and genetic disorders, can contribute to these conditions. Early detection and management are critical for avoiding complications and improving overall health. Medication, growth factors, and intravenous immunoglobulin are some treatment options.

If you are experiencing symptoms of autoimmune or chronic neutropenia, it is critical to seek the advice of a healthcare professional to ensure an accurate diagnosis and appropriate treatment. Autoimmune specialists at Family Medicine Austin provide expert care and support to patients suffering from autoimmune illnesses. They can collaborate with you to create a personalized treatment plan that meets your needs and improves your quality of life. If you need treatment for one of these conditions, do not hesitate to schedule an appointment with one of our experts.

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