Is CCCA an Autoimmune Disease? Symptoms and Testing

Central centrifugal cicatricial alopecia (CCCA) is generally considered a primary scarring alopecia with an inflammatory component, but it is not classified as a systemic autoimmune disease such as lupus ^[1]. Having CCCA does not, by itself, indicate that you have a systemic illness ^[1].

The Role of Inflammation in CCCA

The exact cause of CCCA is still being studied, but it is considered a multifactorial condition. This means it is likely caused by a combination of genetic susceptibility and local scalp inflammation ^[2][3]. Other environmental factors, like hair care practices, have been studied as potential contributors, though they are not considered the sole cause of the condition ^[4].

In CCCA, a type of white blood cell called a lymphocyte gathers around the hair follicle ^[1]. This inflammation can lead to permanent damage and scar-tissue formation, a process known as fibrosis ^[1]. The active inflammation and fibrosis primarily affect the scalp. Researchers have identified variations in genes that maintain hair shaft strength, which may make some people more susceptible to this localized inflammation, but routine genetic testing is not necessary ^[3].

CCCA vs. Lupus

It is common for patients diagnosed with CCCA to worry about lupus, as both conditions can cause hair loss. However, they are fundamentally different:

• CCCA typically begins with hair thinning or breakage at the top or center of the scalp and spreads outward ^[5]. It can cause local symptoms like scalp itching, burning, or tenderness, but these local symptoms do not mean you have a systemic illness.
• Systemic Lupus Erythematosus (SLE) is a systemic autoimmune disease that can affect the skin, joints, kidneys, and other organs ^[6]. While it can cause hair loss, it usually comes with other symptoms like unexplained fevers, joint pain, or rashes on the body ^[6].
• Discoid Lupus Erythematosus (DLE) is a type of lupus confined to the skin. On the scalp, it often forms sharply defined, red, scaly plaques with skin color changes and scarring ^[5][7].

While DLE and CCCA often look different, they can sometimes have overlapping clinical appearances. If your doctor is unsure whether your hair loss is caused by CCCA or discoid lupus, they may perform a scalp biopsy (taking a tiny skin sample from an active area of hair loss) ^[8]. Under a microscope, these two conditions show different patterns of cellular inflammation that, when combined with your clinical examination, help establish a firm diagnosis ^[7].

Do I Need Blood Tests?

If you have CCCA but feel otherwise healthy, routine blood tests to screen for autoimmune diseases are generally not needed solely because of your CCCA diagnosis ^[9].

A large observational study looking at the medical records of Black women found that those with CCCA had a very slight increase in lupus diagnoses (0.9%) compared to those without CCCA (0.3%) ^[9]. They also had higher rates of positive antinuclear antibody (ANA) tests (11.8% vs 2.0%) ^[9]. However, an observational association does not mean CCCA causes lupus, and a positive ANA test does not automatically mean a person has an autoimmune disease. Because the absolute risk remains very low, the study’s authors concluded that screening asymptomatic CCCA patients for other autoimmune diseases is not necessary ^[9].

Similarly, routine metabolic blood tests (such as checking for diabetes or high cholesterol) are not required as part of a CCCA-specific panel. Studies show no significant difference in rates of diabetes or metabolic syndrome between people with CCCA and those without ^[10]. You should continue your routine primary care health screenings based on your age, risk factors, and medical history.

When to Ask for Further Testing

While an automatic autoimmune panel isn’t needed for CCCA, blood testing may be appropriate if your diagnosis is uncertain, if treatments require baseline monitoring, or if you develop new symptoms outside of your scalp. Talk to your doctor if you experience:

• Persistent joint pain or swelling ^[6]
• Unexplained fevers or severe fatigue
• Rashes on your face or body ^[6]
• Persistent mouth or nose sores ^[6]
• A rash or unusual worsening of symptoms after sunlight exposure (photosensitivity) ^[6]

If you notice these or other unusual symptoms, your care team can evaluate your overall health and order the appropriate lab work ^[9][6].