Overview

Fitz-Hugh-Curtis Syndrome (FHCS) is a rare disorder mainly caused by a bacterial infection from Chlamydia trachomatis (chlamydia), although it can also be caused by Neisseria gonorrhoeae (gonorrhoea). It predominantly affects people assigned female at birth (AFABs). These sexually transmitted infections (STIs) can cause pelvic inflammatory disease (PID), meaning the female reproductive system becomes inflamed. If left untreated, the bacteria can spread and reach the layer of tissue surrounding the liver and cause inflammation (perihepatitis). The most common symptoms of FHCS are a sudden pain in the upper right abdomen (just below the rib) and lower abdominal pain. Antibiotics such as doxycycline, ceftriaxone, and metronidazole are generally effective in treating this syndrome.

Pelvic inflammatory disease (PID)

The majority of FHCS cases begin as PID. Initial STIs from C.trachomatis, N.gonorrhoeae, or Mycoplasma genitalium can spread beyond the vagina or cervix and cause inflammation of the whole female reproductive system. Chlamydia is the most common STI in the UK, while gonorrhoea is the second most popular STI in the UK. These are typically transmitted by vaginal fluid or discharge from the penis. These diseases are usually spread during unprotected sex, 

Figure 1: Diagram illustrating the parts of the female reproductive system. Made by Dania Salim in BioRender.

It’s important to note that PID can occur even if neither you nor your partner have been intimate with anyone else. Symptoms can include:

• Painful sex
• Bleeding between periods and during sex
• Lower abdominal pain
• Heavy and/or painful periods
• In some cases, you may experience a fever

Fitz-Hugh-Curtis Syndrome (FHCS)

Although chlamydia and gonorrhoea are the main causes of FHCS, there have been reported cases caused by other bacterial infections. ¹ Once the initial infections begin to spread, the tissue surrounding the liver can become inflamed. This layer of tissue is also known as the liver capsule. FHCS can also affect the diaphragm and abdominal lining, due to the formation of stringy scar tissue between these organs and the liver. This condition mainly impacts AFABs aged between 15 and 30, although a few cases in people assigned male at birth (AMABs)^2,3 have been reported. 

4-14% of AFABs with PID develop FHCS, and this is even higher in adolescent AFABs, with a 27% chance. This is due to the reproductive organs not being fully developed yet, leaving them more susceptible to infection. The bacterial infections are thought to spread via:

• Blood
• The lymphatic system (part of the immune system)
• The bacteria can move from the cervix or vagina through the fallopian tubes to reach the uterus. The bacteria can then move towards the tissue surrounding the liver and other organs. This is currently the preferred hypothesis^2,4

FHCS may also be referred to as perihepatitis by a physician. If left untreated, this condition can lead to complications such as:

• Ectopic pregnancy
• Infertility as a result of PID. Due to the formation of scar tissue in the fallopian tubes,⁵ it may not be possible for eggs to become fertilised. PID can also impact the implantation of the embryo into the uterine lining⁶
• Chronic pain
• Salpingitis (inflamed fallopian tubes)
• Endometritis (inflamed uterine lining)
• An abscess (pus-filled pocket) can form in the reproductive system, which may be fatal

Symptoms

The most commonly reported symptoms are a sudden onset of sharp stomach pain in the upper right area,⁷ of which can progress into lower abdominal pain. Breathing, coughing, and movement can make the pain worse. The pain may also spread to the right shoulder. However, a lack of upper right abdominal pain doesn’t necessarily eliminate the possibility of FHCS.¹ Other symptoms include:

• Body chills
• Night sweats
• Nausea and vomiting
• Headaches
• Fever
• Bleeding between periods, and between or during sex
• Painful sex

Diagnosis

FHCS can often be mistaken for cholecystitis due to the presence of upper right abdominal pain. Your doctor may perform the following to diagnose your condition:

• STI test: to detect the presence of chlamydia or gonorrhoea. This involves obtaining a vaginal/cervical swab or a urine test
• Pregnancy test: this is to ensure that the person is not pregnant, as well as helping the doctor decide which antibiotics might be best for you
• A blood count can test for leukocytosis, which occurs when there is a high number of white blood cells in the blood. This indicates that the body is undergoing an immune response
• Computed Tomography(CT) and Magnetic Resonance Imaging(MRI) scans: this will help your doctor see if there is inflammation in your reproductive organs or surrounding the liver
• Transvaginal ultrasound: This can detect ectopic pregnancies and will show any abscesses
•  Laparoscopy is the gold standard for diagnosis. During this procedure, a cut is made in the stomach, and a small instrument with a camera attached is placed into the abdominal cavity to find the string scar tissue between the liver and abdominal wall

Treatment

Antibiotics

FHCS generally responds well to antibiotic treatment, with 75% of cases being treated successfully. You will likely be given a course of doxycycline, ceftriaxone, and metronidazole over the course of 14 days. However, you may be prescribed different antibiotics based on health factors, such as pregnancy and liver health.

Doxycycline

This prevents the production of proteins essential for the survival of bacterial cells. It can also help reduce inflammation by slowing the passage of white blood cells through the blood. This antibiotic will typically be given as a tablet, to be taken twice a day. This medication can be taken with or without food, although optimal absorption tends to be without food. Avoid taking this medication with calcium, zinc, or iron tablets as these reduce absorption. This should not be taken by pregnant people or those breastfeeding due to its effect on tooth and bone development. You should not take this antibiotic if you have liver problems. 

Side effects include:

• Diarrhoea
• Nausea and vomiting
• Skin sensitivity to the sun
• Teeth yellowing 
• Headaches

Ceftriaxone

This kills bacteria by preventing cells from making their cell wall. Normally, this cell wall provides protection and structure to bacterial cells. Once this cannot be made properly, the cellular contents can begin to leak out, resulting in the cell’s death. This antibiotic is given as a single shot. 

Let your medical practitioner know if you experience:

• Chest pain
• Body chills
• Fever
• Shortness of breath
• Sore throat

Metronidazole

Metronidazole is believed to work by preventing DNA and RNA synthesis in bacteria, which prevents their survival. This will be given as a pill to be consumed twice a day. You should avoid consuming alcohol when using this medication as it can worsen side effects. These include:

• Feeling drowsy
• Dizziness
• Urine becoming a darker colour
• Nausea and vomiting
• Headaches
• High dosages can cause convulsions

Azithromycin

This can be taken as a pill, and it prevents the bacterial cell from making proteins needed for survival. This medication is not suitable for people with liver problems, and people on some antidepressants and antipsychotics. You should not be given this antibiotic if you are taking moxifloxacin. 

Side effects of this medication include:

• Nausea and vomiting
• Diarrhoea
• Loss of appetite
• Headaches
• Feeling dizzy and fatigued

Fluoroquinolones

These include ofloxacin, levofloxacin and moxifloxacin. These antibiotics prevent bacterial cells from dividing by preventing their DNA from being copied. Depending on which is prescribed to you, it may need to be taken once or twice a day. These are normally only provided as a last resort due to their potential to cause severe side effects that can last up to years. 

You should stop taking this medication and let your doctor know immediately if you experience the following:

• Joint pain
• Anxiety or depression
• Suicidal ideation
• Changes in your smell, hearing, taste, or vision
• Swelling or pain in tendons
• Unusual pain or feelings like numbness, weakness, pins and needles

Pain management

If pain medications such as ibuprofen and paracetamol do not help your pain associated with FHCS, you may be prescribed something else. Examples include codeine and acetaminophen.

Laparoscopy

If antibiotics are unsuccessful in treating FHCS, a laparoscopy can also be performed to remove the adhesions between the liver and other organs.

Risk factors

Several factors can increase your risk of developing this condition, including:

• Having unprotected sex
• Using an IUD: within the first 2-3 weeks after IUD insertion, your risk for developing FHCS is increased
• Vaginal douching
• Having a history of STDs, particularly gonorrhoea or chlamydia
• History of PID

Prevention

You can reduce your risk of developing FHCS by:

• Using a condom when having sex until your partner has been cleared for any STDs
• Avoiding multiple sexual partners
• Avoid douching
• Get sexual health tests once a year if you’re sexually active, even if you are in a monogamous relationship 

Summary

FHCS is a rare complication of PID often caused by bacterial infections like chlamydia and gonorrhoea. If PID is left untreated, these infections may spread to the liver's outer layer, causing inflammation and sharp abdominal pain. FHCS primarily affects people AFAB aged between 15-30 years old. The condition is treatable with antibiotics such as doxycycline and ceftriaxone. Early diagnosis and treatment are crucial to prevent complications like chronic pain, infertility, and ectopic pregnancy. Practising safe sex and regular STI testing can reduce your risk of developing this condition.