Scrofula – Causes, Symptoms, Diagnosis, and Risk factors

Scrofula is an infection in the neck lymph nodes. The bacterium Mycobacterium tuberculosis is the predominant cause of Scrofula. The mycobacterium bacteria that cause Scrofula come in many different varieties.

Breathing in air infected with Mycobacterium germs frequently results in Scrofula. From the lungs, the bacteria subsequently go on to the neck’s lymph nodes.

Lymphadenitis is an infection of the lymph nodes that often leads to cervical Tuberculosis. Most cases of extrapulmonary Tuberculosis, including Scrofula, are seen in immunocompromised people. 

Since ancient times, Scrofula has been known to infect humans. Surgery has been increasingly important in detecting and treating Scrofula in modern times.

Scrofula is distinct from Tuberculosis (TB), which involves a lung infection. Only 5% of those with Tuberculosis will develop Scrofula, making it a very uncommon disease.

Causes 

Mycobacterium tuberculosis 

The respiratory infection Mycobacterium tuberculosis is thought to affect one-fourth of the world’s population and has caused more fatalities throughout human history than any other microbe. 

The bacteria Mycobacterium tuberculosis is the predominant cause of Scrofula. Breathing in contaminated air is typically the cause.

Mycobacterium avium intracellulare 

Mycobacterium avium complex is a collection of mycobacteria that includes Mycobacterium intracellulare and Mycobacterium avium and is frequently classed together since they both infect humans. 

This also belongs to the nontuberculous mycobacteria variety. In a small number of cases, mycobacterium avium intracellulare can also lead to Scrofula.

Nontuberculosis bacteria 

Nontuberculous mycobacteria (NTM) are microbes that are typically present in soil and water in many different places of the world. 

Everyone who comes into touch with NTM will likely become infected. However, this is typically only true of those who have underlying lung conditions like bronchiectasis or COPD, a compromised immune system, or who are elderly. 

The most frequent bacterial aetiology of nontuberculosis in children. Putting contaminated objects in their mouths might cause children to become ill.

Symptoms 

Inflammation of lymph nodes  

Breathing in mycobacterium-contaminated air usually causes Scrofula. The lymph nodes in the neck are then infected by the germs after leaving the lungs. The neck will swell in certain places due to the lymph node infection. 

Although they normally don’t hurt, the infected lymph nodes will become inflamed. Usually, one side of the neck will be affected by the infection.

Fever 

Patients with Scrofula experience a chronic, growing or painless mass. In 43% of patients, systemic symptoms include fever, weight loss or malaise.

Malaise, fatigue or a general unwell feeling 

Tuberculous cervical lymphadenitis or Scrofula presents with malaise most often, a typical symptom seen in Scrofula patients.

Night sweats 

One of the common symptoms of Scrofula is excessive nighttime sweating, which frequently signifies the presence of potentially extremely high levels of infection in the body.

Weight loss 

Patients with Scrofula frequently have considerable weight loss, which is thought to be immunosuppressive and a key factor in the severity and course of the condition. Weight loss due to Scrofula is mainly brought on by unexplained reasons. 

Diagnosis 

Biopsy 

The NCBI states that the gold standards for diagnosing Scrofula are fine needle aspiration and excisional biopsy. 

A biopsy of the tissue and fluid inside the inflamed area or areas around the neck is typically used by doctors to diagnose Scrofula. Typically, a fine-needle biopsy is used to diagnose Scrofula. 

During this procedure, fluid is removed from an infected lymph node and tested in a lab for the presence of M. tuberculosis. To prevent the spread of the germs to nearby places, strict precautions are taken.

Tuberculin purified protein derivative (PPD) test 

Since its invention in the 1930s, the tuberculin skin test, which includes observing the immunological response to an injection of Purified Protein Derivative (PPD), has been the most popular method for determining Mycobacterium tuberculosis infection.

Tuberculin skin tests (TST) help identify the presence of Mycobacterium tuberculosis. 

A small amount of the tuberculin fluid is injected into the skin on the lower part of the arm to conduct the tuberculin skin test. 

A trained healthcare professional must check for a reaction on the arm of a person who received a tuberculin skin test within 48 to 72 hours of the initial visit. The presence of Mycobacterium tuberculosis in the body is confirmed if the test result is positive.

X-ray 

Chest x-rays and CT scan of the neck help diagnose Scrofula.

Treatment  

Antibiotics 

Antibiotics help Scrofula, similar to their effect on pulmonary Tuberculosis. A doctor suggests a combination of medications that will stop the infection. The treatment lasts longer. 

Antibiotic drug administration for six to nine months will be necessary. A lengthier course of antibiotics may be essential if a person has HIV or another immune system problem.

Following antibiotics are most typically suggested.

• Isoniazid 
• Rifampin 
• Ethambutol 
• Pyrazinamide

The duration of antibiotic medication can be months or even years. To minimise inflammation in afflicted lymph nodes, oral steroids may be beneficial.

It is typical for the lymph nodes to enlarge or for new inflammatory lymph nodes to emerge throughout the course of treatment. This is referred to as a paradoxical upgrading reaction. Even if this occurs, it’s crucial to continue the treatment.

When another type of mycobacteria is the source of the infection, medications like rifampin, ethambutol and clarithromycin are prescribed.

Surgery

Surgery is an option if the medications don’t provide any significant effect.

An NCBI book titled ‘Scrofula’ states that if there is no concurrent pulmonary TB and other associated extrapulmonary TB, excision and removal of the afflicted lymph nodes may be sufficient to treat Scrofula in immunocompetent patients. 

This is particularly true for immunocompetent children who have been infected with other mycobacteria. However, it is not always effective since there is a high probability of recurrence, and the development of fistulas might cause the condition to worsen. 

Surgery is advised in cases of treatment failure and discomfort brought on by lymph nodes. Children with cervical lymphadenitis due to nontuberculous mycobacteria also require surgical treatment as surgery produces better outcomes.

Risk factor 

Environment 

The risk of acquiring Scrofula is higher in regions with significant tuberculosis transmission. 

More pulmonary tuberculosis infections and Scrofula cases typically occur in developing nations. The germs that cause Scrofula can spread from person to person.

Reactivated Tuberculosis 

The bacteria that cause Tuberculosis can remain in the body for years without making a person sick. Some people’s immune systems prevent the germs from proliferating, so they never experience symptoms.

Bacteria that cause Tuberculosis can reactivate at any time. If that occurs, the patient will begin to exhibit Scrofula or Tuberculosis symptoms within a few weeks. ‌

Immune disorders 

Scrofula affects people more frequently when their immune systems are compromised. The doctor might do an HIV test for patients with this disease. Scrofula tends to affect HIV-positive individuals more frequently.

Conclusion

Scrofula is challenging to diagnose based on just clinical evidence. The majority of the symptoms are non-specific and mimic those of other infectious diseases, making diagnosis difficult. 

Due to similarities with neck swellings in various other diseases, the symptoms and physical findings are also hard. Therefore, a multidisciplinary healthcare team is greatly valued and necessary for arriving at accurate and timely diagnoses.

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