Whenever I suggest the diagnosis of tuberculosis in an individual with a swelling in the neck, I face the immediate retort- “I do not have cough or any other lung symptom for that matter. How can I suffer from tuberculosis?” Factually, tuberculosis can not only involve the non-pulmonary organs, it does so frequently, and rather with an increasing tendency in the modern era. Historically however, tuberculosis of non- pulmonary organs, such as the bones was recognized long before the lung involvement came to be known. The portraits of hunchbacks pictured by the Egyptian artists on the wall of the tombs of over 5000 years earlier, are



considered to suggest the tubercular vertebral destruction, now known as caries of the spine. Remnants of skeletal tuberculosis have been found in the Neolithic Mediterranean and Ancient Egyptian mummies. It is also said that the queen Nefertiti and her husband king Akhenaten of ancient Egypt died of tuberculosis. The presence of skeletal tuberculosis was substantiated further by the examination a well preserved Egyptian “mummy” of 3400 B.C. in whom the vertebral destruction and an associated abscess could be clearly ascertained. The mention of lung tuberculosis can be found only in the later descriptions, in the ancient Chinese, Babylonian and Indo Aryan literature of 2600-1500 BC era, about 1-2 millennia later than the skeletal tuberculosis.

Tuberculosis affects not only the humans but also the members of most other living species. Man first acquired tuberculosis from the cattle. A recent article in the premium scientific journal Nature reports an interesting observation that the transmission of tuberculosis to humans occurred from the seals, and that the history of origin of tuberculosis may be relatively recent than was previously believed. The finding has been however contested by other scientists who recognize the disease to be more ancient.

History aside, tuberculosis is unique for its ubiquitous nature and a wide variety of clinical manifestations. Tuberculosis is known as primarily a disease of the respiratory system, particularly the lungs. It is one of the few diseases which can involve almost any part of the body. Cancer is also known to affect any organ, but cancer is not a single disease. Cancers of different sites have different causes, treatments and natural course. On the other hand, the cause of tuberculosis of any organ is the same i.e. the micro-organism, Tubercle bacillus – also called the Mycobacterium tuberculosis. The treatment and natural course of tuberculosis of different organs are broadly similar with minor variations.

Tuberculosis continuous to pose a global health challenge with over 8 million new cases and about 1.3 million deaths every year. India alone accounts for about one fourth of the global burden. Over 15 percent of patients of tuberculosis suffer from disease of the organs other than the lungs i.e. the extra pulmonary tuberculosis. This type of tuberculosis is important for several reasons: It is a poorly recognized clinical entity; is difficult to diagnose and treat, and carries a significant morbidity and mortality.

After the lungs, the lymph nodes constitute the most common sites for tuberculosis. Lymph node

tuberculosis, known as the King’s Evil, scrofula or struma was widely recognized during the Middle

Ages in Europe where the royality touch was believed to cure the disease. King Henry IV of France is

said to have touched as many as 1,500 individuals at a time while Charles II of England is said to have cured more than 90,000 victims between 1660 and 1682. Lymph nodes comprise of small nodular glands of lymphatic tissue present in the superficial regions in the neck, axillae, elbows and groins; and deeper region in thorax, abdomen and pelvis. While the superficial nodes when involved with tuberculosis, are either visible or palpable; the deeper lymph nodes are not easily detected. The enlarged glands may press upon or erode the surrounding structures, resulting in the onset of different clinical symptoms and signs. The superficial gland enlargement may appear innocuous, but highly demoralizing, particularly in case of younger individuals. I am aware of a case when an otherwise well planned marriage broke down because of the presence of an enlarged lymph node in the neck of the girl.

Pleural tuberculosis is the second most common extra pulmonary site of tuberculosis. It results in accumulation of fluid in the pleural cavity surrounding the lungs, causing lung compression and breathlessness. Fluid can similarly accumulate in the pericardial cavity around the heart and the peritoneal cavity in the abdomen. The diagnosis is these cases is made by the examination of the fluid obtained by aspiration.

Tuberculosis of brain and its covering membranes, the meninges is a serious form of tuberculosis especially common in children. Delayed diagnosis can result in marked disability complications such as mental retardation, paralyses, blindness, deafness, or sometimes death. Tuberculosis of spine may similarly prove to be serious and disabling. The cold abscess formation and vertebral destruction may result in spinal cord compression and cause paraplegia (paralysis of both lower limbs), and sometimes quadriplegia (paralysis below the neck), depending upon the site of the lesion.

Almost any bone other than the spinal vertebrae can also be involved. Tuberculosis of the intestinal tract may result in intestinal obstruction while genitourinary tuberculosis can cause infertility, blood in the urine or urinary obstruction. Other sites which can sometimes be affected include the heart, the skin, the mucus membranes, the throat and the eyes. Importantly, the organ involvement also determines the disease-severity and type of residual damage which tuberculosis can leave even after healing.

Tuberculosis of the non-pulmonary organs is difficult to diagnose for several reasons, most importantly because of the lack of awareness and disease-suspicion. The disease manifestations are highly variable to conform to a specific pattern. It is generally late when the disease is first suspected. The Revised National Tuberculosis Control Programme of Government of India advocates sputum examination for diagnosis of pulmonary tuberculosis if the patient complains of cough that lasts for over two week duration. There are no definite guidelines to suspect and diagnose extra pulmonary tuberculosis. Therefore, the disease continues to advance without being checked in time.

It is also difficult to establish the diagnosis because of the lack of ease to obtain the appropriate specimens for testing. Unlike pulmonary tuberculosis where sputum examination and chest x-ray are the easy to do tests, most of the extra pulmonary organs require invasive-investigations and biopsies for diagnosis. Such tests are difficult for patients to undergo and carry some inherent risks, even if small. Moreover, the tests cannot be frequently repeated. There are other problems related to medical decisions, for example when to start the treatment, how to monitor the progress and decide the end-point. It is also very difficult to decide about the disease-persistence, recurrence or development of drug resistance (etc). A large number of decisions are essentially empiric based on soft criteria rather than hard scientific evidence.

The broader principles of treatment of tuberculosis of non-pulmonary organs remain the same as for the treatment of the lungs. There are a few controversies among different subject experts, for example about the duration of treatment. As per the Revised National Tuberculosis Control Programme guidelines, the prolongation of therapy, but for spinal, neurological and cardiac tuberculosis, does not provide any additional benefit. The ortho-pedicians, neurologist and cardiologists generally tend to give treatment for longer periods.

There is a general increase in the number of patients with extra pulmonary tuberculosis. Presence of other comorbid conditions such as diabetes, and other immuno-suppressive conditions, particularly the Human Immunodeficiency Virus (HIV) infection are partly responsible for this increase. Unfortunately the consequences of non-pulmonary forms of tuberculosis are more fearsome. Thus, there is a greater need to be cautious and careful.

Origin of tuberculosis

In their attempt to look into the history of tuberculosis, an international group of archaeologists and geneticists searched for tuberculosis DNA in 68 sets of remains of bone samples in 2012. The scientists could recover the genetic material in three 1000 year- old mummies from Chiribaya culture of southern Peru. The most closely related DNA belonged to the tuberculosis strains found only in seals. Possibly the hunters picked up tuberculosis from seals. The fossil evidence however suggests that tuberculosis is far older.

Burden of extra-pulmonary tuberculosis

Nearly one-third of the world population (app. 2 billion) is infected with tuberculous bacilli while over 8 million develop tuberculosis every year. The 2012 global figures released by the World Health Organization suggested an estimated 8.6 million new cases and 1.3 million TB deaths. India alone accounted for 26% of new cases while China contributed about 12 percent. On the other hand, the Western countries account for an incidence rate of less than 10 cases per 100000 population. About 1.2 million people suffered from tuberculosis of organs other than the lungs. Extra-pulmonary TB remains difficult to diagnose and treat in most situations.

From Louvre museum, Paris

The glorious Queen Nefertiti and King Akhenaten of ancient Egypt (ca.1370 BC- ca.1330 BC)

are believed to have died of tuberculosis.


Dr Surinder K. Jindal, MD, FCCP, FAMS, FNCCP

(Ex-Professor & Head, Department of Pulmonary Medicine

Postgrad Instt of Med Edu & Res, Chandigarh, India)

Medical Director, Jindal Clinics, SCO 21, Dakshin Marg, Sector 20 D,

Near Guru Ravi Das Bhawan, Chandigarh, India 160020.

Email: dr.skjindal@gmail.com

Website: jindalchest.com

Ph. Clincis: +91 172 4911000, Res. +91 172 2712030/ 31