Sarcoidosis

Sarcoidosis

Sarcoidosis is a relatively uncommon disease which in India, has been more commonly diagnosed in the last few years.  Unlike diseases such as asthma, tuberculosis, pneumonias and lung cancer, sarcoidosis is rather an unfamiliar term amongst patients.  It is somewhat difficult to understand and appreciate the nuances of sarcoidosis.

A patient with sarcoidosis may present with varied manifestations.  Commonly, there are symptoms of low grade fever, weakness, malaise, joint pains, dry cough and/or breathlessness.  Sometimes, the patient is entirely asymptomatic and the diagnosis is made incidentally on chest x-ray examination.  While lungs are the most frequently involved organs, skin, eyes, liver, spleen and nervous system are the other common sites of disease.  Almost any part of the body can be involved in sarcoidosis.

The exact cause of sarcoidosis is not known.  This is quite enigmatic for both the patient and the doctor to accept a relatively indefinable disease.  It is an immunological disorder but the exact aberrations are not known.  There are a number of clinical, radiological and histopathological similarities with tuberculosis.  But tuberculosis and sarcoidosis are two distinct diseases with entirely different treatments.

Sarcoidosis is generally suspected on clinical and radiological criteria.  The diagnosis is confirmed on histopathological findings of biopsies from the involved organs, for example the lungs, lymph nodes or liver (etc.).  Bronchoscopy, and often endo-bronchial ultrasound (EBUS) guided fine needle aspirations are required to obtain biopsy material from the lungs and the lymph nodes.  It is important to confirm the diagnosis in view of the treatment issues involving the use of corticosteroids, generally for prolonged periods.  It is also important to exclude other diseases such as tuberculosis and lymphomas.

Sarcoidosis is a treatable disease, but recurrences may occur.  Strict monitoring is required for side-effects of drugs such as the corticosteroids, during the treatment.  The response to the treatment is fairly good.  Recurrence of disease requires treatment with alternate drugs which may not be as effective as the corticosteroids.  

Sarcoidosis involving the heart and the nervous system is considered as more serious.  In chronic cases, sarcoidosis may result in diffuse lung fibrosis and respiratory disability.  These cases respond poorly to standard modes of therapy.  Overall prognosis of sarcoidosis is generally good provided the disease is diagnosed before any permanent damage has occurred.

A considered decision is always important before the treatment is instituted. These cases are diagnosed with the help of biopsies from the involved organ/s. Most often, they need fiberoptic bronchoscopy to obtain transbronchial and endo-bronchial lung biopsy.  This is particularly so because the sarcoidosis treatment requires corticosteroid administration, which are avoided in case there is the possibility of an alternate diagnosis, such as tuberculosis (or lymphomas).EBUS guided fine needle aspiration biopsy adds to the accuracy of obtaining tissues especially in case only the thoracic lymph nodes are involved. 

We at our centre, see around 1-2 new cases of sarcoidosis every week, the diagnostic success with bronchoscopic procedures is almost 100 percent. This further emphasizes the frequency of its occurrence and the need for proper diagnosis.

The Use and Abuse of Corticosteroids

The Use and Abuse of Corticosteroids

Corticosteroids sometimes referred to simply as ‘steroids’, form a large groups of drugs with a common chemical structure.  They possess a diverse range of pharmacological and clinical actions.  Steroids with muscle building potential called “anabolic steroids” are sometimes used for wasting diseases, and abused by athletes or other sports persons.  We for the present, are primarily concerned here with corticosteroids used as medicine for different diseases.

Corticosteroids are normally occurring hormones in the body.  There are three types of steroids – mineralo-corticoids, glucocorticoids and the gonadocorticoids (sex hormones).  The gonadocorticoids are produced by the gonads (testes in males and ovaries in females), while the mineralo- and glucocorticoids are produced by adrenal glands located in the abdomen, resting over the two kidneys – therefore they are also called supra-renal glands.  Their production and release is governed by the pituitary gland located in the brain.

Corticosteroids, which are used as medicine, are chemically synthesized.  There is a large number of synthetic corticosteroids manufactured for use as drugs. While individual products have one or the other advantage, the primary actions are common to all the brands.  The mineralo-corticoids influence metabolism of ‘minerals’ i.e. sodium and potassium, while the gluco-corticoids are important in glucose and fat metabolism.

There are extensive indications for the medical use of corticosteroids.  Their use in adrenal deficiency is primarily for replacement therapy.  More commonly, they are used for suppression of immune activity or to control inflammation in a large number of immunological and inflammatory diseases.  In respiratory conditions, they are used in different stages of asthma and other allergies, chronic obstructive lung diseases, sarcoidosis, certain type of interstitial lung diseases, and pulmonary vasculitic disorders.  They are also used in many other medical illnesses – rheumatoid arthritis, polyarteritis nodosa, systemic vasculitides, skin allergies, urticaria, eczema, eye allergies, allergic rhinitis and a number of other common or uncommon diseases.  Steroids are lifesaving in severe anaphylactic shock, acute severe asthma, transplant rejections and diffuse lung haemorrhage (etc.).

There is no other choice when the medical indication for this use is clear and strong.  What is more worrying is their irrational and prolonged use, frequently without the knowledge of the patient.  There is no monitoring of side effects or attempts at their prevention.  It is common in this country for some medical practitioners to use small doses of corticosteroids even when there is no medical indication, to provide ‘magical relief’ to the patient to earn credit.  This is an abuse which must be avoided at all costs.

Corticosteroids, when used for longer periods in an un-regulated fashion, can lead to occurrence of complications such as salt and water accumulation which manifests with swelling of face and feet, hypertension, glucose intolerance or even frank diabetes,.  They also cause osteoporosis and bone- loss predisposing to spontaneous fractures of bones and complications such as collapse of vertebrae or necrosis of hip-joint bones. Fat redistribution results in thinning of limbs (arms and legs), accumulation of fat over the neck and the upper-back. Muscle wasting, abnormal striae over the body (arms and abdomen), acne and petechiae can also occur.  The symptoms of acidity and gastro-esophageal reflux (retrosternal burning, dyspepsia) are common.  There is also an increased frequency of infection, including tuberculosis in such patients.  

It is important to give supplemental calcium and vitamin D to most of the patients receiving corticosteroids.  Regular monitoring is most important which should be done for blood pressure, blood sugar levels, weight gain/ loss, serum calcium and vitamin D levels.

In summary, the corticosteroids use constitutes a double edged sword.  The use, when medically indicated, works like a wonder. Most importantly, the long-term effects need to be carefully assessed. The wisdom lies in the discretion to decide the indication.  This should be done on the basis of available medical evidence, and not on the whims and fancies of an individual practitioner. 

Tricity Forum talk

Dr. S. K. Jindal delivering a talk on chronic cough in the bi-monthly Tricity Chest Forum. The meeting was held at Hotel Park Plaza, Sec 17, Chandigarh on 21st June.

Free chest camp

Free chest camp

 to

 be held

 on 22/6/14

 from 10 am to 1 pm

 at

 Jindal Clinics

SCO 21, Sector 20D,

Chandigarh

Addiction of Asthma-inhalers?

Addiction of Asthma-inhalers?

S.K. Jindal,

MD (Medicine), FAMS, FCCP, FNCCP

(Former Professor & Head, Department of Pulmonary Medicine,

 PGIMER, Chandigarh, India)

Medical Director

Jindal Clinics, SCO 21, Sector 20 D, Chandigarh, India 160020

Inhalational drugs constitute the mainstay for treatment of asthma and other obstructive respiratory disorders such as the chronic obstructive pulmonary disease (COPD).  Asthma in particular commonly starts in childhood when drug administration with the use of an inhaler is considered a taboo.  Parents get worried the moment they are advised to use an inhaler for management of a child’s problem of asthma.  Similarly the other patients particularly the young individuals immediately pose the most frequently asked questions to the doctor: “Don’t you think that the inhalers are the addicting drugs?  I do not wish to get addicted to them for the rest of my life”. 

It is a genuine concern of any right thinking individual.  Addiction is a dreadful habit which must be avoided at all costs.  But do the inhalers truly end up in addiction?

Inhaler-addiction is a misconception – a wrong belief which somehow continues to propagate in spite of the evidence to the contrary.  Addiction commonly results from misuse or abuse of drugs which act upon the brain to produce a stimulatory, euphoric, soothing or hallucinogenic effect.  Importantly, an inhaler is only a device for administration of a drug used for asthma.  It is not a drug by itself.  Therefore, there is no question of inhaler-addiction.  If at all, the addiction will happen to anti-asthma drug/s, and not the inhalers.

Why not give drugs by mouth as pills or syrups?  The answer to this question is simple – the inhalational dose is several times smaller (in micrograms).  Therefore, the side-effects are much less, almost negligible.  This is particularly important in case of corticosteroids which are essentially required for maintenance treatment of asthma.  Moreover, the inhalation produces a quick effect like a locally administered drug.

Corticosteroids remain the most feared drugs because of their numerous side-effects when used for prolonged periods.  This fear is practically reduced to negligible when used in smaller doses by inhalational route.  Unfortunately, there is no other good substitute to inhalational corticosteroids for treatment of asthma.

The misconception about inhaler-addiction is largely based on the fact that the treatment is required to be continued for longer periods, sometimes indefinitely.  This produces a false notion that there occurs an addiction to the treatment.  Factually, continued treatment is the essential requirement for disease-control.  The continued use is not in any case akin to the use (or abuse) of an addicting psychotropic drug.  This is one misconception which must be get rid off - the earlier the better.