ANA Titre (Immunofluorescence) Test

Cirrhosis of liver is slow and gradual replacement of normal healthy liver tissue with scar tissue which results in poor liver function and blockage of flow of blood through liver which comes from intestines. As more scar tissue replaces normal healthy liver, liver begins to fail.

What causes cirrhosis?

1. Alcohol: Amount of alcohol which can cause liver damage varies from person to person, however those who consume alcohol for long in significant amount are more prone to develop liver damage.
2. Hepatitis B or hepatitis C: Caused by hepatitis B and C viruses, respectively, which are acquired by contact with contaminated blood (like needlestick injury, blood transfusion, injection drug abuser), sexual contact with infected person and from mother to child during childbirth.
3. Non-alcoholic fatty liver disease (NAFLD): Caused by presence of extra fat in liver (after excluding significant alcohol intake) which causes inflammation and scarring leading to cirrhosis. Usually seen in patients who are overweight or obese, having diabetes, high fat in blood, high blood pressure or metabolic syndrome.
4. Drugs causing liver injury.
5. Others: Autoimmune hepatitis (body`s own immunity acting against liver), Wilson disease (build up of copper in body), hemochromatosis (excess of iron in body), certain bile duct disorders, etc.

What are the symptoms of cirrhosis?

1. Early disease can present with nonspecific symptoms like weakness, fatigue (loss of energy), decrease wish to take food, vomiting or nausea, weight loss, etc.
2. Advanced disease can present with fluid accumulation in legs (edema) or abdomen (ascites), facial puffiness, yellowish discoloration of eyes or urine (jaundice), redness of palm (palmer erythema), decrease urine output, small red lesions on skin, easy bleeding following trauma, blood coming out of mouth/anus/other body site, black colored feaces, mental confusion, flapping tremors of hand, etc. In men it can cause impotence, breast enlargement and shrinking of testis.

What are the complication of cirrhosis?

Portal hypertension: It is a common complication of cirrhosis which is due to increased pressure in portal vein. Portal vein is main blood vessel which carries blood to liver from stomach, intestines, spleen, gallbladder and pancreas. Because of scar formation in liver the normal flow of blood from these organs to liver is hampered. As a result of blockage of blood flow to liver there are few complications which can arise like accumulation of fluid in abdomen (ascites) and legs (edema), formation of enlarged veins (varices) in food pipe (esophagus), stomach, etc., enlargement of spleen (splenomegaly), mental confusion/altered behavior/altered sensorium (hepatic encephalopathy), respiratory discomfort (hepatic hydrothorax or hepato-pulmonary syndrome) or decrease urine output/rise in creatinine (hepato-renal syndrome).

1. Increase risk of infection because of immune system dysfunction.
2. Development of liver cancer (Hepatocellular carcinoma)
3. Easy brusibility or bleeding following light trauma.
4. Gall bladder stone formation
5. Metabolic bone disease

What are the stages of cirrhosis?

1. Compensated cirrhosis: Liver damage is damaged but no abdominal swelling (ascites), blood in vomiting or black/red color feaces (variceal bleed), altered behavior/sensorium (hepatic encephalopathy), respiratory discomfort (hepatic hydrothorax or hepato-pulmonary syndrome) or decrease urine output/rise in creatinine (hepato-renal syndrome).
2. Decompensated cirrhosis: Presence of abdominal swelling (ascites), blood in vomiting or black/red color feaces (variceal bleed), altered behavior/sensorium (hepatic encephalopathy), respiratory discomfort (hepatic hydrothorax or hepato-pulmonary syndrome) or decrease urine output/rise in creatinine (hepato-renal syndrome).

How cirrhosis is diagnosed?

1. Your doctor will take good history and do proper physical examination. If there is suspicion of cirrhosis then he will subject you to some blood test, ultrasound abdomen, fibroscan/elastography, upper GI endoscopy and if needed CT scan or MRI of abdomen or liver biopsy.

2. Ultrasound of abdomen in cirrhosis may shows coarse liver echotexture, nodular liver surface, dilated portal vein or collateral, enlarged spleen or abdominal fluid (ascites).

How to prevent cirrhosis?

Best way to avoid development of cirrhosis from predisposing stage of liver illness is to recognize and treat early. Few advices to keep liver healthy are:

1. Dietary modification: Eat healthy balanced diet. Avoid high calorie food or drinks, saturated fat, sugar and refined carbohydrates. Keep yourself hydrated.
2. Lifestyle modification: Maintain healthy body weight. Avoid being overweight or obese. Aerobic exercise (like brisk walk 30-45min/day atleast 5days/week) regularly helps to lower liver fat.
3. Avoid: Avoid use of contaminated needles, sharing of items of personal hygiene (like shaving razors, toothbrush, nail clippers, etc), use of illicit drugs, self medication with over the counter drugs or using drugs beyond doctor`s advice.
   1. Practice safe protected sex
   2. Alcohol
4. Motivate others: to stop drinking and follow hand hygiene and doctor`s advice.
5. Hand hygiene: Wash hand with soap and water regularly before eating, after going to toilet and after touching dirty objects.
6. Vaccination: For hepatitis A or hepatitis B, if you are not vaccinated or not already infected or unsafe antibody titre. Transmission rate of hepatitis B from mother to child at birth can be reduced with vaccination and immunization of newborn starting within 12 hours of birth as well as by using antiviral drugs (if indicated).

Prevent others from getting infected from you if you harbor virus causing liver damage.

How to treat cirrhosis?

Treatment of cirrhosis is based on cause of cirrhosis and complication of cirrhosis. Main aim of treatment in early stage of cirrhosis is to slow the progression of cirrhosis and prevent complications development

1. Stop alcohol, antiviral drugs for hepatitis b or hepatitis C, proper sugar level if diabetes, steroid or other medications for autoimmune hepatitis, medications to reduce copper from body in patients with Wilson's disease, etc.
2. Drugs to lower portal pressure (beta-blocker or nitrates), drugs to remove fluid from body (diuretics), drugs to lower ammonia level for encephalopathy (lactulose and others), drugs to improve kidney function (albumin, terlipressin and others).
3. Opt for liver ransplant, If cirrhosis is life threatening or treatment of cirrhosis complication is ineffective.
4. Low salt high protein diet
5. Stop alcohol intake even if you have other cause of cirrhosis.
6. Stop smoking
7. Avoid over the counter drugs (especially pain killers)
8. Vaccinate if not done already or infected

   If you wish to discuss about any specific problem, you can consult a gastroenterologist.

Kindler’s Syndrome is a rare skin blistering disease named after Theresa Kindler. It is a kind of skin complication which forms skin blisters and lesions on the mucous membranes. This can happen in any part of your body but mainly concentrates on the area where you have experienced any frictions, bruises or minor trauma, especially on the feet and the hands. In some rare occurrences of Kindler’s Syndrome, it can develop on the internal organs like the food pipe, stomach or the respiratory tract.

This disease generally occurs if you suffer from a disorder called autosomal recessive. It means that you may have inherited an anomalous gene from each parent. It usually happens during childhood and reduces as you age.

Symptoms:
Some of the symptoms of Kindler syndrome are:

1. Blisters occurring on the body in your infancy and periods of early childhood.
2. Slowly you will observe altered pigmentation and cutaneous atrophy (A severe dermal condition).
3. Blistering due to certain skin trauma appearing on the hands and feet.
4. Some observe abnormalities like dental or ophthalmic
5. The growth of actinic Keratoses

Treatment:
Kindler’s Syndrome requires a specialized dermatologist for the treatment. In some countries, diagnosis involves skin biopsy. It involves undergoing Immunofluorescence Antigen Mapping or transmission of Electron Microscopy. Blood testing of genes is slowly being developed as a diagnostic test.

Other treatments involve:

1. A healthy and nutritious diet to heal the disease from within.
2. Maintaining a good dental hygiene.
3. Use sunscreen with a high protein formula.
4. Avoid exposure to the sun as much as possible.

Lymphogranuloma venereum (LGV) is a STD caused by the three different types of the “Chlamydia trachomatis” bacterium. LGV is generally a disease of the lymphatic system. It is characterized by small, asymptomatic skin lesions along the pelvis or groin region. It has been noticed that men are more prone to this than women.

What are the symptoms of lymphogranuloma venereum?
The symptoms may start within a few days or months after coming in contact with the bacteria. Some of the common symptoms are:
1. Pain during bowel movement
2. The growth of small painless sores in the genital area
3. Your skin might swell and turn red.
4. There can be drainage from the lymph nodes in the groin.
5. Blood and puss may pass from the rectum as well

What are the possible complications related to lymphogranuloma venereum?
1) The patient may have brain inflammation although this is very rare
2) The patient may suffer from infection in joints, eyes, heart, and liver
3) The rectum may get narrower and scary
4) There may be long-term swelling and inflammation of the genitals

How can you diagnose lymphogranuloma venereum?
1. Generally, doctors suspect lymphogranuloma venereum when the patient is suffering from genital ulcers, swollen lymph nodes and proctitis (inflammation of the rectum and anus).
2. Rectal specimens, genital lesions, and lymph node specimens are taken to test for Chlamydia trachomatis. This is detected by culture, nucleic acid detection, and direct immunofluorescence tests.
What are the treatments available for lymphogranuloma venereum?

People with LGV must be treated with antibiotics and drainage of the bulbous growths may also be necessary for symptomatic relief. Generally, two antibiotics are used to treat such patients:
1. Doxycycline, may be used to treat patients who are not pregnant.
2. Erythromycin is used in other cases and especially for women who are pregnant.

HIV positive patients and HIV-negative patients are treated in the same way. However, HIV patients may have to continue the treatment for a longer period of time due to the compromised immune system. The patients have to abstain from sexual activity till the completion of the antibiotic dose. If you wish to discuss about any specific problem, you can consult a doctor and ask a free question.

Vital notes:

• RA is a chronic debilitating joint disease that affects small joints of hands and foot.
• More common in females aged 20 to 50 years.
• It is not synonymous with Rheumatic Fever which occurs in children aged less than 15 years.
• It has got nothing to do with monthly Penicillin injections and checking ASO titre. Somebody on Penicillin injections or being checked for ASO titre should change the doctor immediately.
• Untreated disease may cause deformity of hands and foot causing deterioration of quality of life.
• Erosion of small joints along with loss of entire joint architecture is the basic disease process.
• Early morning stiffness of joints along with swelling of one or more small joints of hands is very characteristic.
• Treatment is done with Methotrexate, Hydroxychloroquine Sulfate, Sulfasalazine, Leflunomide and short courses of steroids.
• Difficult diseases are treated with costly injections called biologics.
• The motto of treatment is “ catch early, hit hard and achieve and maintain treatment goal “
• Lung and cardiac complications are common in long term disease.
• Rheumatologists but not the Orthopedicians nor neurologist are the best person to treat the disease.                    

Introduction:

Rheumatoid Arthritis is an autoimmune disease of mankind where the cells and molecules who are responsible for security of our body, misidentify the joint tissue as foreign substance and start destroying them similar to the way they usually do when a bacteria or virus enters the body. This misdirected attack towards our own body is called autoimmune phenomenon.

The worst affected parts of the body are the joints of hands and foot. These small joints that are responsible for performance of day to day activities like eating, holding, writing, typing, washing etc become painful and swollen causing stiffness and deformity.

This results in poor quality of life and subsequent depression. Early diagnosis and continuous treatment with specific medicines is cornerstone of treatment.

What are the symptoms?

Symptoms vary from person to person and also from early stage to late stage of the disease.

 In early stage there may be mild pain and stiffness involving 1 to 4 small joints of the hands and feet with a typical pattern of stiffness of those joints after rising from bed. During this phase it is very difficult to diagnose the disease. But it is very important to catch the disease in this stage. Researchers have proved that joint damage and bony erosions start even before the symptoms have manifested and hence earlier the treatments better the results.

In a full blown case of Rheumatoid arthritis the picture is very classical of a middle aged female presenting with pain and swelling of hand and foot joints along with early morning stiffness that responds to steroids brilliantly. Wrists and the knuckles are predominantly involved. Patients are unable to hold a pen or open the door or do cooking etc. Gripping the wrist firmly causes pain and the hand joints are warm.

In a advanced case of the disease we often find elderly people presenting with crooked hands with areas of permanent swelling and skin changes due to chronic friction. The skin fold may contract chronic fungal infections and the joints are swollen and painful.

A few other organs are damaged in silence and they create serious problem at the advanced stage of the disease. Lungs, Heart and Blood Vessels are frequently affected. There is accelerated cholesterol deposition in arteries of heart causing increased risk of heart attack and brain stroke. These patients frequently contract infections which are difficult to treat.

Involvement of spine or low back is uncommon. However, cervical spine or the neck region is the only part of the spine that may be affected in this disease.

What is the difference between Rheumatic Fever & Rheumatoid Arthritis?

There is a heaven & hell difference between these two entities. Rheumatic Fever is a disease of childhood. It usually occurs in children aged less than 15 years and is characterised by excruciating pain, swelling and redness of one or more big joints of arms and legs that responds excellently to Aspirin. This disorder may cause cardiac problems and is the leading cause of Valvular Herat disease in India. It is often characterised by some skin rashes and rarely neurologic problems. The disease process, the causative agent and the clinical symptoms are completely different from that of Rheumatoid arthritis.

Why Penicillin injections are given on a monthly basis?

Patients of Rheumatic fever often suffer a damage to the valves of the heart during the attack of Rheumatic fever. This damaged valves are potential sites of infections that may develop in the later part of the life. This infection is called Infective Endocarditis and may cause serious complications if not addressed properly. Penicillin injections are given as a form of prevention of development of this infection and have got no relationship with the wellness or cure of the disease. People who are aged above 15 years and are being treated with penicillin for the first time for joint pain occurring in small joints of hands should immediately change their doctor and consult a rheumatologist.

How is Rheumatoid Arthritis diagnosed?

Rheumatoid Arthritis is diagnosed by its classical symptoms and some laboratory investigations. To identify early rheumatoid cases we usually follow a scoring system which includes counting of number of joints involved, the duration of the symptoms, checking ESR and CRP in blood and looking for the positivity of Rheumatoid Factor and Anti CP Antibody. Patients with high values of Anti CP Antibody are very much likely to suffering from rheumatoid arthritis and also they are very much likely to suffer a aggressive disease course

Does each and every patient of rheumatoid arthritis develop crooked hands and foot?

No. Not all patients suffer from such a devastating complications. There are important characteristics which if present predict severe destructive disease. Females, Smokers, High values of Anti CCP or Rheumatoid Arthritis, Family History of Rheumatoid Arthritis, Early evidence of Joint destruction on X Ray are a few important features who will go forward to develop destructive disease.

How Rheumatoid Arthritis is treated?

Continuous Physical activity of the involved joints is the corner stone of treatment. More the joints are active, less the damage.

Cessation of smoking is mandatory. Smokers tend to develop early joint destruction

Medicines that used in treating rheumatoid arthritis are together called Disease Modifying Anti Rheumatoid Drugs or DMARDs.

Methotrexate, Hydroxychloroquine Sulfate, Sulfasalazine, Leflunomide are the principal DAMRDs. Of these, methotrexate is the backbone of therapy. Methotrexate is used on a once a weekly basis starting from a dose of 5 mg to 50mg/week and a weekly 5mg of Folic Acid supplement is given to neutralise the adverse effects of Methotrexate. The detail of the drugs is mentioned below.

Why I am being given Steroids?

Steroids are given for a short period of increased disease activity. In rheumatoid arthritis we do not prescribe steroids in a regular basis. It is often prescribed when the patient is put on the DMARDs for the first time and when there is break through pain or active disease during the treatment.

Steroids are used in low dose in a decreasing dosage pattern over weeks and usually are not continued more than a month.

What are Biologic agents? Why they are so costly?

Biologic agents are highly specific molecules who directly block the action of the culprit chemicals in our body that cause the joint destruction. They are highly researched molecule an d is manufactured abroad. They are usually available in injection formats and have their own side effect profile which includes reactivation of Tuberculosis and other opportunistic infections. Usually patients are checked for latent Tuberculosis Infections, HIV, Hepatitis B and C before the treatment is started.

Biologics cause excellent response to the disease causing alleviation of pain and swelling and sense of well being but unfortunately they do not provide any cure. Hence, if biologics are stopped then there are chances that the disease activity may worsen requiring more biologics or aggressive treatment with DMARDs.