Doctors across the nation have agreed that the symptoms President Umar Musa Yar’Adua admitted to on a national television on May 29, 2009 are consistent with Churg-Strauss syndrome.
The pericarditis that made him seek medical attention in Saudi Arabia in November 2009 was said to be a mere complication of the Syndrome.
Pericaditis, doctors say, is curable, but in Yar’Adua’s case it was different because his pericarditis was a complication of an already pre-existing condition - Churg-Strauss syndrome.
Speaking on the "Churg-Strauss" syndrome, Consultant Surgeon, Dr Emmanuel Enabulele, said it is an immunological rejection of the body organs by the immune system. “The immune system rejects the body organs. That was why Yar’Adua was going for several medical check-ups abroad and he also was on a high dose of steroids. Steroids also depresse the immune system and leads to infections. And that was why he had chest infections, he was always coughing, and other infections.
“The syndrome is a multi-organ failure, it affected his kidneys, liver, heart, everywhere, it even affected his skin, if you look closely, you will find rashes on his skin. By the time he went to Saudi Arabia, he was at the end-stage of the syndrome.
All the time he was Governor in Katsina, he already had it. It is a progressive kind of thing, the syndrome keeps progressing, it has no cure, it is terminal,” he said.
Doctors also say that as Katsina State governor, Yar’Adua could not function at full capacity because of the condition. Consultant Cardialogist, Dr Austin Obaze noted that at a time Yar’Adua left Katsina for six months to seek medical treatment abroad. “That could be the same kind of thing the Yar’Adua group was hoping on when he spent about the same length of time in Saudi Arabia. They hoped he will get better, come back and take over power, but the Condition was at its end-stage.
Dr Obaze said, “It is like any other disease that involves the immune system. The immune system is meant to defend the body, but now finds the organs strange to work with, that is the disease Yar’Adua had.
“He didn’t make public appearance because obviously he was not looking good. He had to take steroid to fight those infections, his immune system was depressed further because of the steroid he was taking, it was like creating artificial Acquired Immune Deficiency Syndrome (AIDS) and he died. Churg Strauss has no cure, you can only manage the condition. The steroids were given to prolong life, but it depresses immunity as its side-effect,” he said.
Yar’Adua tried to downplay the severity of his health problems, but several medical doctors knowledgeable about his physical condition have reaffirmed that Yar’Adua was diagnosed of Churg Strauss disease and has been on a regimen of treatment since.
Doctors also say that Yar’Adua is also dogged by asthma, which may have been responsible for his persistent cough and laboured breathing during some nationwide broadcasts.
A source who spoke anonymously said that Dr Salisu Borau Banye, Yar’Adua personal physician, has been treating him over the years for asthma. Our source indicated that, while Governor of Katsina, Yar’Adua had equipped the General Hospital in Katsina with dialysis machine to take care of his kidney treatments. Dr. Banye acted as the hospital’s Medical Director than.
It will also be recalled that in a nationally televised interview, Yar’Adua submitted that his arteries had dilated by 21/2 diameters, a condition that doctors describe as “vasculitis,” a major feature of Churg Strauss syndrome.
FACTS ABOUT CHURG-STRAUSS SYNDROME
Churg-Strauss syndrome (also known as “allergic granulomatosis) is a medium and small vessel autoimmune vasculitis, leading to necrosis. It involves mainly the blood vessels of the lungs (it begins as a severe type of asthma), gastro-intestinal system, and peripheral nerves, but also affects the heart, skin and kidneys. It is a rare disease that is non-inheritable, non-transmissible. Churg-Strauss syndrome was once considered a type of polyarteritis nodosa due to its similar morphologies.
The syndrome was first described by Drs. Jacob Churg and Lotte Strauss at Mount Sinai Hospital in New York City in 1951.
Diagnosing the disease
Diagnostic markers include eosinophil granulocytes and granulomas in affected tissue and anti-neutrophil cytoplaSmic antibodies (ANCA) against neutrophil granulocytes. Differentiation from Wegener’s granulomatosis can be difficult, though the increasing use of ANCA has made the distinction more routine. Wegener’s is closely associated with c-ANCA, unlike Churg-Strauss, which shows elevations of p-ANCA.
Disease stages
This disease has three distinct stages. The first stage often involves the sinuses and the onset of allergies not previously had or the worsening of pre-existing allergies. The second stage involves the onset of acute asthma. Normally, the person would not have had asthma previously. The third and final stage involves the various organ systems. This stage is by far the most life threatening and painful. Often the person will develop severe nerve pain in the legs, arms and bands. Purple marks will appear on the skin and often sores will appear in the mouth or nose. The disease can affect the heart, lungs, kidneys and liver. People can live for many years in the first two stages before progressing to stage three.
Risk stratification
The French Vasculitis Study Group has developed a five-point score (“five-factor score” or FFS) that predicts the risk of death in Churg-Strauss syndrome. These are reduced renal function (creatinine >1.58 mgldL or 140 µmol/I), proteinuria (> 1 g/24h), gastrointestinal hemorrhage, infarction or pancreatitis, involvement of the central nervous system or cardiomyopathy. Presence of 1 of these indicates severe disease (5-year mortality 26 percent) and 2 or more very severe disease (mortality 46 percent), while absence of any of these 5 indicates a milder case (mortality 11.9 percent).
Treatment
Treatment for Churg Strauss syndrome includes glucocorticoids such as prednisolone and other immunosuppressive drugs such as azathioprine and cyclophosphamide. In many cases the disease can be put into a type of chemical remission through drug therapy, but the disease is chronic and lifelong.
A systematic review conducted in 2007 indicated that all patients should be treated with high-dose steroids, but that in patients with an FFS of 1 or higher cyclophosphamide pulse therapy should be commenced, with 12 pulses leading to fewer relapses than 6. Remission can be maintained with a less toxic drug, such as
azathioprine or methotrexate.
Research
A case study in 2000 noticed a “marked and severe inflammatory reaction characterized by eosinophilia and glomerulonephritis” and wondered whether this “might have been triggered by the leukotriene receptor antagonist, montelukast.’, Researchers have searched for links between drugs such as montelukast (brand name of Singulair) and Churg Strauss syndrome; in another study in 2000, researchers did not find a cause-and-effect relationship and wrote: “The occurrence of Churg Strauss syndrome in asthmatic patients receiving leukotriene modifiers appears to be related to unmasking of an underlying vasculitic syndrome that is initially clinically recognized as moderate to severe asthma and treated with corticosteroids. Montelukast does not appear to directly cause the syndrome in these patients. A case study in 2006 was inconclusive but suggested the need for further research.
Famous patients
'Patient', a 192 page memoir by the musician Ben Watt published May 1 1997 by Penguin Books, deals with Watt’s mid-1990s experience with Churg Strauss syndrome and his recovery. Watt’s case was unusual in that it mainly affected his gastrointestinal tract, leaving his lungs largely unaffected; this unusual presentation contributed to a delay in proper diagnosis. His treatment required the removal of large sections of necrotized intestine, leaving Watt on a permanently restricted diet.
Umaru Musa Yar’Adua, the president of Nigeria from 2007-2010, reportedly suffered from Churg Strauss syndrome and died in office of complications of the disease.
The DJ and author Charlie Gillett was diagnosed with Churg Strauss in 2006; he died four years later.
By Azoma Chikwe (Friday, May 07, 2010)
The pericarditis that made him seek medical attention in Saudi Arabia in November 2009 was said to be a mere complication of the Syndrome.
Pericaditis, doctors say, is curable, but in Yar’Adua’s case it was different because his pericarditis was a complication of an already pre-existing condition - Churg-Strauss syndrome.
Speaking on the "Churg-Strauss" syndrome, Consultant Surgeon, Dr Emmanuel Enabulele, said it is an immunological rejection of the body organs by the immune system. “The immune system rejects the body organs. That was why Yar’Adua was going for several medical check-ups abroad and he also was on a high dose of steroids. Steroids also depresse the immune system and leads to infections. And that was why he had chest infections, he was always coughing, and other infections.
“The syndrome is a multi-organ failure, it affected his kidneys, liver, heart, everywhere, it even affected his skin, if you look closely, you will find rashes on his skin. By the time he went to Saudi Arabia, he was at the end-stage of the syndrome.
All the time he was Governor in Katsina, he already had it. It is a progressive kind of thing, the syndrome keeps progressing, it has no cure, it is terminal,” he said.
Doctors also say that as Katsina State governor, Yar’Adua could not function at full capacity because of the condition. Consultant Cardialogist, Dr Austin Obaze noted that at a time Yar’Adua left Katsina for six months to seek medical treatment abroad. “That could be the same kind of thing the Yar’Adua group was hoping on when he spent about the same length of time in Saudi Arabia. They hoped he will get better, come back and take over power, but the Condition was at its end-stage.
Dr Obaze said, “It is like any other disease that involves the immune system. The immune system is meant to defend the body, but now finds the organs strange to work with, that is the disease Yar’Adua had.
“He didn’t make public appearance because obviously he was not looking good. He had to take steroid to fight those infections, his immune system was depressed further because of the steroid he was taking, it was like creating artificial Acquired Immune Deficiency Syndrome (AIDS) and he died. Churg Strauss has no cure, you can only manage the condition. The steroids were given to prolong life, but it depresses immunity as its side-effect,” he said.
Yar’Adua tried to downplay the severity of his health problems, but several medical doctors knowledgeable about his physical condition have reaffirmed that Yar’Adua was diagnosed of Churg Strauss disease and has been on a regimen of treatment since.
Doctors also say that Yar’Adua is also dogged by asthma, which may have been responsible for his persistent cough and laboured breathing during some nationwide broadcasts.
A source who spoke anonymously said that Dr Salisu Borau Banye, Yar’Adua personal physician, has been treating him over the years for asthma. Our source indicated that, while Governor of Katsina, Yar’Adua had equipped the General Hospital in Katsina with dialysis machine to take care of his kidney treatments. Dr. Banye acted as the hospital’s Medical Director than.
It will also be recalled that in a nationally televised interview, Yar’Adua submitted that his arteries had dilated by 21/2 diameters, a condition that doctors describe as “vasculitis,” a major feature of Churg Strauss syndrome.
FACTS ABOUT CHURG-STRAUSS SYNDROME
Churg-Strauss syndrome (also known as “allergic granulomatosis) is a medium and small vessel autoimmune vasculitis, leading to necrosis. It involves mainly the blood vessels of the lungs (it begins as a severe type of asthma), gastro-intestinal system, and peripheral nerves, but also affects the heart, skin and kidneys. It is a rare disease that is non-inheritable, non-transmissible. Churg-Strauss syndrome was once considered a type of polyarteritis nodosa due to its similar morphologies.
The syndrome was first described by Drs. Jacob Churg and Lotte Strauss at Mount Sinai Hospital in New York City in 1951.
Diagnosing the disease
Diagnostic markers include eosinophil granulocytes and granulomas in affected tissue and anti-neutrophil cytoplaSmic antibodies (ANCA) against neutrophil granulocytes. Differentiation from Wegener’s granulomatosis can be difficult, though the increasing use of ANCA has made the distinction more routine. Wegener’s is closely associated with c-ANCA, unlike Churg-Strauss, which shows elevations of p-ANCA.
Disease stages
This disease has three distinct stages. The first stage often involves the sinuses and the onset of allergies not previously had or the worsening of pre-existing allergies. The second stage involves the onset of acute asthma. Normally, the person would not have had asthma previously. The third and final stage involves the various organ systems. This stage is by far the most life threatening and painful. Often the person will develop severe nerve pain in the legs, arms and bands. Purple marks will appear on the skin and often sores will appear in the mouth or nose. The disease can affect the heart, lungs, kidneys and liver. People can live for many years in the first two stages before progressing to stage three.
Risk stratification
The French Vasculitis Study Group has developed a five-point score (“five-factor score” or FFS) that predicts the risk of death in Churg-Strauss syndrome. These are reduced renal function (creatinine >1.58 mgldL or 140 µmol/I), proteinuria (> 1 g/24h), gastrointestinal hemorrhage, infarction or pancreatitis, involvement of the central nervous system or cardiomyopathy. Presence of 1 of these indicates severe disease (5-year mortality 26 percent) and 2 or more very severe disease (mortality 46 percent), while absence of any of these 5 indicates a milder case (mortality 11.9 percent).
Treatment
Treatment for Churg Strauss syndrome includes glucocorticoids such as prednisolone and other immunosuppressive drugs such as azathioprine and cyclophosphamide. In many cases the disease can be put into a type of chemical remission through drug therapy, but the disease is chronic and lifelong.
A systematic review conducted in 2007 indicated that all patients should be treated with high-dose steroids, but that in patients with an FFS of 1 or higher cyclophosphamide pulse therapy should be commenced, with 12 pulses leading to fewer relapses than 6. Remission can be maintained with a less toxic drug, such as
azathioprine or methotrexate.
Research
A case study in 2000 noticed a “marked and severe inflammatory reaction characterized by eosinophilia and glomerulonephritis” and wondered whether this “might have been triggered by the leukotriene receptor antagonist, montelukast.’, Researchers have searched for links between drugs such as montelukast (brand name of Singulair) and Churg Strauss syndrome; in another study in 2000, researchers did not find a cause-and-effect relationship and wrote: “The occurrence of Churg Strauss syndrome in asthmatic patients receiving leukotriene modifiers appears to be related to unmasking of an underlying vasculitic syndrome that is initially clinically recognized as moderate to severe asthma and treated with corticosteroids. Montelukast does not appear to directly cause the syndrome in these patients. A case study in 2006 was inconclusive but suggested the need for further research.
Famous patients
'Patient', a 192 page memoir by the musician Ben Watt published May 1 1997 by Penguin Books, deals with Watt’s mid-1990s experience with Churg Strauss syndrome and his recovery. Watt’s case was unusual in that it mainly affected his gastrointestinal tract, leaving his lungs largely unaffected; this unusual presentation contributed to a delay in proper diagnosis. His treatment required the removal of large sections of necrotized intestine, leaving Watt on a permanently restricted diet.
Umaru Musa Yar’Adua, the president of Nigeria from 2007-2010, reportedly suffered from Churg Strauss syndrome and died in office of complications of the disease.
The DJ and author Charlie Gillett was diagnosed with Churg Strauss in 2006; he died four years later.
By Azoma Chikwe (Friday, May 07, 2010)
Comments
Post a Comment