Churg-Strauss syndrome
Encyclopedia
Churg–Strauss syndrome 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
), gastrointestinal system, and peripheral nerves, but also affects the heart, skin and kidneys. It is a rare disease that is non-inheritable and non-transmissible. Churg–Strauss syndrome was once considered a type of polyarteritis nodosa
due to their similar morphologies.
The syndrome was first described by Drs. Jacob Churg and Lotte Strauss at Mount Sinai Hospital
in New York City
in 1951.
s and granuloma
s in affected tissue and anti-neutrophil cytoplasmic antibodies
(ANCA) against neutrophil granulocyte
s. Differentiation from Wegener's granulomatosis
is not difficult. Wegener's is closely associated with c-ANCA, while Churg-Strauss can be associated with elevations of p-ANCA.
People can live for many years in the first two stages before progressing to stage three.
(creatinine >1.58 mg/dL or 140 μmol/l), (2) proteinuria
(>1 g/24h), (3) gastrointestinal hemorrhage, infarction or pancreatitis
, (4) involvement of the central nervous system
or (5) cardiomyopathy
. Presence of 1 of these indicates severe disease (5-year mortality 26%) and 2 or more very severe disease (mortality 46%), while absence of any of these 5 indicates a milder case (mortality 11.9%).
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
.
." Researchers have searched for links between drugs such as montelukast
(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.
, by the musician Ben Watt
, 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.
Autoimmune disease
Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. In other words, the body actually attacks its own cells. The immune system mistakes some part of the body as a pathogen and attacks it. This may be restricted to...
vasculitis
Vasculitis
Vasculitis refers to a heterogeneous group of disorders that are characterized by inflammatory destruction of blood vessels. Both arteries and veins are affected. Lymphangitis is sometimes considered a type of vasculitis...
, leading to necrosis
Necrosis
Necrosis is the premature death of cells in living tissue. Necrosis is caused by factors external to the cell or tissue, such as infection, toxins, or trauma. This is in contrast to apoptosis, which is a naturally occurring cause of cellular death...
. It involves mainly the blood vessels of the lungs (it begins as a severe type of asthma
Asthma
Asthma is the common chronic inflammatory disease of the airways characterized by variable and recurring symptoms, reversible airflow obstruction, and bronchospasm. Symptoms include wheezing, coughing, chest tightness, and shortness of breath...
), gastrointestinal system, and peripheral nerves, but also affects the heart, skin and kidneys. It is a rare disease that is non-inheritable and non-transmissible. Churg–Strauss syndrome was once considered a type of polyarteritis nodosa
Polyarteritis nodosa
Polyarteritis nodosa is a vasculitis of medium & small-sized arteries, which become swollen and damaged from attack by rogue immune cells. Polyarteritis nodosa is also called Kussmaul disease or Kussmaul-Maier disease...
due to their similar morphologies.
The syndrome was first described by Drs. Jacob Churg and Lotte Strauss at Mount Sinai Hospital
Mount Sinai Hospital, New York
Mount Sinai Hospital, founded in 1852, is one of the oldest and largest teaching hospitals in the United States. In 2011-2012, Mount Sinai Hospital was ranked as one of America's best hospitals by U.S...
in New York City
New York City
New York is the most populous city in the United States and the center of the New York Metropolitan Area, one of the most populous metropolitan areas in the world. New York exerts a significant impact upon global commerce, finance, media, art, fashion, research, technology, education, and...
in 1951.
Diagnosis
Diagnostic markers include eosinophil granulocyteEosinophil granulocyte
Eosinophil granulocytes, usually called eosinophils or eosinophiles , are white blood cells that are one of the immune system components responsible for combating multicellular parasites and certain infections in vertebrates. Along with mast cells, they also control mechanisms associated with...
s and granuloma
Granuloma
Granuloma is a medical term for a tiny collection of immune cells known as macrophages. Granulomas form when the immune system attempts to wall off substances that it perceives as foreign but is unable to eliminate. Such substances include infectious organisms such as bacteria and fungi as well as...
s in affected tissue and anti-neutrophil cytoplasmic antibodies
Anti-neutrophil cytoplasmic antibody
Anti-neutrophil cytoplasmic antibodies are a group of autoantibodies, mainly of the IgG type, against antigens in the cytoplasm of neutrophil granulocytes and monocytes...
(ANCA) against neutrophil granulocyte
Neutrophil granulocyte
Neutrophil granulocytes are the most abundant type of white blood cells in mammals and form an essential part of the innate immune system. They are generally referred to as either neutrophils or polymorphonuclear neutrophils , and are subdivided into segmented neutrophils and banded neutrophils...
s. Differentiation from Wegener's granulomatosis
Wegener's granulomatosis
Wegener's granulomatosis , more recently granulomatosis with polyangiitis , is an incurable form of vasculitis that affects the nose, lungs, kidneys and other organs. Due to its end-organ damage, it is life-threatening and requires long-term immunosuppression...
is not difficult. Wegener's is closely associated with c-ANCA, while Churg-Strauss can be associated with 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 asthmaAsthmaAsthma is the common chronic inflammatory disease of the airways characterized by variable and recurring symptoms, reversible airflow obstruction, and bronchospasm. Symptoms include wheezing, coughing, chest tightness, and shortness of breath...
. Normally, the person would not have had asthma previously. - The third and final stage involves the various organ systems. Stage three is by far the most life threatening and painful. Often the person will develop severe nerve pain in their legs, arms and hands. 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, liver and digestive system.
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 (1) reduced renal functionRenal function
Renal function, in nephrology, is an indication of the state of the kidney and its role in renal physiology. Glomerular filtration rate describes the flow rate of filtered fluid through the kidney...
(creatinine >1.58 mg/dL or 140 μmol/l), (2) proteinuria
Proteinuria
Proteinuria means the presence of anexcess of serum proteins in the urine. The protein in the urine often causes the urine to become foamy, although foamy urine may also be caused by bilirubin in the urine , retrograde ejaculation, pneumaturia due to a fistula, or drugs such as pyridium.- Causes...
(>1 g/24h), (3) gastrointestinal hemorrhage, infarction or pancreatitis
Pancreatitis
Pancreatitis is inflammation of the pancreas. It occurs when pancreatic enzymes that digest food are activated in the pancreas instead of the small intestine. It may be acute – beginning suddenly and lasting a few days, or chronic – occurring over many years...
, (4) involvement of the central nervous system
Central nervous system
The central nervous system is the part of the nervous system that integrates the information that it receives from, and coordinates the activity of, all parts of the bodies of bilaterian animals—that is, all multicellular animals except sponges and radially symmetric animals such as jellyfish...
or (5) cardiomyopathy
Cardiomyopathy
Cardiomyopathy, which literally means "heart muscle disease," is the deterioration of the function of the myocardium for any reason. People with cardiomyopathy are often at risk of arrhythmia or sudden cardiac death or both. Cardiomyopathy can often go undetected, making it especially dangerous to...
. Presence of 1 of these indicates severe disease (5-year mortality 26%) and 2 or more very severe disease (mortality 46%), while absence of any of these 5 indicates a milder case (mortality 11.9%).
Treatment
Treatment for Churg–Strauss syndrome includes glucocorticoids such as prednisolonePrednisolone
Prednisolone is the active metabolite of prednisone, which is also used as a drug.-Uses:Prednisolone is a corticosteroid drug with predominant glucocorticoid and low mineralocorticoid activity, making it useful for the treatment of a wide range of inflammatory and auto-immune conditions such as...
and other immunosuppressive drugs such as azathioprine
Azathioprine
Azathioprine is a purine analogue immunosuppressive drug. It is used to prevent organ rejection following organ transplantation and to treat a vast array of autoimmune diseases, including rheumatoid arthritis, pemphigus, inflammatory bowel disease , multiple sclerosis, autoimmune hepatitis, atopic...
and cyclophosphamide
Cyclophosphamide
Cyclophosphamide , also known as cytophosphane, is a nitrogen mustard alkylating agent, from the oxazophorines group....
. 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
Systematic review
A systematic review is a literature review focused on a research question that tries to identify, appraise, select and synthesize all high quality research evidence relevant to that question. Systematic reviews of high-quality randomized controlled trials are crucial to evidence-based medicine...
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
Methotrexate
Methotrexate , abbreviated MTX and formerly known as amethopterin, is an antimetabolite and antifolate drug. It is used in treatment of cancer, autoimmune diseases, ectopic pregnancy, and for the induction of medical abortions. It acts by inhibiting the metabolism of folic acid. Methotrexate...
.
Montelukast
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, montelukastMontelukast
Montelukast is a leukotriene receptor antagonist used for the maintenance treatment of asthma and to relieve symptoms of seasonal allergies. It is usually administered orally...
." Researchers have searched for links between drugs such as montelukast
Montelukast
Montelukast is a leukotriene receptor antagonist used for the maintenance treatment of asthma and to relieve symptoms of seasonal allergies. It is usually administered orally...
(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
Asthma
Asthma is the common chronic inflammatory disease of the airways characterized by variable and recurring symptoms, reversible airflow obstruction, and bronchospasm. Symptoms include wheezing, coughing, chest tightness, and shortness of breath...
receiving leukotriene modifiers appears to be related to unmasking of an underlying vasculitic syndrome
Vasculitis
Vasculitis refers to a heterogeneous group of disorders that are characterized by inflammatory destruction of blood vessels. Both arteries and veins are affected. Lymphangitis is sometimes considered a type of vasculitis...
that is initially clinically recognized as moderate to severe asthma and treated with corticosteroids. Montelukast
Montelukast
Montelukast is a leukotriene receptor antagonist used for the maintenance treatment of asthma and to relieve symptoms of seasonal allergies. It is usually administered orally...
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
The memoir PatientPatient (memoir)
Patient is the name of a 192-page memoir by musician Ben Watt. It was published May 1, 1997 by Penguin Books . The book dealt largely with Watt's experience with a rare disease, Churg-Strauss syndrome, and his recovery....
, by the musician Ben Watt
Ben Watt
Benjamin Brian Thomas Watt is a British musician, DJ, and record producer, best known as one half of the duo, Everything but the Girl.-Family:...
, 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
Umaru Musa Yar'Adua
Umaru Musa Yar'Adua was the President of Nigeria and the 13th Head of State. He served as governor of Katsina State in northern Nigeria from 29 May 1999 to 28 May 2007. He was declared the winner of the controversial Nigerian presidential election held on 21 April 2007, and was sworn in on 29 May...
, the president of Nigeria
Nigeria
Nigeria , officially the Federal Republic of Nigeria, is a federal constitutional republic comprising 36 states and its Federal Capital Territory, Abuja. The country is located in West Africa and shares land borders with the Republic of Benin in the west, Chad and Cameroon in the east, and Niger in...
from 2007–2010, reportedly suffered from Churg–Strauss syndrome and died in office of complications of the disease.
The DJ and author Charlie Gillett
Charlie Gillett
Charlie Gillett , was a British radio presenter, musicologist and writer, mainly on rock and roll and other forms of popular music...
was diagnosed with Churg-Strauss in 2006; he died four years later.
External links
- Churg-Strauss syndrome - MedLink Neurology Clinical Summary