epidemic pleurodynia
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Bornholm disease, also known as epidemic pleurodynia, is a condition characterized by myositis of the abdomen or chest caused by the
Coxsackie B virus Coxsackie B is a group of six serotypes of coxsackievirus (CVB1-CVB6), a pathogenic enterovirus, that trigger illness ranging from gastrointestinal distress to full-fledged pericarditis and myocarditis (coxsackievirus-induced cardiomyopathy). Th ...
or other viruses. The myositis manifests as an intermittent stabbing pain in the musculature that is seen primarily in children and young adults. It is named after the
Danish Danish may refer to: * Something of, from, or related to the country of Denmark People * A national or citizen of Denmark, also called a "Dane," see Demographics of Denmark * Culture of Denmark * Danish people or Danes, people with a Danish a ...
island of Bornholm in the Baltic Sea where an outbreak was one of the first to be described.


Signs and symptoms

The expected symptoms of Bornholm disease include
fever Fever, also referred to as pyrexia, is defined as having a temperature above the normal range due to an increase in the body's temperature set point. There is not a single agreed-upon upper limit for normal temperature with sources using val ...
, pleuritic chest pain, or epigastric
abdominal pain Abdominal pain, also known as a stomach ache, is a symptom associated with both non-serious and serious medical issues. Common causes of pain in the abdomen include gastroenteritis and irritable bowel syndrome. About 15% of people have a m ...
that is frequently spasmodic. Bornholm associated chest pain is distinguished by attacks of severe pain in the lower chest, often on the right side. In a prior study, the episodes were shown to last five to ten minutes and then subside for thirty minutes. The pain is exacerbated by movement and makes walking and breathing more difficult. Patients have found relief from the pain by lying still for a brief period of time. The slightest movement of the rib cage causes a sharp increase in pain, which makes it difficult to breathe, although it generally passes off before any actual harm occurs. The attacks are unpredictable and strike "out of the blue" with a feeling like an iron grip around the rib cage. The colloquial names for the disease, such as ''the devil's grip'', (see "other names" below) reflect this symptom. Bornholm disease is a clinical diagnosis that uses the spasmodic pain,
fever Fever, also referred to as pyrexia, is defined as having a temperature above the normal range due to an increase in the body's temperature set point. There is not a single agreed-upon upper limit for normal temperature with sources using val ...
, and relapses to distinguish the illness from other potential causes of pain such as
appendicitis Appendicitis is inflammation of the appendix. Symptoms commonly include right lower abdominal pain, nausea, vomiting, and decreased appetite. However, approximately 40% of people do not have these typical symptoms. Severe complications of a ru ...
or
myocardial infarction A myocardial infarction (MI), commonly known as a heart attack, occurs when blood flow decreases or stops to the coronary artery of the heart, causing damage to the heart muscle. The most common symptom is chest pain or discomfort which may ...
. Tachycardia and arrhythmias have been found with Bornholm disease by using an electrocardiogram (ECG). Murmurs, rubs, and pericardial effusions have been detected on physical examination. Maculopapular rashes can also be present with Bornholm disease


Etiology

Inoculation of throat washings taken from people with this disease into the brains of newborn mice revealed that
enterovirus ''Enterovirus'' is a genus of positive-sense single-stranded RNA viruses associated with several human and mammalian diseases. Enteroviruses are named by their transmission-route through the intestine ('enteric' meaning intestinal). Serologic ...
es in the
Coxsackie B virus Coxsackie B is a group of six serotypes of coxsackievirus (CVB1-CVB6), a pathogenic enterovirus, that trigger illness ranging from gastrointestinal distress to full-fledged pericarditis and myocarditis (coxsackievirus-induced cardiomyopathy). Th ...
group were likely to be the cause of pleurodynia, and those findings were supported by subsequent studies of
IgM Immunoglobulin M (IgM) is one of several isotypes of antibody (also known as immunoglobulin) that are produced by vertebrates. IgM is the largest antibody, and it is the first antibody to appear in the response to initial exposure to an antig ...
antibody responses measured in serum from people with pleurodynia. Other viruses in the enterovirus family, including echovirus and
Coxsackie A virus Coxsackie A virus (CAV) is a cytolytic Coxsackievirus of the ''Picornaviridae'' family, an enterovirus (a group containing the polioviruses, coxsackieviruses, and echoviruses). Viral structure and genome Coxsackie A virus is a subgroup of ent ...
, are less frequently associated with pleurodynia. Echovirus types 1,6,8,9, and 19 and Coxsackie A virus types 4,6,9, and 10 are associated with Bornholm disease. The most common strains causing Bornholm disease are Coxsackie B3 and A9. Viral proliferation in the muscles of the chest wall, diaphragm, and abdomen are thought to contribute to the typical presentation that characterizes the illness.


Epidemiology

The most common cause of Bornholm disease, Coxsackie B virus, is shed in large amounts in the feces of infected persons and is spread primarily through the fecal-oral route. Respiratory secretions and oral-oral methods have also shown to be modes of transmission. In previous cases the disease has been spread by sharing drink containers, and has been contracted by laboratory personnel working with the virus. The pharynx is typically the initial site for entering the body, however the virus will proliferate in lymphatic tissues and use the blood stream to reach the muscles and produce symptoms. Preventative measures to decrease transmission of the virus causing Bornholm disease emphasize hand hygiene. In previous studies of Bornholm disease the majority of the patients affected were children.


Physical exam findings

In a studied case of Bornholm disease the chest pain was unable to be reproduced on palpation and failed to improve with changes in position. The pain was made worse during deep inhalation. A pleural rub was present, however lung auscultation was clear and rashes were absent.


Laboratory findings and imaging

In a prior case of Bornholm disease the laboratory results showed the white blood cell count,
hemoglobin Hemoglobin (haemoglobin BrE) (from the Greek word αἷμα, ''haîma'' 'blood' + Latin ''globus'' 'ball, sphere' + ''-in'') (), abbreviated Hb or Hgb, is the iron-containing oxygen-transport metalloprotein present in red blood cells (erythrocyt ...
,
hematocrit The hematocrit () (Ht or HCT), also known by several other names, is the volume percentage (vol%) of red blood cells (RBCs) in blood, measured as part of a blood test. The measurement depends on the number and size of red blood cells. It is norm ...
,
creatinine Creatinine (; ) is a breakdown product of creatine phosphate from muscle and protein metabolism. It is released at a constant rate by the body (depending on muscle mass). Biological relevance Serum creatinine (a blood measurement) is an importa ...
, liver function test (LFT),
troponin image:Troponin Ribbon Diagram.png, 400px, Ribbon representation of the human cardiac troponin core complex (52 kDa core) in the calcium-saturated form. Blue = troponin C; green = troponin I; magenta = troponin T.; ; rendered with PyMOL Troponin, ...
, and
creatine kinase Creatine kinase (CK), also known as creatine phosphokinase (CPK) or phosphocreatine kinase, is an enzyme () expressed by various tissues and cell types. CK catalyses the conversion of creatine and uses adenosine triphosphate (ATP) to create pho ...
(CK) were all within normal limits. The chest x-ray showed bilateral
pleural effusions A pleural effusion is accumulation of excessive fluid in the pleural space, the potential space that surrounds each lung. Under normal conditions, pleural fluid is secreted by the parietal pleural capillaries at a rate of 0.6 millilitre per kil ...
which resolved after infection. The
erythrocyte sedimentation rate The erythrocyte sedimentation rate (ESR or sed rate) is the rate at which red blood cells in anticoagulated whole blood descend in a standardized tube over a period of one hour. It is a common hematology test, and is a non-specific measure of ...
(ESR) and
C-reactive protein C-reactive protein (CRP) is an annular (ring-shaped) pentameric protein found in blood plasma, whose circulating concentrations rise in response to inflammation. It is an acute-phase protein of hepatic origin that increases following interleukin- ...
(CRP) levels were found to be elevated. The electrocardiogram (EKG) did not show any abnormalities related to ischemia.


Treatment and prognosis

Treatment is symptomatic and includes the administration of non-steroidal anti-inflammatory agents or the application of heat to the affected muscles. Intercostal 2% xylocaine injections with normal saline have been used to relieve symptoms in certain cases. Relapses during the weeks following the initial episode are a characteristic feature of this disease. Bornholm disease typically lasts between one day and one week with an average illness duration of four days. In 20% of cases studied, the illness lasted between one and two weeks. The illness in children was found to be shorter than the illness in adults. Patients typically make a complete recovery with supportive care. Although recovering from Bornholm disease is expected, some rare complications include myocarditis,
respiratory failure Respiratory failure results from inadequate gas exchange by the respiratory system, meaning that the arterial oxygen, carbon dioxide, or both cannot be kept at normal levels. A drop in the oxygen carried in the blood is known as hypoxemia; a rise ...
, hepatic necrosis with coagulopathy, and
disseminated intravascular coagulopathy Disseminated intravascular coagulation (DIC) is a condition in which blood clots form throughout the body, blocking small blood vessels. Symptoms may include chest pain, shortness of breath, leg pain, problems speaking, or problems moving parts ...
(DIC). Aseptic meningitis, pericarditis and pleurisy are also known potential complications of Bornholm disease. Another uncommon complication is
orchitis Orchitis is inflammation of the testes. It can also involve swelling, pains and frequent infection, particularly of the epididymis, as in epididymitis. The term is from the Ancient Greek ὄρχις meaning "testicle"; same root as ''orchid''. S ...
that manifests as unilateral testicular pain and swelling in the days or weeks following the expected symptoms of Bornholm disease.


Differential diagnoses

Acute appendicitis Appendicitis is inflammation of the appendix. Symptoms commonly include right lower abdominal pain, nausea, vomiting, and decreased appetite. However, approximately 40% of people do not have these typical symptoms. Severe complications of a rupt ...
,
cholecystitis Cholecystitis is inflammation of the gallbladder. Symptoms include right upper abdominal pain, pain in the right shoulder, nausea, vomiting, and occasionally fever. Often gallbladder attacks (biliary colic) precede acute cholecystitis. The pai ...
,
pancreatitis Pancreatitis is a condition characterized by inflammation of the pancreas. The pancreas is a large organ behind the stomach that produces digestive enzymes and a number of hormones. There are two main types: acute pancreatitis, and chronic pancr ...
,
pulmonary embolism Pulmonary embolism (PE) is a blockage of an artery in the lungs by a substance that has moved from elsewhere in the body through the bloodstream ( embolism). Symptoms of a PE may include shortness of breath, chest pain particularly upon breathin ...
, acute coronary syndrome,
costochondritis Costochondritis, also known as chest wall pain syndrome or costosternal syndrome, is a benign inflammation of the upper costochondral (rib to cartilage) and sternocostal (cartilage to sternum) joints. 90% of patients are affected in multiple ri ...
, amongst others


History

In 1872, Anders Daae and Christian Horrebow Homann reported an epidemic of pleurodynia occurring in the community of
Bamble Bamble is a municipality in Vestfold og Telemark county, Norway. It is part of the traditional region of Grenland. The administrative centre of the municipality is the village of Langesund. General information Name The Old Norse form of the na ...
,
Norway Norway, officially the Kingdom of Norway, is a Nordic country in Northern Europe, the mainland territory of which comprises the western and northernmost portion of the Scandinavian Peninsula. The remote Arctic island of Jan Mayen and the ...
, giving rise to the name "Bamble disease". Subsequent reports, published only in Norwegian, referred to the disease by this name.
Niels Ryberg Finsen Niels Ryberg Finsen (15 December 1860 – 24 September 1904) was a Faroese- Icelandic physician and scientist. In 1903, he was awarded the Nobel Prize in Medicine and Physiology "in recognition of his contribution to the treatment of diseases, e ...
also described the disease in Iceland in 1874. In 1933,
Ejnar Sylvest Ejnar Sylvest (1880 – 1972) was a Danish family physician who described Bornholm disease or the "devil's grip" following an outbreak affecting 23 fisherman in Melstedgård and Gudhjem Gudhjem is a small town and fishing port on the northern co ...
gave a doctoral thesis describing a Danish outbreak of this disease on Bornholm Island entitled "Bornholm disease-myalgia epidemica", and this name has persisted. In 1949 the Coxsackie B virus was isolated and established as an etiology of Bornholm disease.


Other names

Bornholm disease is also known as Bamble disease, the devil's grip, devil's grippe, epidemic myalgia, epidemic pleurodynia.


References


External links


Bornholm Disease
on patient.co.uk {{Viral diseases Enterovirus-associated diseases Bornholm