Kawasaki syndrome

from Wikipedia, the free encyclopedia
Classification according to ICD-10
M30.3 mucocutaneous lymph node syndrome (MCLS)
ICD-10 online (WHO version 2019)

The Kawasaki syndrome or mucocutaneous lymph node syndrome ( MCLS ) is an acute , febrile , systemic disease characterized by inflammation ( necrotizing vasculitis ) of the small and medium-sized arteries is identified. In addition, systemic inflammation is present in many organs. The cause is unknown; one suspects an infectious development, which is favored by a hereditary basis. Kawasaki syndrome mostly affects young children. The disease is named after the Japanese pediatrician Tomisaku Kawasaki .

Epidemiology

In Germany, around 9 out of 100,000 children under the age of five fall ill every year, while the incidence of Kawasaki syndrome in Japan in the same age group is around 185 out of 100,000. 75 percent of all patients are younger than five years, and children very often fall ill in their second year of life. Boys are affected by the disease about one and a half times as often as girls. In the northern hemisphere there are more diseases in winter and spring than in summer and autumn; in contrast, in tropical regions there is no seasonality .

Since April 2020 a new type of syndrome has been observed in the USA and some European countries, which is similar to Kawasaki syndrome and which has been given the name MIS-C (multisystem inflammatory syndrome in children). MIS-C may be related to infections caused by the SARS-CoV-2 pathogen. In May 2020, the WHO called for MIS-C to be monitored and researched. By mid-May 2020, 103 children in New York State alone had been treated with symptoms similar to Kawasaki syndrome. Of these children, 60% tested positive for the COVID-19 disease caused by the pathogen, while antibodies against SARS-CoV-2 were detectable in the remaining 40%. The age group from 5 to 14 years is particularly affected.

Clinical manifestations

Expressions of the Kawasaki syndrome

There are three phases:

  1. The acute febrile period : it lasts up to ten days. The fever usually starts suddenly and the typical symptoms develop over three to four days, which can then lead to a diagnosis of Kawasaki syndrome (see Diagnostic criteria below).
  2. The subacute phase : it lasts two to four weeks. Scaling on the hands and feet is typical.
  3. The convalescence phase : It can last months with occasional tiredness and poor performance.

Main symptoms

100% fever
Longer than 5 days, antibiotic-resistant, can be reduced with antipyretics , with recurring peaks above 40 ° C, lasting about 10 days without therapy.
90% symptoms of the oral cavity, lips
Dry, often swollen, bright red, chapped lips, strawberry tongue ( lacquer tongue ), intense reddening of the oral mucosa and throat, lasting as long as the fever.
85% conjunctivitis
Usually bilateral redness of the conjunctiva, not purulent, painless, starts shortly after the fever, lasts about 7 days.
80% exanthem
Diverse, mostly non-itchy, trunk-accentuated skin rash without blisters occurs within 5 days of the onset of the fever ("colorful picture", often similar to measles or scarlet fever , but sometimes also similar to Henoch-Schönlein purpura ).
70% hands and feet
Acute redness and painful swellings in the palms and soles of the feet. From the second to third week of the disease, flaking of the skin that begins on the tips of the fingers and toes and can spread over the entire palms of the hands and feet.
70% enlargement of the neck lymph nodes
Acute, non-purulent, slightly painful swelling of the cervical lymph nodes, often on one side in the anterior triangle of the neck with a diameter of more than 1.5 cm.

Side symptoms

Cardiovascular symptoms
These occur mainly in the early phase and are largely responsible for long-term morbidity and mortality : myocarditis (50%), pericarditis , heart valve problems , aneurysms of the coronary vessels and other arteries, Raynaud's symptoms.
Neurological symptoms
Often pronounced irritability, non-infectious meningitis , central hearing loss.
respiratory tract
Cough, runny nose, hoarseness.
Abdominal symptoms (in 30% of all patients)
Abdominal pain, diarrhea, vomiting, paralytic ileus , gallbladder hydrops ( can be detected by sonography in 15% of all patients ), enlargement of the liver and spleen.
Urinary tract
Urethritis with painful urination and white blood cells in the urine.
Joints
Pain and, less often, inflammation in multiple joints in the first week of the disease, when these symptoms begin after the 10th day, more in large, weight-bearing joints.
eyes
Anterior uveitis , which quickly improves and heals without consequences.

Investigation methods

The diagnosis of complete Kawasaki syndrome is made clinically and requires fever and four out of five of the other criteria mentioned, as well as excluding other conditions with similar symptoms. Then the diagnosis can be made after five days of fever. Children who do not meet these criteria are diagnosed as so-called incomplete Kawasaki syndrome if the side symptoms and / or laboratory findings listed below apply partially or predominantly. This is more common in children under one year of age and is particularly problematic here because of the increased risk of coronary artery aneurysms.

Laboratory findings cannot prove or disprove the disease; they can make the suspected diagnosis more likely or less likely in the case of ambiguous clinical signs:

serology
AECA (anti-endothelial cell antibodies)
Leukocytes
Left-shift leukocytosis (50% of patients have> 15 leukocytes / nl).
Erythrocyte sedimentation rate , CRP
Often increased, sometimes greatly increased.
hemoglobin
Increasing anemia with prolonged illness.
Platelets
Strongly increased number, often from the second to third week of the disease, then also> 1,000 platelets / nl.
Transaminases
Often slightly elevated serum levels.
albumin
Decreased, more pronounced with longer and severe illness.
sodium
Decreased, possibly also as an expression of increased ADH secretion.
urine
Sterile leukocyturia.
Liquor
In 30 to 50% more monocytes , often without an increase in protein.
Synovial fluid
Sterile,> 100,000 leukocytes / ml.

New marker in the urine . In 2012, American researchers succeeded in finding markers in the urine of affected children that make the disease more quickly diagnosable. These biomarkers are filamin C (excreted from necrotic heart and skeletal muscle cells in the urine) and meprin A (enzyme of the inflammatory reaction). Diagnosis based on these markers was made with 98% accuracy in a study of 107 patients.

Differential diagnoses

Viral and bacterial diseases, leptospira , systemic form of juvenile idiopathic arthritis , polyarteritis nodosa , mercury poisoning . There are numerous other possibilities for confusion with incomplete Kawasaki syndrome. If the cause of the fever is unclear , the differential diagnosis of Kawasaki syndrome should be considered at an early stage , and in addition to laboratory tests, an echocardiography should be carried out to examine the coronary arteries.

Complications

Coronary arteries

Calcified, aneurysmally dilated coronary vessels

The long-term course of the disease depends on whether changes occur in the coronary arteries. One or more aneurysms develop in about 25% of untreated children . In this case, a light blood thinner such as acetylsalicylic acid (100 mg) is recommended for children every day. About half of these aneurysms regress within a year. About 20% of patients develop stenoses over the course of years , which, if left untreated, lead to heart attacks in almost half of the cases .

therapy

The disease is usually treated as an inpatient. The aim of the therapy is to reduce inflammation and avoid coronary artery aneurysms, which usually occur in the second to third week. It could be shown that therapy could reduce the occurrence of coronary artery changes from 25% to 2 to 4%. Therefore, starting therapy before the tenth day is crucial for a favorable course. The initial therapy is:

  • Immunoglobulins : 2 g / kg body weight in twelve hours as an infusion , possibly a second time after a very early dose and an unsatisfactory response.
  • Acetylsalicylic acid (ASA) in high doses (30 to 100 mg / kg body weight / day) for 14 days until the acute inflammation subsides. The dose is controversial.
  • Whether cortisone-like drugs are helpful for “therapy failures” is still the subject of research; they probably show at least an additive effect. Pulse cortisone treatment was no better than placebo.

Long-term therapy afterwards is recommended with acetylsalicylic acid 3 to 5 mg / (kg * day) for about six to eight weeks; further measures depend on the occurrence of coronary aneurysms : As long as an aneurysm is present, the low dose of ASA should never be discontinued become. If a large aneurysm with constrictions has formed, blood clotting should be inhibited using other medications such as phenprocoumon . Bypasses etc. may also come into question.

Long-term expectations

The acute illness usually heals without complications . If complications occur in the coronary arteries , according to current estimates, the risk of arteriosclerosis is increased due to the changed vascular wall . For this reason, Kawasaki patients should undergo long-term cardiac follow-up. Another symptom is the body's poor performance when exercising ( sport ).

history

The Kawasaki syndrome has been observed in Japan since 1961 and described as an independent clinical picture in 1967 by the doctor Tomisaku Kawasaki . A study from 2011 links the disease to air currents from the sea. The trailblazer for the discovery of Kawasaki syndrome in Germany was Professor Dr. Hansjörg Cremer.

See also

literature

Web links

Commons : Kawasaki Syndrome  - Collection of Pictures, Videos, and Audio Files

Individual evidence

  1. ^ Willibald Pschyrembel: Clinical Dictionary , 266th edition, de Gruyter Verlag, Berlin / Boston 2014, ISBN 978-3-11-033997-0 , p. 1081.
  2. ↑ Peter Reuter: Springer Clinical Dictionary 2007/2008 , 1st edition, Springer Medizin Verlag, Heidelberg 2007, ISBN 978-3-540-34601-2 , p. 1098 (keyword: lymph node syndrome, mucocutaneous).
  3. ^ Maxim Zetkin , Herbert Schaldach: Lexikon der Medizin , 16th edition, Ullstein Medical, Wiesbaden 1999, ISBN 3-86126-126-X , p. 1026.
  4. ^ Roche Lexicon Medicine , 5th edition, Urban & Fischer, Munich / Jena 2003, ISBN 3-437-15156-8 , p. 986.
  5. ↑ H. Cremer: Kawasaki Syndrome . In: Dieter Adam, HW Doerr, H. Link, H. Lode (eds.): Die Infektiologie . Springer, Berlin 2004, ISBN 3-540-00075-5 , pp. 1208 .
  6. Jump up ↑ Y. Nakamura, M. Yashiro, R. Uehara, I. Oki, M. Watanabe, H. Yanagawa: Epidemiologic features of Kawasaki disease in Japan: results from the nationwide survey in 2005-2006 . In: Journal of Epidemiology . Vol. 18, No. 4 , 2008, p. 167-172 , PMID 18635901 .
  7. ↑ JC Burns, L. Herzog, O. Fabri, AH Tremoulet, X. Rodo, R. Uehara, D. Burgner, E. Bainto, D. Pierce, M. Tyree, D. Cayan: Seasonality of Kawasaki disease: a global perspective . In: PLoS One . Vol. 8, No. 9 . Springer, 2014, p. e74529 , PMC 3776809 (free full text).
  8. ↑ www.who.int: Multisystem inflammatory syndrome in children and adolescents with COVID-19 (May 15, 2020)
  9. ↑ COVID-19: Reports of Kawasaki Syndrome in Children. In: Deutsches Ärzteblatt. May 4, 2020, accessed May 15, 2020 .
  10. ↑ Novel inflammation in corona. In: Tagesschau.de. May 14, 2020, accessed May 20, 2020 .
  11. ↑ Gerd Harald Herold et al .: Internal Medicine 2020 , Selbstverlag , Cologne 2020, ISBN 978-3-9814660-9-6 , p. 690.
  12. ↑ Kawasaki disease: urine markers found for reliable diagnosis. on: aerzteblatt.de , December 20, 2012.
  13. ↑ A. Kentsis et al.: Urine proteomics for discovery of improved diagnostic markers of Kawasaki disease. In: EMBO Molecular Medicine. doi: 10.1002 / emmm.201201494 .
  14. ↑ Kawasaki syndrome. University Children's Hospital Zurich, 2016, accessed July 2020 .
  15. ↑ Tomisaku Kawasaki : Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children . In: Arerugī . tape 16 , 1967, p. 178-222 .
  16. ↑ Xavier Rodó, Joan Ballester, Dan Cayan, Marian Melish, Yoshikazu Nakamura, Ritei Uehara, Jane Burns: Association of Kawasaki disease with tropospheric wind patterns. In: Scientific reports . tape 1 , 2011, ISSN  2045-2322 , p. 152 , doi : 10.1038 / srep00152 , PMID 22355668 , PMC 3240972 (free full text).
  17. ↑ COVID-19: Kawasaki syndrome in children is an independent disease Ärzteblatt, June 9, 2020