[Successful treatment of clinical manifestations of Muckle-Wells syndrome with anakinra].
METHODS
A 58-year-old woman and her 18-year-old daughter were first seen in our rheumatology centre after having experienced many years of periodic fever, arthralgia and urticaria. Some months ago a diagnosis of Muckle-Wells syndrome (MWS) had been made and confirmed by genetic testing. The mother had developed partial deafness and substantial loss of vision. Her daughter had been suffering mainly from urticaria and fever at least once daily.


METHODS
Because of the established role of interleukin (IL)-1 in this hereditary disease and some positive case reports, we decided to treat these patients with the IL-1 receptor antagonist anakinra. The patients reported a great success of this treatment with virtually complete absence of any acute MWS-associated symptoms.


CONCLUSIONS
Therapy of MWS with anakinra seems to be highly efficacious for several clinical manifestations of this disease, including laboratory markers for inflammation. It is possible that organ destruction may be prevented by this medication.
Dybowski
F
F
Rheumazentrum Ruhrgebiet--St.-Josefs-Krankenhaus, Landgrafenstrasse 15, 44652 Herne.
Jakobs
B
B
Altmeyer
P
P
Braun
J
J
D000210:Acute-Phase Reaction / Q000276:immunology*; D000293:Adolescent; D028226:Amyloidosis, Familial / Q000188:drug therapy / Q000235:genetics; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D018771:Arthralgia / Q000209:etiology; D003231:Conjunctivitis / Q000235:genetics; D005221:Fatigue; D005260:Female; D034381:Hearing Loss / Q000235:genetics; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein; D007674:Kidney Diseases / Q000188:drug therapy / Q000235:genetics; D008875:Middle Aged; D009154:Mutation; D010375:Pedigree; D017472:Receptors, Interleukin-1 / Q000037:antagonists & inhibitors*; D012795:Sialoglycoproteins / Q000627:therapeutic use*; D013577:Syndrome; D016896:Treatment Outcome; D014581:Urticaria / Q000188:drug therapy* / Q000235:genetics; D015354:Vision, Low / Q000235:genetics
D018501:Antirheumatic Agents; C507763:IL1RN protein, human; D053590:Interleukin 1 Receptor Antagonist Protein; D017472:Receptors, Interleukin-1; D012795:Sialoglycoproteins