2011
Successful Anakinra Therapy in 2 Patients with Schnitzler Syndrome
SZTURZ, Petr, Zdeněk ADAM, Roman HÁJEK a Jiří MAYERZákladní údaje
Originální název
Successful Anakinra Therapy in 2 Patients with Schnitzler Syndrome
Název anglicky
Successful Anakinra Therapy in 2 Patients with Schnitzler Syndrome
Autoři
SZTURZ, Petr (203 Česká republika, garant, domácí), Zdeněk ADAM (203 Česká republika, domácí), Roman HÁJEK (203 Česká republika, domácí) a Jiří MAYER (203 Česká republika, domácí)
Vydání
Onkologie, Switzerland, Karger, 2011, 0378-584X
Další údaje
Jazyk
čeština
Typ výsledku
Článek v odborném periodiku
Obor
30200 3.2 Clinical medicine
Stát vydavatele
Německo
Utajení
není předmětem státního či obchodního tajemství
Odkazy
Impakt faktor
Impact factor: 0.868
Kód RIV
RIV/00216224:14110/11:00052239
Organizační jednotka
Lékařská fakulta
UT WoS
000290560200009
Klíčová slova anglicky
Schnitzler syndrome; Monoclonal gammopathy; Urticaria; Interleukin; Anakinra
Příznaky
Mezinárodní význam
Změněno: 15. 10. 2012 15:27, Mgr. Michal Petr
V originále
Background: Schnitzler syndrome is a rare idiopathic disease characterized by chronic urticaria, presence of monoclonal IgM immunoglobulin, signs of inflammation and further symptoms. Often unrecognized, it may evolve into a lymphoproliferative disorder. Case Report: Two male patients presented pruritic and nonpruritic urticarias at the age of 45 and 43 years, respectively. Based on further symptoms (fever or subfebrile temperatures, bone and joint pains), laboratory tests (monoclonal IgM kappa, leukocytosis, elevated ESR and CRP) and radiological findings (osteolytic-osteosclerotic and hyperostotic skeletal changes), they were subsequently diagnosed with Schnitzler syndrome. Follow-up times were 15 years and 1 year, respectively. We monitored disease activity in the second patient, finding a correlation with CRP, ESR and interleukin IL-6 and IL-18 serum levels. As regards therapy, we used several medications (antihistamines, bisphosphonates, corticoids, 2-chlorodeoxyadenosine, interferon-alpha, cyclosporine, thalidomide, bortezomib) and PUVA treatment without achieving complete remission. Only anakinra (interleukin-1 receptor antagonist) minimized Schnitzler symptoms in both patients with very good drug tolerance. The first patient has been on anakinra monotherapy for 3 years (10/2007-10/2010) at a dosing interval of 24-48 hours without any signs of Schnitzler syndrome. Conclusions: Biological therapy using anakinra proved effective in Schnitzler syndrome, a rare entity with potentially life-threatening complications (malignancy, systemic amyloidosis).
Anglicky
Background: Schnitzler syndrome is a rare idiopathic disease characterized by chronic urticaria, presence of monoclonal IgM immunoglobulin, signs of inflammation and further symptoms. Often unrecognized, it may evolve into a lymphoproliferative disorder. Case Report: Two male patients presented pruritic and nonpruritic urticarias at the age of 45 and 43 years, respectively. Based on further symptoms (fever or subfebrile temperatures, bone and joint pains), laboratory tests (monoclonal IgM kappa, leukocytosis, elevated ESR and CRP) and radiological findings (osteolytic-osteosclerotic and hyperostotic skeletal changes), they were subsequently diagnosed with Schnitzler syndrome. Follow-up times were 15 years and 1 year, respectively. We monitored disease activity in the second patient, finding a correlation with CRP, ESR and interleukin IL-6 and IL-18 serum levels. As regards therapy, we used several medications (antihistamines, bisphosphonates, corticoids, 2-chlorodeoxyadenosine, interferon-alpha, cyclosporine, thalidomide, bortezomib) and PUVA treatment without achieving complete remission. Only anakinra (interleukin-1 receptor antagonist) minimized Schnitzler symptoms in both patients with very good drug tolerance. The first patient has been on anakinra monotherapy for 3 years (10/2007-10/2010) at a dosing interval of 24-48 hours without any signs of Schnitzler syndrome. Conclusions: Biological therapy using anakinra proved effective in Schnitzler syndrome, a rare entity with potentially life-threatening complications (malignancy, systemic amyloidosis).
Návaznosti
| LC06027, projekt VaV |
| ||
| MSM0021622434, záměr |
| ||
| MUNI/A/1012/2009, interní kód MU |
|