Successful Anakinra Therapy in 2 Patients with Schnitzler Syndrome

SZTURZ, Petr, Zdeněk ADAM, Roman HÁJEK and Jiří MAYER. Successful Anakinra Therapy in 2 Patients with Schnitzler Syndrome. Onkologie. Switzerland: Karger, 2011, vol. 34, No 5, p. 265-268. ISSN 0378-584X. Available from: https://dx.doi.org/10.1159/000327816.

Basic information

• Original name: Successful Anakinra Therapy in 2 Patients with Schnitzler Syndrome
• Name (in English): Successful Anakinra Therapy in 2 Patients with Schnitzler Syndrome
• Authors: SZTURZ, Petr (203 Czech Republic, guarantor, belonging to the institution), Zdeněk ADAM (203 Czech Republic, belonging to the institution), Roman HÁJEK (203 Czech Republic, belonging to the institution) and Jiří MAYER (203 Czech Republic, belonging to the institution).
• Edition: Onkologie, Switzerland, Karger, 2011, 0378-584X.

Other information

• Original language: Czech
• Type of outcome: Article in a journal
• Field of Study: 30200 3.2 Clinical medicine
• Country of publisher: Germany
• Confidentiality degree: is not subject to a state or trade secret
• WWW: URL
• Impact factor: Impact factor: 0.868
• RIV identification code: RIV/00216224:14110/11:00052239
• Organization unit: Faculty of Medicine
• Doi: http://dx.doi.org/10.1159/000327816
• UT WoS: 000290560200009
• Keywords in English: Schnitzler syndrome; Monoclonal gammopathy; Urticaria; Interleukin; Anakinra
• Tags: International impact

Abstract

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).

Abstract (in English)

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).

Links

• LC06027, research and development project: Name: Univerzitní výzkumné centrum - Česká myelomová skupina (Acronym: LC MGUS)

Investor: Ministry of Education, Youth and Sports of the CR, University Research Centre - Czech Myeloma Group

• MSM0021622434, plan (intention): Name: Od klasických prognostických markerů ke klinicky aplikovatelným farmakogenomickým a farmakoproteomickým projektům u mnohočetného myelomu a monoklonálních gamapatií

Investor: Ministry of Education, Youth and Sports of the CR, From classic prognostic markers to clinical applications in selected pharmacogenomic and pharmacoproteomic projects in multiple myeloma and monoclonal gammapathies

• MUNI/A/1012/2009, interní kód MU: Name: Optimalizace diagnostiky a terapie maligních chorob a komplikací, které tyto maligní nemoci provázejí, s využitím nových molekulárně biologických metod.

Investor: Masaryk University, Category A