Abstract
Background and purpose
Schnitzler syndrome is characterized by chronic urticaria, monoclonal gammopathy, and a risk of developing lymphoproliferative disorders. Patients frequently present with bone pain, fever, arthralgia, and lymphadenopathy. The purpose of this study is to retrospectively review and evaluate potentially attributable imaging abnormalities in a series of patients with clinically diagnosed Schnitzler syndrome.
Materials and methods
Clinical and pathological chart review identified 22 patients meeting Strasbourg criteria for Schnitzler syndrome. Imaging of these patients was retrospectively reviewed by a musculoskeletal radiologist and a radiology fellow to determine and characterize significant “positive” findings that could potentially be attributed to their primary disease process.
Results
Evidence of abnormal bone remodeling was present in 64 % (14/22) of patients with 40 bone abnormalities. Lesions were predominantly or entirely sclerotic and most commonly involved the distal femora (n = 11), proximal tibiae (nine), and innominate bones (six). Patterns of involvement were variable, but typically eccentrically intramedullary, cortical, and juxta-articular. Skeletal surveys and targeted radiographs identified only 37 % (10/27) of abnormal bones subsequently “positive” by additional modalities. PET and bone scans were all positive in patients with bone sclerosis (13/13 and 17/17 bone sites, respectively).
Conclusions
Schnitzler syndrome often presents with characteristic osteosclerosis, most commonly around the knees and in the pelvis. In patients with a suggestive clinical history, the radiologist could direct an appropriate imaging strategy or might to be the first to suggest the diagnosis. Bone scan may be the most appropriate initial screening tool in suspected cases.
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References
Lecompte M, Blais G, Bisson G, Maynard B. Schnitzler's syndrome. Skeletal Radiol. 1998;27(5):294–6.
Schnitzler LSB, Boasson M, Gardais J, Tourmen A. Urticaire chronique, lésions osseuses, macroglobulinémie IgM: maladie de Waldenström? Bull Soc Fr Dermatol Syphiligr. 1974;81:363.
Simon A, Asli B, Braun-Falco M, De Koning H, Fermand JP, Grattan C, et al. Schnitzler's syndrome: diagnosis, treatment, and follow-up. Allergy. 2013;68(5):562–8.
Vandenhende MA, Bentaberry F, Morlat P, Bonnet F. Anakinra: an effective treatment in the Schnitzler syndrome. Joint Bone Spine. 2011;78(6):636–7.
Besada E, Nossent H. Dramatic response to IL1-RA treatment in longstanding multidrug resistant Schnitzler's syndrome: a case report and literature review. Clin Rheumatol. 2010;29(5):567–71.
Jain T, Offord CP, Kyle R, Dingli D. Schnitzler syndrome - an under diagnosed clinical entity. Haematologica. 2013;98(10):1581–5.
Florez AF, Gallardo Agromayor E, Garcia-Barredo R, Landeras Alvaro RM, Gorrino Angulo M, Martinez-Taboada VM, et al. Radiological aid to clinical diagnosis of Schnitzler's syndrome: multimodality imaging approach. Clin Rheumatol. 2008;27(1):107–10.
Bertrand A, Feydy A, Belmatoug N, Fantin B. Schnitzler's syndrome: 3-year radiological follow-up. Skeletal Radiol. 2007;36(2):153–6.
De Waele S, Lecouvet FE, Malghem J, Jamar F, Lambert M. Schnitzler's syndrome: an unusual cause of bone pain with suggestive imaging features. AJR Am J Roentgenol. 2000;175(5):1325–7.
Singh B, Ezziddin S, Rabe E, Biersack HJ, Ahmadzadehfar H. Bone scan appearance supportive of Schnitzler's syndrome: report of two new cases. Clin Nucl Med. 2006;31(3):151–3.
Sokumbi O, Drage LA, Peters MS. Clinical and histopathologic review of Schnitzler syndrome: the Mayo Clinic experience (1972–2011). J Am Acad Dermatol. 2012;67(6):1289–95.
Terpos E, Asli B, Christoulas D, Brouet JC, Kastritis E, Rybojad M, et al. Increased angiogenesis and enhanced bone formation in patients with IgM monoclonal gammopathy and urticarial skin rash: new insight into the biology of Schnitzler syndrome. Haematologica. 2012;97(11):1699–703.
Zanglis A, Valsamaki P, Fountos G. Erdheim-Chester disease: symmetric uptake in the (99 m)Tc-MDP bone scan. Hell J Nucl Med. 2008;11(3):164–7.
Spyridonidis TJ, Giannakenas C, Barla P, Apostolopoulos DJ. Erdheim-Chester disease: a rare syndrome with a characteristic bone scintigraphy pattern. Ann Nucl Med. 2008;22(4):323–6.
Nunez R, Tronco GG, Rini JN, Hofman J, Amoashiy M, Bhuiya T, et al. Radionuclide bone imaging in Erdheim-Chester disease. Clin Nucl Med. 2005;30(1):32–4.
Pena Pardo FJ, Banzo Marraco I, Quirce Pisano R, Hernandez Allende R, Carril Carril JM. Bone scintigraphy in Erdheim-Chester disease. Rev Esp Med Nucl. 2003;22(4):253–6.
Szturz P, Hlavaty L, Prasek J, Dvorakova D. Erdheim-Chester disease and Schnitzler syndrome: so near, and yet so far. Int J Hematol. 2013;98(4):379–81.
de Koning HD, Bodar EJ, van der Meer JW, Simon A. Schnitzler syndrome: beyond the case reports: review and follow-up of 94 patients with an emphasis on prognosis and treatment. Semin Arthritis Rheum. 2007;37(3):137–48.
Li J, Zhou DB. New advances in the diagnosis and treatment of POEMS syndrome. Br J Haematol. 2013;161(3):303–15.
Dispenzieri A, Kyle RA, Lacy MQ, Rajkumar SV, Therneau TM, Larson DR, et al. POEMS syndrome: definitions and long-term outcome. Blood. 2003;101(7):2496–506.
Schrodl K, Nigg A, Treitl M, Flaig M, Jansson A, Schulze-Koops H, et al. Schnitzler's disease as an important differential diagnosis of chronic recurrent multifocal osteomyelitis: a case report. Case Rep Rheumatol. 2012;2012:318791.
Iyer RS, Thapa MM, Chew FS. Chronic recurrent multifocal osteomyelitis: review. AJR Am J Roentgenol. 2011;196(6 Suppl):S87–91.
Chen CC, Andrich MP, Mican JM, Metcalfe DD. A retrospective analysis of bone scan abnormalities in mastocytosis: correlation with disease category and prognosis. J Nucl Med. 1994;35(9):1471–5.
Launay D, Dutoit-Lefevre V, Faure E, Robineau O, Hauspie C, Sobanski V, et al. Effect of in vitro and in vivo anakinra on cytokines production in Schnitzler syndrome. PLoS One. 2013;8(3):e59327.
Krause K, Weller K, Stefaniak R, Wittkowski H, Altrichter S, Siebenhaar F, et al. Efficacy and safety of the interleukin-1 antagonist rilonacept in Schnitzler syndrome: an open-label study. Allergy. 2012;67(7):943–50.
Krause K, Feist E, Fiene M, Kallinich T, Maurer M. Complete remission in 3 of 3 anti-IL-6-treated patients with Schnitzler syndrome. J Allergy Clin Immunol. 2012;129(3):848–50.
Gutgemann I, Stevens K, Loftus D, Schmidt-Wolf IG, George TI. VEGF and osteosclerosis in POEMS syndrome. Ann Hematol. 2008;87(3):243–5.
Iagaru A, Young P, Mittra E, Dick DW, Herfkens R, Gambhir SS. Pilot prospective evaluation of 99mTc-MDP scintigraphy, 18 F NaF PET/CT, 18 F FDG PET/CT and whole-body MRI for detection of skeletal metastases. Clin Nucl Med. 2013;38(7):e290–6.
Acknowledgments
Special thanks to Olayema Sokumbi, M.D., for her assistance in clinical chart review.
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Niederhauser, B.D., Dingli, D., Kyle, R.A. et al. Imaging findings in 22 cases of Schnitzler syndrome: characteristic para-articular osteosclerosis, and the “hot knees” sign differential diagnosis. Skeletal Radiol 43, 905–915 (2014). https://doi.org/10.1007/s00256-014-1857-y
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DOI: https://doi.org/10.1007/s00256-014-1857-y