NASAL TRANSCRIPTOME IN GPA
6. Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 1990;33:1101–7. 7. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990;33:1094–100. 8. Sridhar S, Schembri F, Zeskind J, Shah V, Gustafson AM, Steiling K, et al. Smoking-induced gene expression changes in the bronchial airway are reflected in nasal and buccal epithelium. BMC Genomics 2008;9:259. 9. Spira A, Beane J, Pinto-Plata V, Kadar A, Liu G, Shah V, et al. Gene expression profiling of human lung tissue from smokers with severe emphysema. Am J Respir Cell Mol Biol 2004;31:601–10. 10. Aubry MC. Necrotizing granulomatous inflammation: what does it mean if your special stains are negative? Mod Pathol 2012;25 Suppl 1:S31–8. 11. Laudien M, Hasler R, Wohlers J, Bock J, Lipinski S, Bremer L, et al. Molecular signatures of a disturbed nasal barrier function in the primary tissue of Wegener’s granulomatosis. Mucosal Immunol 2011;4:564–73. 12. Schreiber A, Kettritz R. The neutrophil in antineutrophil cytoplasmic autoantibody-associated vasculitis. J Leukoc Biol 2013;94:623–31.
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13. Bleharski JR, Kiessler V, Buonsanti C, Sieling PA, Stenger S, Colonna M, et al. A role for triggering receptor expressed on myeloid cells-1 in host defense during the early-induced and adaptive phases of the immune response. J Immunol 2003;170: 3812–8. 14. Daikeler T, Regenass S, Tyndall A, Gencay MM, Roth M, Christ-Crain M, et al. Increased serum levels of soluble triggering receptor expressed on myeloid cells-1 in antineutrophil cytoplasmic antibody-associated vasculitis. Ann Rheum Dis 2008;67: 723–4. 15. Lyons PA, Rayner TF, Trivedi S, Holle JU, Watts RA, Jayne DR, et al. Genetically distinct subsets within ANCA-associated vasculitis. N Engl J Med 2012;367:214–23. 16. Kilty SJ, Bosse Y, Cormier C, Endam LM, Desrosiers MY. Polymorphisms in the SERPINA1 (a1-antitrypsin) gene are associated with severe chronic rhinosinusitis unresponsive to medical therapy. Am J Rhinol Allergy 2010;24:e4–9. 17. Rotta G, Matteoli G, Mazzini E, Nuciforo P, Colombo MP, Rescigno M. Contrasting roles of SPARC-related granuloma in bacterial containment and in the induction of anti-Salmonella typhimurium immunity. J Exp Med 2008;205:657–67. 18. Oh Y, Oh I, Morimoto J, Uede T, Morimoto A. Osteopontin has a crucial role in osteoclast-like multinucleated giant cell formation. J Cell Biochem 2014;115:585–95.
DOI: 10.1002/art.39172
Clinical Images: Chloroma—an uncommon cause of arthritis
The patient, a 51-year-old man, was referred to a rheumatologist for acute right elbow pain. He had a 1-month history of a testicular mass. Physical examination revealed synovitis of the elbow, with a swollen joint and total functional disability of the arm. He had no other arthritis or pain, no fever, and no systemic symptoms. Multiple foci of increased fluorodeoxyglucose (FDG) uptake throughout the skeleton, corresponding to areas of bone condensation in the shoulders, pelvis, and femurs, was seen on 18F-FDG–positron emission tomography integrated with computed tomography (PET/CT) (A). Intense 18F-FDG uptake was also detected in the right elbow joint. Articular involvement was particularly evident when CT volume rendering techniques and PET maximum intensity projections was used in combination (D). Arthrocentesis revealed orange-colored turbid fluid, with .10,500 mononuclear cells per mm3, which were atypical in morphology (B). Fluorescence-activated cell sorting demonstrated the expression of CD33, CD13, CD65, CD11b, CD4, and CD14. Cytogenetic analysis and fluorescence in situ hybridization showed tetraploidy of chromosome 8, with 4 copies of the ETO gene and additional mutations of AML1 (21q22), MYH11 (16q13), and CBFB (16q22). A musculoskeletal power Doppler ultrasound revealed active elbow synovitis and effusion (C). Chloroma was diagnosed. An intensive systemic chemotherapeutic regimen for acute myeloid leukemia was initiated (idarubicin and high-dose cytarabine), with the addition of intrathecal chemotherapy (methotrexate, cytarabine, and methylprednisolone) for central nervous system infiltration. Complete elimination of arthritis was obtained a few days later. Chloroma (also known as myeloid sarcoma or granulocytic sarcoma) is a less common form of extramedullary acute myeloid leukemia (2.5–9.1%) (1), which may have a variety of clinical presentations. Bone is a commonly reported site of infiltration, with osteocondensation usually seen. Intraarticular localization is less frequently described (1–3). 1. Bakst RL, Tallman MS, Douer D, Yahalom J. How I treat extramedullary acute myeloid leukemia. Blood 2011;118:3785–93. 2. Pileri SA, Ascani S, Cox MC, Campidelli C, Bacci F, Piccioli M, et al. Myeloid sarcoma: clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients. Leukemia 2007;21:340–50. 3. Paydas S, Zorludemir S, Ergin M. Granulocytic sarcoma: 32 cases and review of the literature. Leuk Lymphoma 2006;47:2527–41.
Aurelien Guffroy, MD Mehdi Helali, MD Blandine Merle, MD Alessio Imperiale, MD, PhD Emmanuel Chatelus, MD Strasbourg Hospital and University Strasbourg, France
6. Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 1990;33:1101–7. 7. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990;33:1094–100. 8. Sridhar S, Schembri F, Zeskind J, Shah V, Gustafson AM, Steiling K, et al. Smoking-induced gene expression changes in the bronchial airway are reflected in nasal and buccal epithelium. BMC Genomics 2008;9:259. 9. Spira A, Beane J, Pinto-Plata V, Kadar A, Liu G, Shah V, et al. Gene expression profiling of human lung tissue from smokers with severe emphysema. Am J Respir Cell Mol Biol 2004;31:601–10. 10. Aubry MC. Necrotizing granulomatous inflammation: what does it mean if your special stains are negative? Mod Pathol 2012;25 Suppl 1:S31–8. 11. Laudien M, Hasler R, Wohlers J, Bock J, Lipinski S, Bremer L, et al. Molecular signatures of a disturbed nasal barrier function in the primary tissue of Wegener’s granulomatosis. Mucosal Immunol 2011;4:564–73. 12. Schreiber A, Kettritz R. The neutrophil in antineutrophil cytoplasmic autoantibody-associated vasculitis. J Leukoc Biol 2013;94:623–31.
2239
13. Bleharski JR, Kiessler V, Buonsanti C, Sieling PA, Stenger S, Colonna M, et al. A role for triggering receptor expressed on myeloid cells-1 in host defense during the early-induced and adaptive phases of the immune response. J Immunol 2003;170: 3812–8. 14. Daikeler T, Regenass S, Tyndall A, Gencay MM, Roth M, Christ-Crain M, et al. Increased serum levels of soluble triggering receptor expressed on myeloid cells-1 in antineutrophil cytoplasmic antibody-associated vasculitis. Ann Rheum Dis 2008;67: 723–4. 15. Lyons PA, Rayner TF, Trivedi S, Holle JU, Watts RA, Jayne DR, et al. Genetically distinct subsets within ANCA-associated vasculitis. N Engl J Med 2012;367:214–23. 16. Kilty SJ, Bosse Y, Cormier C, Endam LM, Desrosiers MY. Polymorphisms in the SERPINA1 (a1-antitrypsin) gene are associated with severe chronic rhinosinusitis unresponsive to medical therapy. Am J Rhinol Allergy 2010;24:e4–9. 17. Rotta G, Matteoli G, Mazzini E, Nuciforo P, Colombo MP, Rescigno M. Contrasting roles of SPARC-related granuloma in bacterial containment and in the induction of anti-Salmonella typhimurium immunity. J Exp Med 2008;205:657–67. 18. Oh Y, Oh I, Morimoto J, Uede T, Morimoto A. Osteopontin has a crucial role in osteoclast-like multinucleated giant cell formation. J Cell Biochem 2014;115:585–95.
DOI: 10.1002/art.39172
Clinical Images: Chloroma—an uncommon cause of arthritis
The patient, a 51-year-old man, was referred to a rheumatologist for acute right elbow pain. He had a 1-month history of a testicular mass. Physical examination revealed synovitis of the elbow, with a swollen joint and total functional disability of the arm. He had no other arthritis or pain, no fever, and no systemic symptoms. Multiple foci of increased fluorodeoxyglucose (FDG) uptake throughout the skeleton, corresponding to areas of bone condensation in the shoulders, pelvis, and femurs, was seen on 18F-FDG–positron emission tomography integrated with computed tomography (PET/CT) (A). Intense 18F-FDG uptake was also detected in the right elbow joint. Articular involvement was particularly evident when CT volume rendering techniques and PET maximum intensity projections was used in combination (D). Arthrocentesis revealed orange-colored turbid fluid, with .10,500 mononuclear cells per mm3, which were atypical in morphology (B). Fluorescence-activated cell sorting demonstrated the expression of CD33, CD13, CD65, CD11b, CD4, and CD14. Cytogenetic analysis and fluorescence in situ hybridization showed tetraploidy of chromosome 8, with 4 copies of the ETO gene and additional mutations of AML1 (21q22), MYH11 (16q13), and CBFB (16q22). A musculoskeletal power Doppler ultrasound revealed active elbow synovitis and effusion (C). Chloroma was diagnosed. An intensive systemic chemotherapeutic regimen for acute myeloid leukemia was initiated (idarubicin and high-dose cytarabine), with the addition of intrathecal chemotherapy (methotrexate, cytarabine, and methylprednisolone) for central nervous system infiltration. Complete elimination of arthritis was obtained a few days later. Chloroma (also known as myeloid sarcoma or granulocytic sarcoma) is a less common form of extramedullary acute myeloid leukemia (2.5–9.1%) (1), which may have a variety of clinical presentations. Bone is a commonly reported site of infiltration, with osteocondensation usually seen. Intraarticular localization is less frequently described (1–3). 1. Bakst RL, Tallman MS, Douer D, Yahalom J. How I treat extramedullary acute myeloid leukemia. Blood 2011;118:3785–93. 2. Pileri SA, Ascani S, Cox MC, Campidelli C, Bacci F, Piccioli M, et al. Myeloid sarcoma: clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients. Leukemia 2007;21:340–50. 3. Paydas S, Zorludemir S, Ergin M. Granulocytic sarcoma: 32 cases and review of the literature. Leuk Lymphoma 2006;47:2527–41.
Aurelien Guffroy, MD Mehdi Helali, MD Blandine Merle, MD Alessio Imperiale, MD, PhD Emmanuel Chatelus, MD Strasbourg Hospital and University Strasbourg, France
