Differential diagnosis of rheumatic diseases and blood cancers involving the nasal cavity and accessory sinuses

To provide the clinical, laboratory, radiological, morphological, and immunomorphological signs that permit the differential diagnosis to be made in patients with involvement of the nasal cavity and accessory sinuses (NCAS). In the period 2009 to 2013, the Laboratory for Intensive Therapy for Rheuma...

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Published in:	Terapevtic̆eskii arhiv Vol. 86; no. 5; pp. 62 - 72
Main Authors:	Vasil'ev, V I, Sokol, E V, Sedyshev, S Kh, Gorodetskiĭ, V R, Aleksandrova, E N, Logvinenko, O A, Pal'shina, S G, Rodionova, E B, Radenska-Lopovok, S G, Probatova, N A, Kokosadze, N V, Pavlovskaia, A I, Kovrigina, A M, Varlamova, E Iu, Safonova, T N, Borovskaia, A B, Gaĭduk, I V, Mukhortova, O V, Aslanidi, I P, Nasonov, E L
Format:	Journal Article
Language:	Russian
Published:	Russia (Federation) "Consilium Medicum" Publishing house 01-01-2014
Subjects:	Adult Diagnosis, Differential diseases DNA, Viral - blood epstein-barr virus Female hematological oncology Herpesvirus 4, Human - isolation & purification Humans igg4-related diseases Lymphoma, Extranodal NK-T-Cell - complications Lymphoma, Extranodal NK-T-Cell - diagnosis Lymphoma, Extranodal NK-T-Cell - immunology Lymphoma, Extranodal NK-T-Cell - physiopathology Male Middle Aged Monitoring, Immunologic - methods nasal accessory sinuses nasal cavity Nasal Cavity - pathology natural killer cell lymphoma Paranasal Sinus Diseases - diagnosis Paranasal Sinus Diseases - etiology Paranasal Sinus Diseases - immunology Paranasal Sinus Diseases - physiopathology Paranasal Sinuses - pathology Radiography - methods Rheumatic Diseases - classification Rheumatic Diseases - complications Rheumatic Diseases - diagnosis Rheumatic Diseases - immunology Rheumatic Diseases - physiopathology Symptom Assessment - methods
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Summary:	To provide the clinical, laboratory, radiological, morphological, and immunomorphological signs that permit the differential diagnosis to be made in patients with involvement of the nasal cavity and accessory sinuses (NCAS). In the period 2009 to 2013, the Laboratory for Intensive Therapy for Rheumatic Diseases, V.A. Nasonova Research Institute of Rheumatology, Russian Academy of Medical Sciences, associated the disease onset with NCAS involvement in 39 (7.6%) of 512 examinees. NCAS involvement was present at disease onset in 100% of the patients with natural killer (NK) cell lymphoma (NK/T lymphoma), in 84.5% of those with Wegener granulomatosis (WG), in 29.5% of those with IgG4-related disease (IgG4-RD), and in 17.5% of those with sarcoidosis. Such an onset could be extremely rarely observed in histiocytosis. Despite the similar clinical manifestations, NCAS involvements in NK/T lymphoma of nasal type and WG at disease onset show clear differences in the laboratory and systemic manifestations of these diseases. The patients with lymphoma have no characteristic laboratory abnormalities at disease onset, except the 100% presence of Epstein-Barr virus (EBV) DNA in blood and, only as a tumor grows, fever appears and there are elevated C-reactive protein and lactate dehydrogenase levels and pronounced destructive changes in the facial bones with mandatory hard palate destruction; at the same time the signs of systemic involvement are virtually absent. The patients with WG at disease onset have fever, high erythrocyte sedimentation rate, elevated C-reactive level, significant anemia, leukocytosis and 90% are found to have anti-neutrophil cytoplasmic antibodies with the rapid development of systemic manifestations: involvements of the lung, kidney, and peripheral nervous system. Destructive changes in the facial bones are minimal and hard palate destructions are absent. The patients with IgG4-RD, sarcoidosis, and juvenile xanthogranuloma have similar clinical and laboratory manifestations in the absence of hemorrhagic nasal discharge, nasal septal perforation, and facial bone destruction, with the practically involvement of the salivary/lacrimal glands and orbital regions. A third of the patients are observed to have different allergic manifestations, moderate eosinophilia, and signs of autoimmune disorders (the presence of rheumatoid and antinuclear factors, hypergammaglobulinemia). Elevated serum IgG4 levels are characteristic of IgG4-RD. Blood anti-neutrophil cytoplasmic antibodies, EBV DNA, and IgG4 levels should be determined in all patients with NCAS involvement. Mini-invasive incision biopsies of the nasal mucosa, orbital regions, and major salivary glands should be done, by morphologically verifying the diagnosis of sarcoidosis, histiocytosis, and WG and by making an immunomorphological examination to diagnose NK/T lymphoma and IgG4-RD.
ISSN:	0040-3660 2309-5342