IgG4-RELATED DISEASE. CLINICAL NOTES

IgG4-related diseases are a new nosological entity that encompasses a few previously known diseases. IgG4-related systemic disease is diagnosed if two or more affected organs are detected. This group of diseases has two similar signs: serological (elevated serum IgG4 subclass concentrations) and histological (organ and tissue infiltration from plasmo-cytes secreting IgG4, and eosinophils, and the development of fibrosclerosis and phlebitis obliterans). The paper describes two cases. In one case, a multisystemic disease was observed virtually at its onset whereas in the other this lesion was diagnosed several years after the natural course of the disease

Дифференциальная диагностика плазматических дискразий в ревматологической практике

Plasma cell dyscrasias (PCD) present certain difficulties in differentially diagnosing with autoimmune rheumatic diseases (RD) as they have some clinical and laboratory manifestations that are characteristic of the latter. Objective: to generalize the experience in diagnosing PCD at the Research Institute of Rheumatology (RIR), Russian Academy of Medical Sciences (RAMS). Subjects and methods. Nineteen patients admitted to the RIR, RAMS, for rheumatological referral diagnoses were diagnosed as having different types of PCD, both PCD in the presence of RD (n = 10) and primary PCD without RD (n = 9). Immunochemical studies of serum and urinary proteins and bone marrow trepanobiopsy were performed in all the patients. Histological studies were made of the minor salivary glands (n = 12), lymph nodes (n = 3), parotid salivary glands - PSG (n = 5), spleen (n = 1), cranial bones (n = 2) and humerus (n = 1). The immunophenotype of tumor lymphocytes in the biopsy and trepanobiopsy specimens were determined by an immunoflurenscence method with standard monoclonal antibody panels and the paraffin sections of biopsy specimens were examined by an immunoperoxidase technique. Biopsy and trepanobiopsy speciments and myelograms were assessed by the researchers of the N.N. Blokhin Russian Cancer Research Center, RAMS. Results. Over 10 years of follow-up, 19 patients (13 females and 6 males) were diagnosed as having different types of PCD: multiple myeloma (MM) (n = 9), extramedullary plasmacytoma of lymph nodes (n = 2), solitary plasmacytoma (SP) of bone (n = 2), Bence-Jones myeloma (BJM) (n = 2), primary amyloidosis (PA) (n = '), lymphoplasmacytic lymphoma (n = '), Waldenström's macroglobulinemia (WM) (n = 2). In the presence of RA, '0 patients with PCD developed Sjogren's disease (SD) (n = 7), rheumatoid arthritis (RA) + SD (n = 2), RA (n = '); and 9 patients had primary PCD (MM (n = 5), BJM (n = '), WM (n = '), SP (n = '), and PA (n = ')). These 9 patients with different rheumatological diagnoses were long followed up and referred to the RIR, RAMS, to specify these. One third of the patients had recurrent conjunctivitis, enlarged PSG and xerostomia (55%). Arthralgias, arthritis of minor and large joints, flexion contractures of hands, ossalgias, and pains in the lumbar spine and ribs were present in '0-45% of the patients. Vascular lesions, such as Reynaud's phenomenon, recurrent purpuras, lower extremity ulcerative lesions, and toe gangrenes, were observed in '0-35% of the patients with PCD. Lesions in the reticuloendothelial system, such as lymphadenopathy, splenomegaly ('5%), hepatomegaly (45%), and fever (25%), were also detected. Conclusion. Many clinical and laboratory manifestations of primary PCD and RD are similar and only the absence of classical laboratory markers of autoimmune disease, as well as the presence of serum monoclonal immunoglobulins and urinary Bence Jones protein suggest the presence of PCD, both primary PCDs and those with RD.Плазматические дискразии (ПД) представляют определенные трудности для дифференциальной диагностики с аутоиммунными ревматическими заболеваниями (РЗ), так как имеют некоторые клинические и лабораторные проявления, присущие последним. Цель исследования - обобщить опыт диагностики ПД в НИИ ревматологии РАМН. Материал и методы. У 19 пациентов, поступивших в НИИ ревматологии РАМН с направительными диагнозами РЗ, диагностированы различные варианты ПД, как на фоне имеющегося РЗ (у 10), так и первичные ПД без признаков РЗ (у 9). Всем больным проводили иммунохимическое исследование белков сыворотки крови и мочи, трепанобиопсию костного мозга. Выполняли гистологическое исследование малых слюнных желез (у 12), лимфатических узлов (у 3), околоушных слюнных желез - ОСЖ (у 5), селезенки (у 1), костей черепа (у 2) и плечевой кости (у 1). Иммунофенотип опухолевых лимфоцитов в биоптатах и трепанобиоптатах определяли им-мунофлюоресцентным методом со стандартными панелями моноклональных антител, а парафиновые срезы биоптатов изучали им-мунопероксидазным методом. Биоптаты, трепанобиоптаты и миелограммы оценивали сотрудники РОНЦ им. Н.Н. Блохина РАМН. Результаты исследования. За '0лет наблюдения у 19 больных (13 женщин и 6 мужчин) диагностированы различные варианты ПД: множественная миелома (ММ) - у 9, внекостная плазмоцитома лимфатических узлов (ВП) - у 2, солитарная плазмоцитома костей (СП) - у 2, миелома Бенс-Джонса (МЫ) - у 2, первичный амилоидоз (ПА) - у 1, лимфоплазмоцитарная лимфома (ЛПЛ) - у 1, макроглобулинемия Вальденстрема (МВ) - у 2. У 10 больных ПД развились на фоне РЗ: болезни Шёгрена - у 7, ревматоидного артрита (РА) + синдрома Шёгрена - у 2, РА у - 1, и у 9 больных ПД были первичными (ММ - у 5, МЫ - у 1, МВ - у 1, СП - у ', ПА - у '). Эти 9 больных длительно наблюдались с различными ревматологическими диагнозами и были направлены в НИИ ревматологии РАМН для уточнения таковых. У трети больных присутствовали рецидивирующие конъюнктивиты, увеличение ОСЖ и ксеростомия (у 55%). Артралгии, артрит мелких и крупных суставов, сгибательные контрактуры кистей, оссалгии, боли в поясничном отделе позвоночника и в ребрах имелись у '0-45% больных. Сосудистые поражения: феномен Рейно, рецидивирующие пурпуры, язвенные поражения нижних конечностей и гангрена пальцев стоп наблюдались у '0-35% больных ПД. Выявлены также поражение ретикулоэндотелиальной системы: лимфаденопатия, спленомегалия ('5%), гепатомегалия (45%) и лихорадка (25%). Заключение. Многие клинико-лабораторные проявления первичных ПД и РЗ являются сходными и только отсутствие классических лабораторных маркеров аутоиммунного заболевания, а также наличие моноклональных Ig в сыворотке крови и белка BJ в моче позволяет предположить наличие ПД, как первичных, так и на фоне РЗ

SUKhOI SINDROM KAK PROYaVLENIE AL-AMILOIDOZA

Amiloidoz – gruppa zabolevanii, kharakterizuyushchikhsya otlozheniem v organakh i tkanyakh spetsificheskikh interstitsial'nykh nerastvorimykh fibrill, privodyashchikh k tyazhelym funktsional'nym narusheniyam i smerti

CLINICAL MANIFESTATIONS OF VASCULITIS AT THE ONSET OF MULTIPLE MYELOMA

The paper describes two patients (a 50-year woman and a 72-year man) in whom the clinical manifestations of ulceronecrotic vasculitis had long preceded before the diagnosis of multiple myeloma was made. In the former, monoclonal cryoglobulinemia type I induced ulcerative lesions with the development of dry toe gangrene and paraproteinemic renal lesion. In the latter, ulceronecrotic vasculitis with the development of dry toe gangrene was a manifestation of monoclonal paraproteinemia without signs of cryoglobulinemia. Both patients were found to have monoclonal blood secretion (РIgGλ и РIgGκ) and urine Bence Jones protein (BJλ+BJκ, BJκ) in the absence of immunological markers of vasculitis developing in patients with rheumatic diseases. Immunochemical study of serum/urine and, when monoclonal secretion of PIg+BJ is detected, further examination for plasma cell dyscrasia should be performed in all cases of vascular disorders (cold allergy, Raynaud's syndrome, purpura, ulcers of cruses, and gangrene of distal phalanxes of the hands/feet) in patients with atypical vasculitis. The timely immunochemical study of blood and urine will make it possible to diagnose plasma cell dyscrasia (different types of myelomic disease, Waldenstrцm macroglobulinemia, primary amyloidosis) at the early stage of the disease and to rule out unjustifiably diagnosed vasculitis in patients with atypical vascular lesions

Differential diagnosis of plasma cell dyscrasias in rheumatological practice

Plasma cell dyscrasias (PCD) present certain difficulties in differentially diagnosing with autoimmune rheumatic diseases (RD) as they have some clinical and laboratory manifestations that are characteristic of the latter. Objective: to generalize the experience in diagnosing PCD at the Research Institute of Rheumatology (RIR), Russian Academy of Medical Sciences (RAMS). Subjects and methods. Nineteen patients admitted to the RIR, RAMS, for rheumatological referral diagnoses were diagnosed as having different types of PCD, both PCD in the presence of RD (n = 10) and primary PCD without RD (n = 9). Immunochemical studies of serum and urinary proteins and bone marrow trepanobiopsy were performed in all the patients. Histological studies were made of the minor salivary glands (n = 12), lymph nodes (n = 3), parotid salivary glands - PSG (n = 5), spleen (n = 1), cranial bones (n = 2) and humerus (n = 1). The immunophenotype of tumor lymphocytes in the biopsy and trepanobiopsy specimens were determined by an immunoflurenscence method with standard monoclonal antibody panels and the paraffin sections of biopsy specimens were examined by an immunoperoxidase technique. Biopsy and trepanobiopsy speciments and myelograms were assessed by the researchers of the N.N. Blokhin Russian Cancer Research Center, RAMS. Results. Over 10 years of follow-up, 19 patients (13 females and 6 males) were diagnosed as having different types of PCD: multiple myeloma (MM) (n = 9), extramedullary plasmacytoma of lymph nodes (n = 2), solitary plasmacytoma (SP) of bone (n = 2), Bence-Jones myeloma (BJM) (n = 2), primary amyloidosis (PA) (n = '), lymphoplasmacytic lymphoma (n = '), Waldenström's macroglobulinemia (WM) (n = 2). In the presence of RA, '0 patients with PCD developed Sjogren's disease (SD) (n = 7), rheumatoid arthritis (RA) + SD (n = 2), RA (n = '); and 9 patients had primary PCD (MM (n = 5), BJM (n = '), WM (n = '), SP (n = '), and PA (n = ')). These 9 patients with different rheumatological diagnoses were long followed up and referred to the RIR, RAMS, to specify these. One third of the patients had recurrent conjunctivitis, enlarged PSG and xerostomia (55%). Arthralgias, arthritis of minor and large joints, flexion contractures of hands, ossalgias, and pains in the lumbar spine and ribs were present in '0-45% of the patients. Vascular lesions, such as Reynaud's phenomenon, recurrent purpuras, lower extremity ulcerative lesions, and toe gangrenes, were observed in '0-35% of the patients with PCD. Lesions in the reticuloendothelial system, such as lymphadenopathy, splenomegaly ('5%), hepatomegaly (45%), and fever (25%), were also detected. Conclusion. Many clinical and laboratory manifestations of primary PCD and RD are similar and only the absence of classical laboratory markers of autoimmune disease, as well as the presence of serum monoclonal immunoglobulins and urinary Bence Jones protein suggest the presence of PCD, both primary PCDs and those with RD

Rheumatic masks of plasma cell dyscrasias

Objective: to consider clinical practice problems in the differential diagnosis of different types of plasma cell dyscrasias (PCD). Subjects and methods. Fourteen patients (8 men and 6 women) aged 52±12 years, in whom rheumatic diseases (RD) were ruled out and who were diagnosed as having primary PCD: different types of myeloma in 7 patients, myeloma + AL-amyloidosis in 2, AL-amyloidosis in 3, and Waldenstrom’s macroglobulinemia in 2, were examined. Results and discussion. The most common maldiagnosed RDs in patients with PCD were seronegative rheumatoid arthritis (RA), systemic lupus erythematosus, Sjogren’s disease, and different forms of vasculitis. The most frequent masks of RD were kidney (78%) and osteoarticular system (64%) lesions, vascular disorders (36%), peripheral polyneuropathies (36%), and enlarged salivary glands with xerostomia (28.5%). Serum and urine immunochemical study should be performed in all patients who have clinical manifestations of seropositive RA, spondyloarthritis, intensive bone pain syndrome, ulceronecrotic vasculitis, enlarged submandibular salivary glands with macroglossia in the absence of markers of autoimmune disease for the timely diagnosis of PCD and the exclusion of RD. The paper estimates the sensitivity and specificity of main methods used to diagnose different types of PCD