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КлМчний випадок I
Clinical Case
БШЬ.
СуГЛОБИ. JOINTS. I ХРЕБЕТ SPINE I
УДК616.31:616.316-008.6 DOI: https://doi.Org/10.22141/2224-1507.10.4.2020.220459
Ye.D. Yehudina1 ©, S.A. Trypilka2 ,S.Kh. Ter-Vartanian1
1Clinic of Modern Rheumatology, Kyiv, Ukraine
2Rheumatologist of the Communal Non-Commercial Enterprise of Kharkiv Regional Council «Regional Clinical Hospital», Kharkiv,
Ukraine
A clinical case of B-cell lymphoma in a patient with overlap, primary Sjogren's syndrome and scleredema adultorum of Buschke
For citation: Bol', sustavy, pozvonocnik. 2020;10(4):186-192. doi: 10.22141/2224-1507.10.4.2020.220459_
Abstract. This is a presentation of case report on B-cell lymphoma in a patient with overlapping primary Sjogren's syndrome (PSS) and scleredema adultorum of Buschke. Scleredema adultorum of Buschke (SB) is a rare disorder characterized by a diffuse indurative edema and skin tightening. A putative classification of this disease is based on the etiological factors and encompasses three types: Type 1 is associated with an infectious disease; Type 2 with paraproteinemia; Type 3 - with diabetes mellitus. There are very rare cases involving malignant neoplasms or autoimmune diseases. This clinical case describes a rare combination of SB with an autoimmune disease - PSS. It highlights the importance of studying other SB associations, in particular with autoimmune diseases, to ensure proper management and treatment of these patients. For a 51-year-old patient, the disease onset featured a dense facial edema, dry skin, dry eye syndrome and a feeling of sand in the eyes, subfebrile fever and dense skin formations in the low neckline, mammary glands and interscapular region, which was pathohistologically regarded as scleredema Buschke. Lymphadenopathy, xerophthalmia, sialodenitis, Raynaud's syndrome were revealed, lung damage (alveolitis and bronchiolitis), positive ANA, anti-SSa/La, antiSSb/Ro were diagnosed. Based on this symptom complex, the patient was diagnosed with an overlap syndrome of SB with PSS. The use of combined therapy with methotrexate and methylprednisolone allowed to reduce the disease activity, clinical and laboratory appearance of the disease. However, 9 months after the onset of the first skin symptoms, there was a bulging of the left eye, widespread lymphadenopathy. Histopathological examination revealed the diagnosis of non-Hodgkin B-cell lymphoma. The use of rituximab therapy resulted in a significant regression of the orbital tumor. The relevance of this clinical observation is not only due to the rarity of combined pathology, but also to the need for an early diagnostics of lymphoma developing against the background of PSS, based on the risk factors, which include low levels of C4 and/or C3, enlargement of the parotid salivary glands, cryoglobulinemia, persistent splenomegaly's systemic activity and lymphadenopathy, since the frequency of this disease is quite high in this patient population.
Keywords: scleredema adultorum of Buschke; Sjogren's syndrome; lymphoma; overlap syndrome; clinical case
Introduction
The overall progress achieved in the studies of systemic scleroderma and scleroderma-like conditions with various clinical forms being distinguished, along with differentiation and specification of nosologies in line with the new forms of induced scleroderma, resulted in a concept of scleroderma disease group. According to the recent beliefs, the term of "scleroderma" unites a wide circle of scleroderma diseases: systemic and focal scleroderma, diffuse eosinophilic fasci-
itis, induced scleroderma — chemical, iatrogenic, paraneoplastic and tumor-associated scleroderma and pseudoscle-roderma.
The group of scleroderma-like conditions involves a rare disease: scleredema adultorum of Buschke (edema-attended scleroderma, progressive benign subcutaneous induration, adult scleroderma) [1].
Scleredema adultorum of Buschke (SB) is a rare disease characterized by a diffuse indurated edema and induration
© 2020. The Authors. This is an open access article under the terms of the Creative Commons Attribution 4.0 International License, CC BY, which allows others to freely distribute the published article, with the obligatory reference to the authors of original works and original publication in this journal.
Для корреспонденции: Егудина Елизавета Давидовна, доктор медицинских наук, профессор образовательного центра, Клиника современной ревматологии, Днепровская набережная, 25, г. Киев, 49000, Украина; e-mail: elizavetaegudina@gmail.com; контактный тел. +38(099)0595475.
For correspondence: Ye.D. Yehudina, MD, PhD, professor, Head of the Educational Center, Clinic of Modern Rheumatology, Dniprovska еmbankment, 25, Kyiv, Ukraine; e-mail: elizavetaegudina@gmail. com; phone +38(099)0595475.
Full list of author information is available at the end of the article.
of the skin, commonly involving the face, neck, shoulder girdle and upper body, with a pathological process focused in the hypoderm. It may resolve itself spontaneously in the next 2 years or have a progressive or durable course with a potentially lethal outcome [2]. This disease is traditionally classified into three types depending on the etiological factor present: Type 1 is usually preceded by a contagious fever; Type 2 is associated with paraproteinemia; Type 3 — with diabetes mellitus [2]. There are very few cases associated with malignant neoplasm (biliary cancer or autoimmune disease) [3-5]. The SB-associated autoimmune diseases involve rheumatoid arthritis (RA), spondylarthritis and primary Sjogren's syndrome (PSS).
The primary Sjogren's syndrome (PSS) is a systemic autoimmune disease with a lot of clinical manifestations ranged from the "dry syndrome" to systemic extraglandu-lar afflictions [6]. While the "dry syndrome" is primarily affecting the life quality by reducing it and may result in topical lesions involving mucous membranes, the systemic affliction determines the overall prognosis of the disease outcome. The risk of PSS patients' developing lymphoma grows from 3 % in the first 5 years to 9.8 % after 15 years, i.e. up to 40 times over the population risk [7]. The present lymphadenopathy, constant enlargement of parotid gland, palpable purpura, low serum C4 rates and cryoglobulinemia predict an elevated risk of this complication's developing and may even increase the risk of lymphoma 5 times over [8]. The mean age of disease onset is about 50 years; the SS diagnosis usually precedes the lymphoma by 7 years on average [8]. The most common localizations are parotid gland and other saliva glands, followed by orbits, stomach, thyroid gland, lungs and upper respiratory pathways, rarely other foci [7].
Clinical case
There is a clinical case presented of the B-lymphoma developing in a patient afflicted with an overlap syndrome of SB and PSS.
The 51-year-old patient was admitted to the Clinic of Modern Rheumatology on 25.10.2019, complaining at a dense facial edema, dryness of skin and eyes, visual gritti-ness, temperature being raised to the subfebrile rates, dense cutaneous formations with hyperemia in the cleavage, mammary gland and interscapular area of up to 10 cm in diameter. The patient has been suffering for 3 months before the above-mentioned complaints started. The patient was examined: the total blood count was marked by thrombocy-topenia of up to 110*1012/l, leukopenia of up to 3.15*109/l, increased erythrocyte sedimentation rate (ESR) of up to 30 mm/hour, while the biochemical blood assay was marked by barely any pathology. The ultrasound examination of saliva and thyroid gland was performed on 07.10.2019: all the saliva glands had a pronounced spongy structure; being coarse-cellular, they belonged to a "sponge" type; all groups of saliva glands were afflicted by sialadenitis, neck lymph nodes manifested hyperplasia; the patient was diagnosed with multinodular goiter and cervical lymphadenopathy. The ultrasound examination of abdominal cavity organs (ACOs) of 09.10.2019 revealed splenomegaly, lymphade-
nopathy of abdominal cavity; inflammatory echostructure lymph nodes were located in the hepatic portae, the biggest one of 16-4 mm — under the left lobe.
On 23.10.2019, the X-ray of thoracic cage organs revealed an intrathoracic lymphadenopathy (bifurcating lymph nodes of 14 mm, other groups of up to 10 mm). On 25.10.2019, the computer tomography (CT) of thoracic cage organs (TCOs) and abdominal cavity organs (ACOs) demonstrated numerous bilateral minor foci with diffuse contours of up to 1 mm in the lungs (primarily S45 ); they were surrounded by the "mat glass" type changes. On the right side, on the subpleural level (St 34 ), there were singular nodular indurations of up to 3 mm, while along the interlobular pleura there were bilateral nodular indurations of up to 3.5 mm. There are singular air cavities found of up to 9 mm large. Singular paratracheal, para-aortal and bifurcating lymph nodes were up to 4-9 mm in size. Adipose tissue induration was observed in the axillary areas, with the lymph nodes being 7-13-15 mm large. In the upper lateral quadrant of the right and left mammary gland there are areas of consolidation with a more intensive contrast accumulation of up to 13-10 mm. The cutaneous indurations at the level of mammary glands are bilateral and symmetrical. At the sternum level, T1-3 on the right side of the spinous processes is characterized by the subcutaneous adipose induration of the anterior and posterior chest wall. The enlarged size of the spleen (104-154-140 mm), in a para-aortal/vena cava perspective there are numerous lymph nodes with diffuse contours of up to 8-12 mm. The CT signs point to bronchi-olitis/alveoliotis. Conclusions. The focal pulmonary pathology of a non-specific character. Splenomegaly. Adenopathy of lymph nodes is observed in the retroperitoneal space. Mammary glands have neoplasms. Multinodular goiter. On 18.10.2019, magnetic resonance imaging (MRI) examination of the brain revealed signs of the minor focal cerebral lesions which have a vascular genesis and are associated with a moderate expansion of subarachnoid spaces, being the initial signs of dyscirculatory (cerebral microangiopathy-attributed) encephalopathy. As of 19.10.2019, there was a consultation by a neurologist who diagnosed an unspecified cerebral microangiopathy, cervicocranialgia.
During the rheumatologist's consultation on 25.10.2019, an objective examination demonstrated: visual examination reveals a dense edema of facial skin, swelling of parotid saliva glands, dense hyperemic foci of up to 3 cm in diameter around mammary glands, cleavage and interscapular area, thyroid gland enlargement of Grade 1 (Fig. 1, 2). The sub-mandibular and cervical lymph glands are enlarged to 1.5 cm. While auscultating the lungs and heart, the rheuma-tologist did not reveal any pathology. The stomach palpation does not produce any pain, the liver is not enlarged, and the lower pole of the spleen and intestine are palpated and provide the regular palpatory signs.
Considering the CT-manifested TCO and ACO changes, lymphadenopathy, skin leasions, we've concluded that sarcoidosis attended by the skin and lung lesions should be ruled out, as well as the paraneoplastic pathology. The consultation by a pulmanologist, phthisiologist, and oncologist is recommended, along with the skin lesion biopsy.
On 11.11.2019, the pathohistological skin examination revealed that all the dermal tissue is in the state of edema, the collagen fibers levels closest to the epidermis present the considerable degenerative-dystrophic changes and a partial destruction of fibers. In the deeper dermal layers, there are coarse collagen fibers with degeneration, depleted skin processes. The perivascular infiltration occurs via lymphoid nodule-like cells, collagen and mucin are deposited in the derma. Similar infiltrates surround the hair structures. Conclusions: skin changes correspond to the scleredema adul-torum of Buschke (SB). There is a syndrome of mucous-cutaneous-lymphoid manifestations of systemic connective disease.
The pulmonologist found no evidence of sarcoidosis, tuberculosis. The hematologist-oncologist found no evidence of oncopathology. The conclusions were based on the clinical, laboratory and instrumental data.
There was a repeat rheumatologist's consultation on 20.11.2019. Additional examination was recommended: assay of antinuclear antibodies and anti-SSa/Ro and anti-SSb/La, examination of thyroid gland function, assay of anti-thyroid peroxidase autoantibodies (anti-TPO antibodies), ophthalmologist's consultation with Schirmer's test being taken, capillaroscopy.
The capillaroscopy of 24.11.2019 resulted in the following findings: while analyzing the capillary blood flow, there were signs of minor-diameter capillary loop spasm, the capillaries being predominantly of short and medium length account for 70 %; there are short, undercurled spirals, paragraphs registered, along with capillary loops of mostly minor and medium caliber — 30 %. There are multiple avascular fields in 1 and 2 echelons, on the 2-5th digits of both hands (sclerodermic pattern predominantly present on the left hand). Specific gravity weight of capillaries is insignificantly reduced on the 2nd finger of left hand and 5 fingers of both hands (4-5 capillary loops per 1 mm2), the rate of capillary blood flow is insignificantly reduced. The capillary curvature index is 30 %. Raynaud syndrome of 1-2
degree is diagnosed. Microcirculation is of a spastic type. There are signs of slowly-progressing systemic scleroder-ma-type disease.
On 21.11.2019, the antinuclear antibodies (ANA) amounted to 1:1200; the anti-SSa/Ro (50 IU/ml) and anti-SSb/La (46 IU/ml) were found at the high titre. The thyroid gland function is not compromised, Anti-TPO are at an elevated titre (150 IU/ml). The rheumatoid factor is 12 IU/ ml. The ophthalmologist's consultation brought in the following diagnoses: the "dry eye" syndrome, Schirmer's test: 1 mm per 5 minutes.
Considering the data being received at the repeat examination of 18.12.2019, the following diagnosis was made: overlap syndrome of scleredema adultorum of Buschke (SB) (dense facial edema, focal indurations at the back, cleavage, mammary glands) with primary Sjogren's syndrome (PSS) afflicting the saliva glands (sialadenitis), lungs (alveolitis), vessels (Raynaud syndrome), reticuloendothelial system (lymphadenopathy, splenomegaly), dryness syndrome (xe-rophthalmia), thyroid gland (autoimmune thyroiditis, eu-thyroidism), immunological (ANA, anti-SSa/La, anti-SSb/ Ro) and hematological (thrombocytopenia, leukopenia) syndromes.
It is recommended to take methylprednisolone at a daily dose of 12 mg, methotrexate at a weekly dose of 10 mg with a predicted elevation after 2 weeks to a weekly dose of 15 mg, folic acid at a dose of 5 mg 24 hours after the methotrexate being taken once a week, Calcium (1200 mg) and Vitamin D (800 mg) supplements once every 24 hours, to apply topical mometasone ointment on the cutaneous foci, to use "artificial tear" drops locally.
The choice of methylprednisolone and methotrexate therapy was based on the performed clinical studies and a series of cases in the PubMED database which were traced according to the keywords «Sjogren's syndrome and scleredema adultorum of Buschke» and the EULAR 2019 guidelines on SS management [9]. The Table presents the dynamics of patient's laboratory values.
Table. Dynamics of the patient's laboratory values at the pre-examination and treatment stage
" —■—Date Values '———______ 21.10.2019 17.12.2019 09.01.2020 11.02.2020 17.03.2020 19.05.2020
Leukocytes, 109/l 3.15 4.89 8.02 6.14 5.27 5.5
Neutrophils, % 65 63 76,2 50 30 32
Lymphocytes, % 20 18 16,5 28 58 56
Eosinophils, % 1.3 1.5 1.5 3 2 2
Erythrocytes, 1012 /l 4.0 4.45 4.33 4.61 4.47 4.5
Hemoglobin, g/l 111 127 127 132 137 136
Platelets, 1012 /l 110 131 133 133 137 127
ESR, mm/hour 30 20 16 8 2 8
Alanine Aminotransferase, IU 14 21 16 12 18 18
Aspartate aminotransferase, IU 19 19 15 12 14 14
Creatinine, mcmol/l 79 70 65 65 69 70
Glucose, mcmol/l 4.2 4.3 4.2 4.5 4.4 4.8
Total protein, g/l 66 68 67 70 72 73
Notes: ESR - erythrocyte sedimentation rate, IU - international units.
The background therapy of methotrexate, methylpred-nisolone during the first 2 months resulted in a reduced facial edema, saliva gland edema, cutaneous neoplasm regression (Fig. 3), and ESR and leukocyte rate normalization. Considering a significant improvement of clinical picture, absence of complaints, the healthcare providers initiated a methylprednisolone reduction of 2 mg every 2 weeks starting from February 2020, and of 2 mg a month from April until
Fig. 1. Dense facial edema. Parotid gland edema (November 2019)
a complete cessation in May, 2020. The methotrexate treatment of 15 mg a week continues.
The add-up doses of methotrexate and methylprednisolone received by the patient during 7 months before the lymphoma's onset in July 2020 were 420 mg and 1900 mg, respectively.
While observing the patient, the healthcare providers registered a tendency towards neutropenia and lympho-cytosis in the total blood count since March, 2020. Since June, 2020, the patient observed an out swelling of left eye (Fig. 4). There were no general symptoms (fever, general weakness, increased sweating) observed. The skin rash has no dynamics. On 17.07.2020, there was CT performed on the orbits, thoracic cage and abdominal cavity organs: in the left orbit between the lateral and upper rectus muscles there was an extra soft-tissue mass of 6-16-14 mm detected adjacent to the upper lateral area of eyeball; being inseparable from the upper saliva gland. According to the similarly accumulated contrast, the above-mentioned muscles manifest a reduced contour differentiation. There are multiple lymph nodes detected on both sides of the neck, the biggest one in the submental group reaching 4 mm, in the submaxillary group — up to 7 mm, upper jugular group - up to 7 mm, lower jugular group - up to 7 mm, dorsal group — up to 5 mm, supraclavicular group - up to 5 mm. There are peri-vascular, aortopulmonary, bronchopulmonary, bifurcating lymph nodes visualized in the mediastinum of up to 9 mm large. On the pulmonary side, there is a positive dynamic: no infiltrating changes. There is a positive dynamic on the bilateral axillary side: the adipose tissue swelling and size is reduced, thickness of anterior chest wall is reduced as well. In the upper lateral quadrant of the right mammary gland there is a persistent nodular induration of 6-10 mm, characterized by a positive dynamic due to its reduced size and density. The nodular changes of thyroid gland persist. The hepatosplenomegaly is observed (right hepatic lobe is 201 mm long; spleen size is 49-130-141 mm). There are mul-
Fig. 2. Dense hyperemic neoplasm, subcutaneous neoplasm of the cleavage and left mammary gland
(November 2019)
tiple lymph nodes in the hepatoduodenal junction of up to 10 mm large, in the retroperitoneal area of up to 7 mm large, in the pelvis minor area (general and exterior iliac) of up to 8 mm large.
There was a pathohistological study made of the orbital tumor. The histological puncture of left orbital neoplasm (30.07.2020) revealed a mass of polymorph cells with disparate hyperchrome nuclei which are lymphoma-pathog-nomonic. The bone marrow puncture was performed and immunohistochemical assay of the orbital tumor cells. The tumor cells revealed a positive membrane reaction to CD20, CD23, bd2. Part of the tumor cells revealed a positive membrane reaction to CD11c. The tumor cells are negative to CD123, DBA44, annexin F1, CD10, CD30, SOX-11, cy-clin D1, CD3, CD5. Considering the results of histological and immunohistochemical studies, there are bone marrow cells damaged by the B-cell lymphoma whose phenotype is characterized by the nodal lymphoma from the marginal zone cells. The patient had a hematologist-oncologist consultation resulting in a non-Hodgkin's B-cell lymphoma of the nodal marginal zone lymphoma (NMZL), IVa stage with bone marrow damage. According to the hematologist-oncologist's recommendations, the methotrexate therapy suspended, Rituximab therapy of 1 g twice every 2 weeks was initiated. On 1.08.2020 and 25.08.2020, 2 injections were performed, followed by a significant regression of orbital tumor. The patient is observed by hematologist-oncologist and rheumatologist.
The PSS patients may develop numerous systemic signs, both as clinical manifestations functioning as the initial disease signs and those associated with the progressing disease [10]. Among the systemic manifestations resulting in an early and elevated mortality of PSS patients, there are cryo-globulinemic vasculitis, interstitial pulmonary disease and
lymphoma [11]. It is worthy of noting that the PSS patients have a higher lymphoma risk than patients with other autoimmune diseases: 7 and 4 times as often as the systemic lupus erythematosus (SLE) and RA, respectively, and 10 times as often as the healthy subjects [12]. The recent studies dealing with the prognostic marker role in the PSS-associated lymphoma offer a synergetic risk model as the more prognostic factors there are, the higher risk of developing lym-
Fig. 4. Facial and parotid gland edema reduction, though attended by the outswelling of left eye (July2020)
Fig. 3. Subcutaneous neoplasm regression in the mammary gland and interscapular area. Reduction of size, density and hyperemia (April 2020)
phoma. S. Fragkioudaki et al. (2016) [13] offer a predictive non-Hodgkin's lymphoma (NHL) risk parameter involving 7 prognostic markers: a low C4 and/or C3 rate, enlargement of parotid glands, cryoglobulinemia, splenomegaly, systemic activity and lymphadenopathy. It was concluded that patients having no less than 3 factors are likely to develop NHL in 40 % cases while patients having all 7 prognostic factors are likely to develop NHL in 100 % cases. W. Zhang et al. (2010) [14] report that the risk of malignant neoplasm would increase about 30 times in patients with 3 or more risk factors, and 3 times in patients with 1 risk factor compared with patients with no risk factors. Our patient had 4 suggested risk factors: enlarged parotid glands, splenomegaly, systemic activity and lymphadenopathy.
Conclusions
To sum up, the above-mentioned clinical case featured a rare combination of SB and PSS, an autoimmune disease. The reference sources describe only one clinical observation of SB associated with secondary SS (RA-attended) [4] and one associated with primary SS [5]. The importance of other SB associations, namely with autoimmune diseases, is relevant for the adequate management and treatment. Furthermore, the risk of lymphoma developing on the PSS background is rather high, implying an elevated onco-awareness of the practitioners in terms of likely development and target ruling-out of lymphoma in the PSS patients along with risk factor evaluation.
Conflicts of interests. Authors declare the absence of any conflicts of interests and their own financial interest that might be construed to influence the results or interpretation of their manuscript.
Sources of finance: no.
Individual contributions: Yehudina Ye.D., Trypilka S.A. — concept and design of the study; Yehudina Ye.D.- data collection; Yehudina Ye.D., Trypilka S.A. — analysis and data interpretation; Yehudina Ye.D.- article writing; Ter-Vartanian S.Kh. — article editing.
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Received 05.10.2020 Revised 15.10.2020 Accepted 02.11.2020 ■
Information about authors
Ye.D. Yehudina, MD, PhD, professor, Head of the Educational Center, Clinic of Modern Rheumatology, Kyiv, Ukraine; https://orcid.org/0000-0001-8702-5638
S.A. Trypilka, MD, PhD in medicine, Associate of Professor, Rheumatologist of Communal Non-Commercial Enterprise of Kharkiv Regional Council "Regional Clinical Hospital", Kharkiv, Ukraine; https://orcid.org/0000-0001-6630-9893
S. Ter-Vartanian, PhD in medicine, Medical Director, Clinic of Modern Rheumatology, Kyiv, Ukraine
£гуд'ша €.Д.\ Триплка С.А.2, Тер-Вартаньян С.Х.' 'Кл'шЫа сучасно!ревматологи, м. Ки!в, УкраУна
2Комунальненекомерц'шнетдприемствоХарк'шсько!обласно!ради«Обласнакл'ш'чнал'жарня»,м. Харк'ш, УкраУна
КлМчний випадок розвитку В-клггинноТ лiмфоми в пацкнтки з оверлап-синдромом первинного синдрому Шегрена i склередеми Бушке
Резюме. Наведений опис клшчного випадку розвитку В-клггинно! лiмфоми в пацieнтки з оверлап-синдромом первинного синдрому Шегрена (ПСШ) i склередеми Бушке (СБ). Склередема Бушке — рщюсне захворювання, що харак-теризуеться дифузним шдуративним набряком i ущшьнен-ням шири. Прийнята умовна класифшацш цього захворювання залежно вщ етiологiчного чинника: тип 1 — асоцшо-ваний з шфекцшним захворюванням; тип 2 — з парапроте1-немiею i тип 3 — з цукровим дiабетом. 1снують дуже рiдкiснi випадки, пов'язанi iз злояк1сними новоутвореннями або ав-тоiмунними захворюваннями. Даний клшчний випадок опи-суе рщюсне поеднання СБ з автоiмунним захворюванням — ПСШ, що пщкреслюе важливiсть вивчення шших асоцiацiй СБ, зокрема з автоiмунними захворюваннями, для забезпе-чення належного ведення й лiкування таких пащенйв. Захворювання в пащентки 51 року дебютувало з щшьного набряку обличчя, сухостi шкiри, синдрому «сухого ока» й вщчуття тс-ку в очах, пщвищення температури до субфебрильних цифр i щшьних утворень шкiри в дiлянцi декольте, молочних залоз i мiжлопатковiй дшянщ, що патогiстологiчно було розцiнено як СБ. Виявлено лiмфаденопатiю, ксерофтальмт, шалоаде-
шт, синдром Рейно, giarHOCTOBaHO ураження легень (альве-олiт i бронхюли), позитивнi ANA, anti-SSa/La, anti-SSb/Ro. На nigcTaBi даного симптомокомплексу пацieнтцi був вистав-лений дiагноз «оверлап СБ i3 ПСШ». Застосування комбшо-вано! терапи метотрексатом i метилпреднiзолоном дозволило знизити актившсть захворювання, кшшчт й лабораторнi прояви хвороби. Однак через 9 мюяцш пiсля появи перших шюрних симптомiв виникло вибухання лiвого ока, пошире-на лiмфаденопатш. При патогiстологiчному обстеженнi вста-новлено дiагноз неходжинсько! В-клиинно! лiмфоми. Застосування терапи ритуксимабом призвело до значного регре-су пухлини орбити. Актуальшсть даного клiнiчного спостере-ження — не тшьки в рщюсносй поеднано! патологи, а й у не-обхiIдностi проведення ранньо! дiагностики розвитку лмфо-ми на тл ПСШ на пiIдставi факторiв ризику, до яких вщносять низький рiвень С4 i/або С3, збшьшення привушних слинних залоз, крiоглобулiнемiю, спленомегалш, персистуючу сис-темну активнiсть i лшфаденопатш, осюльки частота даного захворювання досить висока в цш популяци пащенпв. Ключовi слова: склередема Бушке; синдром Шегрена; лiм-фома; оверлап-синдром; клшчний випадок
Егудина Е.Д.', Триполка С.А.2, Тер-Вартаньян С.Х.' 'Клиника современной ревматологии, г. Киев, Украина
2Коммунальное некоммерческое предприятие Харьковского областного совета «Областная клиническая больница», г. Харьков, Украина
Клинический случай развития В-клеточной лимфомы у пациентки с оверлап-синдромом первичного синдрома Шегрена и склередемы Бушке
Резюме. Представлено описание клинического случая развития В-клеточной лимфомы у пациентки с оверлап-син-дромом первичного синдрома Шегрена (ПСШ) и склереде-мы Бушке (СБ). Склередема Бушке — редкое заболевание, характеризующееся диффузным индуративным отеком и уплотнением кожи. Принята условная классификация этого заболевания в зависимости от этиологического фактора: тип 1 — ассоциированный с инфекционным заболеванием; тип 2 — с парапротеинемией и тип 3 — с сахарным диабетом. Существуют очень редкие случаи, связанные со злокачественными новообразованиями или аутоиммунными заболеваниями. Данный клинический случай описывает редкое сочетание СБ с аутоиммунным заболеванием — ПСШ, что подчеркивает важность изучения других ассоциаций СБ, в частности с аутоиммунными заболеваниями, для обеспечения надлежащего ведения и лечения таких пациентов. Заболевание у пациентки 51 года дебютировало с плотного отека лица, сухости кожи, синдрома «сухого глаза» и ощущения песка в глазах, повышения температуры до субфебриль-ных цифр и плотных образований кожи в области декольте, молочных желез и межлопаточной области, что патогисто-логически было расценено как СБ. Выявлены лимфаденопа-тия, ксерофтальмия, сиалоаденит, синдром Рейно, диагно-
стировано поражение легких (альвеолит и бронхиолит), позитивные ANA, anti-SSa/La, anti-SSb/Ro. На основании данного симптомокомплекса пациентке был установлен диагноз «оверлап СБ с ПСШ». Применение комбинированной терапии метотрексатом и метилпреднизолоном позволило снизить активность заболевания, клинические и лабораторные проявления болезни. Однако через 9 месяцев после появления первых кожных симптомов возникло выбухание левого глаза, распространенная лимфаденопатия. При патогисто-логическом обследовании установлен диагноз неходжкин-ской В-клеточной лимфомы. Применение терапии ритукси-мабом привело к значительному регрессу опухоли орбиты. Актуальность данного клинического наблюдения — не только в редкости сочетанной патологии, но и в необходимости проведения ранней диагностики развития лимфомы на фоне ПСШ, на основании факторов риска, к которым относят низкий уровень С4 и/или С3, увеличение околоушных слюнных желез, криоглобулинемию, спленомегалию, персисти-рующую системную активность и лимфаденопатию, так как частота данного заболевания достаточно высока в этой популяции пациентов.
Ключевые слова: склередема Бушке; синдром Шегрена; лимфома; оверлап-синдром; клинический случай
