Extranodal marginal zone B cell lymphoma: An unexpected complication in children with Sjögren's syndrome

Collado, Paz; Kelada, Aml; Cámara, María Luisa de la; Zeft, Andrew; Flagg, Aron

doi:10.1016/j.reuma.2017.01.015

Cited by 18 publications

(3 citation statements)

References 9 publications

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“…Additionally, recurrent parotid swelling is the most common hallmark of SS, usually proceeding to sicca symptoms in childhood as in our patient, but the incidence is significantly lower than that of adults, which is associated with the age of onset, relatively short course of disease, and the minor damage of exocrine glands such as the salivary gland and lacrimal gland in juveniles ( 13 ). Diagnostic criteria for jSS are similar to those proposed in adults, but it manifested a lower sensitivity, and gland biopsy findings of the minor SG criteria (>1 focus of 50 lymphocytes/4 mm 2 ) are usually negative in children ( 14 ). The autoantibodies associated with jSS, anti-SSA and anti-SSB, are often detectable before overt glandular dysfunction is detected, both positive in the current patient.…”

Section: Discussionmentioning

confidence: 92%

The First Case Report of Preschool-Onset SS/SLE Coexisting With NMOSD of Chinese Origin

et al. 2022

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Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease (CTD), the main features of which are multiple serum autoantibodies and extensive involvement of multiple systems. The onset age of patients varies from childhood to middle age, with nearly 1/5 in childhood. Sjogren’s syndrome (SS) is also an autoimmune disease characterized by high-degree lymphocytic infiltration of exocrine glands, usually occurring in middle-aged and older women, and rarely in childhood. Neuromyelitis optica spectrum disorder (NMOSD) is an immune-mediated inflammatory demyelinating disease of the central nervous system (CNS) mainly involving the optic nerve and spinal cord. The coexistence of NMOSD and SLE and/or SS is well recognized by both neurologists and rheumatologists, but cases in children have been rarely reported. In this paper, we reported a case of a girl with onset at age 5 clinically featured by recurrent parotid gland enlargement, pancytopenia, hypocomplementemia, multiple positive serum antibodies, and cirrhosis. She was initially diagnosed with SS/SLE overlap syndrome at age 5. Four years later, the patient suffered a sudden vision loss and was examined to have positive AQP4 antibodies in serum and cerebrospinal fluid (CSF), and long segmental spinal swelling, in line with the diagnostic criteria for NMOSD. Up to now, the current patient is of the youngest onset age to develop SS/SLE coexisting with NMOSD, also with cirrhosis. It is important for clinicians to be aware of the possibility of CTDs coexisting with NMOSD in children, especially in those with positive anti-multiple autoantibodies, and to decrease the rate of missed diagnosis.

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Section: Discussionmentioning

confidence: 92%

The First Case Report of Preschool-Onset SS/SLE Coexisting With NMOSD of Chinese Origin

et al. 2022

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“…Lymphoma presents as a firm, painless mass of variable duration. Microscopically it recapitulates Peyer's patch-type lymphoid tissue and is composed of morphologically heterogeneous small B cells, including marginal zone (centrocyte-like) cells, cells resembling monocytoid cells, small lymphocytes, and scattered immunoblasts and centroblast-like cells (75). In some cases, a plasmacytic differentiation is found, with light chain restriction shared by lymphocytes and plasma cells.…”

Section: Histopathological Features Of Salivary Gland Lymphoma and Cl...mentioning

confidence: 91%

Nuts and bolts of salivary gland pathology in primary Sjögren's syndrome

Zabotti,

Giovannini,

Longhino

et al. 2023

Clinical and Experimental Rheumatology

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Primary Sjögren's syndrome (pSS) is a chronic, systemic, inflammatory autoimmune disease characterised by lymphocyte proliferation and progressive damage to exocrine glands. Salivary gland histopathology based on salivary gland biopsy is relevant for the diagnosis of pSS and therefore broadly applied in clinical practice. Tissue can be obtained from labial salivary glands (LSG) biopsy or from major salivary glands (MSG) biopsy, namely the parotid; in this latter scenario, the procedure can be either an open surgical biopsy or a US guided core needle biopsy. In this review we will: i) present the histopathological findings that may be encountered by pathologists on biopsies from pSS patients; ii) discuss the advantages and disadvantages of the surgical and/or imaging guided procedures to obtain tissues from LSG or MSG; iii) describe the histopathological features of lymphoma of MSG in pSS patients.

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“…renal tubular acidosis), vasculitis, central nervous system (CNS) involvement, leukopenia, thrombocytopenia, anaemia, lymphadenopathy and musculoskeletal manifestations (arthralgia, arthritis, myalgia) [3,5,6,8,9]. Lymphoma is a rare complication, but still, it is reported in jSjD [17,18].…”

Section: Introductionmentioning

confidence: 99%

Microvascular status in juvenile Sjögren’s disease: the first nailfold videocapillaroscopy investigation

Lercara,

Malattia,

Hysa

et al. 2024

Clin Rheumatol

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Introduction Juvenile Sjögren’s disease (jSjD) is a rare autoimmune disease characterized by exocrine gland involvement and systemic manifestations, including small vessel vasculitis and Raynaud’s phenomenon (RP). We aimed to investigate the microvascular status in jSjD patients by nailfold videocapillaroscopy (NVC) and the potential correlations with clinical and serological features. Methods Clinical data from thirteen consecutive jSjD patients (11 females and 2 males), with a mean age of 16 ± 4 years, diagnosed before 16 years of age (mean age at diagnosis 12 ± 3) according to the 2016 American College of Rheumatology/EULAR criteria for adult SjD, were collected including age- and sex-matched healthy controls (HCs). Clinical, laboratory, and instrumental data were collected, together with NVC examination. Non-specific and specific NVC parameters were investigated, such as capillary density, capillary dilations, giant capillaries, microhaemorrhages and abnormal shapes. Associations between NVC findings and clinical/serological features were explored and analysed using parametrical and non-parametrical tests. Results Capillary density reduction correlated significantly with articular involvement (arthralgias) (p = 0.024). Microhaemorrhages correlated with lower C3 levels (p = 0.034). No specific NVC pattern for jSjD was identified, whereas abnormal capillary shapes were significantly higher in jSjD patients than HCs (p = 0.005). NVC abnormalities were not associated with SjD-specific instrumental tests (biopsy, imaging, Schirmer’s test). RP was present in 8% of jSjD patients. Conclusions The reduction of capillary density, as well as microhaemorrhages at NVC analysis, are significantly associated with some clinical aspects like articular involvement and serum biomarkers (C3 reduction). The NVC is suggested as safe and further analysis in jSjD patients. Key Points• Juvenile Sjögren’s disease (jSjD) exhibits a higher rate of abnormal shapes at nailfold videocapillaroscopy (NVC) compared to matched healthy controls.• Articular involvement in jSjD is significantly associated with a lower capillary number at NVC.• Microhaemorrhages on NVC are associated with lower C3 levels, prompting this finding as a putative novel negative biomarker/prognostic factor for jSjD.

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Extranodal marginal zone B cell lymphoma: An unexpected complication in children with Sjögren's syndrome

Cited by 18 publications

References 9 publications

The First Case Report of Preschool-Onset SS/SLE Coexisting With NMOSD of Chinese Origin

The First Case Report of Preschool-Onset SS/SLE Coexisting With NMOSD of Chinese Origin

Nuts and bolts of salivary gland pathology in primary Sjögren's syndrome

Microvascular status in juvenile Sjögren’s disease: the first nailfold videocapillaroscopy investigation

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