Primary Sjogren's syndrome : rarity in India

Objective : Primary Sjogren's syndrome (SS) is rarely reported from India. We have studied the clinical spectrum and immunological profile of patients with primary SS. Methods : A prospective analysis of patients with primary Sjogren's syndrome fulfilling San Francisco criteria, seen at our clinic in the last 10 years was carried out. Results : The study included 26 patients, 21 being women. The presenting symptoms included dry eyes, dry mouth, and arthritis/ arthralgia. Extra-glandular manifestations were glomerulonephritis, vasculitis, renal tubular acidosis and peripheral neuropathy. The important laboratory abnormalities were hypergammaglobulinaemia (16/20), antinuclear antibodies (18/26), anti-La (11/19) and anti-Ro (10/19). Minor salivary gland provided a definitive diagnosis in 16/26 (60%). Conclusion : The prevalence of primary Sjogren's syndrome is rare even in tertiary care rheumatology clinics. The clinical and immunological profile as seen here is similar to that reported in Western countries

Primary central nervous system lymphoma: Prognostication as per international extranodal lymphoma study group score and reactive CD3 collar

Background : Primary central nervous system (CNS) lymphoma is being increasingly recognized in immunosuppressed as well as immunocompetent individuals. It has a poor prognosis and the majority of these have diffuse large B-cell type of morphology. Aim : To categorize cases of primary CNS lymphoma according to the International Extranodal Lymphoma Study Group (IELSG) score and to correlate the score with reactive CD3 collar around blood vessels and necrosis. Materials and Methods : We reviewed the clinico-pathological, morphological and immuno-histochemical features of 30 cases of primary CNS lymphoma reported at our institute in the last nine years and categorized them according to the score given by IELSG. Results : All our cases were HIV-negative and had diffuse large cell histology. Twenty-seven of the 30 cases were associated with poor prognostic factors of intermediate to high risk according to the IELSG score. Reactive CD3 collar around the blood vessels was seen in seven cases which had low to intermediate IELSG score. However, no significant statistical difference of perivascular reactive CD3 collar and necrosis was seen with IELSG score. Conclusion : Approximately 90% (27 cases) were associated with poor prognostic factors in the present study according to the IELSG score. Perivascular reactive CD3 collar was seen in cases with low to intermediate IELSG score. A larger study is required to further validate that the presence of reactive perivascular CD3 collar is associated with good prognosis. This histological marker could be supplemented with IELSG score to stratify the patients of primary CNS lymphoma according to their aggressiveness

A case of collapsing glomerulopathy associated with febrile illness

Collapsing glomerulopathy (CG) is a distinct clinicopathological entity characterized by high levels of nephrotic range proteinuria, rapidly progressive renal failure, marked parenchymal injury, and poor response to present therapeutic regimens. Growing awareness has led to the identification of associated conditions other than human immunodeficiency virus (HIV) and idiopathic. We report a case of CG from India in a HIV-negative young female, presenting with heavy proteinuria and rapidly progressing renal failure preceded by a febrile illness

Prevalence of RET/PTC expression in papillary thyroid carcinoma and its correlation with prognostic factors in a north Indian population

<b>Context</b> : The prevalence of Rearranged during Transfection/Papillary Thyroid Carcinoma (RET/PTC) rearrangement in papillary thyroid carcinoma (PTC) varies in different geographic regions and its prognostic significance remains unclear. <b>Aim</b> : The aim of this study was to recognize the prevalence of RET/PTC expression in PTC from the endemically iodine-deficient region in Northern India and to correlate the expression with the clinicopathologic prognostic factors. <b>Settings and Design</b> : Retrospective. Archival tissue used. <b>Materials and Methods</b> : Immunohistochemistry was performed to look for activated RET protein expression in 50 cases of PTC. No patient had any history of prior irradiation<b> . Statistical Analysis Used</b> : Chi-square method, Student <i>t</i> test, and binary regression method. A <i>P</i> value of &lt; .05 was considered significant for all the tests. <b>Results</b> : The prevalence of RET expression was 44&#x0025;. Twenty-six (52&#x0025;) cases showed RET immunoreactivity in histiocytes. Immunoreactivity was the highest in the classic variant of PTC (47.5&#x0025;), followed by tumors with poorly differentiated areas (25&#x0025;) and follicular variant (16.7&#x0025;). RET expression was more prevalent in young patients (45.5 vs. 35.3&#x0025;), females (43.3 vs. 40.0&#x0025;), small tumors (33.3 vs. 26.7&#x0025;), multicentric tumors (36.8 vs. 33.3&#x0025;), tumors with extrathyroidal invasion (38.9 vs. 32.4&#x0025;), and regional lymphadenopathy (55.2 vs. 22.2&#x0025;), while it was less in cases with distant metastases (20 vs. 43.9&#x0025;). There was no significant correlation of immunoreactivity with any prognostic factor. However, when the cases having immunoreactivity within histiocytes (n=26) and histiocytes &#x002B; tumor tissue (n=28) were considered, then the expression was significantly more in cases with lymphadenopathy (<i>P</i> values=.009, in both instances). However, the exact clinical significance of RET/PTC positive histiocytes remained unexplained. <b>Conclusions</b> : Prevalence of RET/PTC in our study was consistent with the reported prevalence from other geographic areas. There was no significant correlation with the clinicopathologic factors. However, uniform techniques of detection and large international collaborative studies could clear the uncertainties regarding the prognostic importance of RET/PTC

Primary central nervous system lymphoma: Prognostication as per international extranodal lymphoma study group score and reactive CD3 collar

<b>Background</b> : Primary central nervous system (CNS) lymphoma is being increasingly recognized in immunosuppressed as well as immunocompetent individuals. It has a poor prognosis and the majority of these have diffuse large B-cell type of morphology. <b>Aim</b> : To categorize cases of primary CNS lymphoma according to the International Extranodal Lymphoma Study Group (IELSG) score and to correlate the score with reactive CD3 collar around blood vessels and necrosis. <b>Materials and Methods</b> : We reviewed the clinico-pathological, morphological and immuno-histochemical features of 30 cases of primary CNS lymphoma reported at our institute in the last nine years and categorized them according to the score given by IELSG. <b>Results</b> : All our cases were HIV-negative and had diffuse large cell histology. Twenty-seven of the 30 cases were associated with poor prognostic factors of intermediate to high risk according to the IELSG score. Reactive CD3 collar around the blood vessels was seen in seven cases which had low to intermediate IELSG score. However, no significant statistical difference of perivascular reactive CD3 collar and necrosis was seen with IELSG score. <b>Conclusion</b> : Approximately 90&#x0025; (27 cases) were associated with poor prognostic factors in the present study according to the IELSG score. Perivascular reactive CD3 collar was seen in cases with low to intermediate IELSG score. A larger study is required to further validate that the presence of reactive perivascular CD3 collar is associated with good prognosis. This histological marker could be supplemented with IELSG score to stratify the patients of primary CNS lymphoma according to their aggressiveness

An insufficient anti-inflammatory cytokine response in mouse brain is associated with increased tissue pathology and viral load during Japanese encephalitis virus infection

Infection of the central nervous system with Japanese encephalitis virus (JEV) results in fatal encephalitis in humans. No reports exist describing the sequence of pathological changes and their correlation to the immune response in the brain following infection with JEV. In this report, we analyzed inducible nitric oxide synthase (iNOS) mRNA, proinflammatory (IFN-γ, TNF-α) and anti-inflammatory (IL-4, IL-10) cytokine expression, viral load, and the correlation of these factors with the major histopathological changes in brain of JEV challenged mice at different time points during infection. We report for the first time that in JE, there is a progressive decline in the level of IL-4. The extent of progressive decrease in IL-4 and IL-10 level following viral infection is inversely correlated to the increased level of proinflammatory cytokines and histopathological changes with negative consequences following viral infection. In contrast, proinflammatory mediators like IFN-γ and TNF-α were significantly upregulated (P &#60; 0.05). A negative correlation between IFN-γ and iNOS indicates their independent actions during JEV infection. To conclude, an insufficient anti-inflammatory cytokine response indicated by IL-4 and IL-10 in the brain is associated with increased tissue pathology and viral load, which regulates inflammatory responses driven by IFN-γ in concert with TNF-α to cause brain tissue damage

Prevalence of RET/PTC expression in papillary thyroid carcinoma and its correlation with prognostic factors in a north Indian population

Context : The prevalence of Rearranged during Transfection/Papillary Thyroid Carcinoma (RET/PTC) rearrangement in papillary thyroid carcinoma (PTC) varies in different geographic regions and its prognostic significance remains unclear. Aim : The aim of this study was to recognize the prevalence of RET/PTC expression in PTC from the endemically iodine-deficient region in Northern India and to correlate the expression with the clinicopathologic prognostic factors. Settings and Design : Retrospective. Archival tissue used. Materials and Methods : Immunohistochemistry was performed to look for activated RET protein expression in 50 cases of PTC. No patient had any history of prior irradiation . Statistical Analysis Used : Chi-square method, Student t test, and binary regression method. A P value of < .05 was considered significant for all the tests. Results : The prevalence of RET expression was 44%. Twenty-six (52%) cases showed RET immunoreactivity in histiocytes. Immunoreactivity was the highest in the classic variant of PTC (47.5%), followed by tumors with poorly differentiated areas (25%) and follicular variant (16.7%). RET expression was more prevalent in young patients (45.5 vs. 35.3%), females (43.3 vs. 40.0%), small tumors (33.3 vs. 26.7%), multicentric tumors (36.8 vs. 33.3%), tumors with extrathyroidal invasion (38.9 vs. 32.4%), and regional lymphadenopathy (55.2 vs. 22.2%), while it was less in cases with distant metastases (20 vs. 43.9%). There was no significant correlation of immunoreactivity with any prognostic factor. However, when the cases having immunoreactivity within histiocytes (n=26) and histiocytes + tumor tissue (n=28) were considered, then the expression was significantly more in cases with lymphadenopathy (P values=.009, in both instances). However, the exact clinical significance of RET/PTC positive histiocytes remained unexplained. Conclusions : Prevalence of RET/PTC in our study was consistent with the reported prevalence from other geographic areas. There was no significant correlation with the clinicopathologic factors. However, uniform techniques of detection and large international collaborative studies could clear the uncertainties regarding the prognostic importance of RET/PTC

Kinetics of cytokine profile during intraperitoneal inoculation of Japanese encephalitis virus in BALB/c mice model

Infection with Japanese encephalitis virus (JEV) is mostly asymptomatic/subclinical in 90% of the individuals. Host immune response during subclinical JEV infection is poorly understood. We assessed iNOS, IFN-γ, TNF-α, IL-10 and IL-4 production in spleen, brain and sera of intraperitoneally challenged BALB/c mice by RT-PCR and ELISA along with brain histopathology at different days post inoculation (d.p.i.). In spleen of virus infected mice, expression of all cytokines including iNOS mRNA were upregulated till 5 d.p.i. followed by decline. At 5 d.p.i., IL-10 expression outcompeted TNF-α, IFN-γ and IL-4. However, in the virus infected mice sera, IL-4 production predominated over TNF-α and IL-10 at 5 d.p.i. Conversely, cytokines expression and iNOS mRNA remained unchanged in the brain of virus infected mice from 1 to 7 d.p.i. A significant increase in the cytokine expression was observed at 11 d.p.i. (P &lt; 0.05) in virus infected mice brain, with the predominance of IL-10 along with the presence of meningeal inflammation and viral RNA by histology and RT-PCR, respectively. We report a biased pattern of cytokine production in sera, brain and spleen of mice intraperitoneally challenged with JEV. IL-10 exerts neuroprotective function during JEV and regulates deleterious effects of proinflammatory cytokines; however, its mechanism needs further investigation