Ziwan-Taoren herb pair can exert an therapeutical effect in primary Sjogren’s syndrome through inhibiting the TLR/NF-κB pathway

Background:Ziwan and Taoren(ZT)is a classic medicine pair in the formula of Mai Dong Di Shao Decoction,has been used to treat primary Sjogren’s syndrome(pSS)for more than 20 years.But its action mechanism is still unknown.This study is aimed to reveal the potential mechanism of ZT treated pSS and discover its active compounds of ZT and therapeutic target for pSS.Methods:Firstly,the potential pathways of ZT for pSS treatment were predicted through network pharmacology and GO and KEGG enrichment analysis.Secondly,the inter-structural relationships between active compounds of ZT and target proteins were visualized using molecular docking techniques.Finally,efficacy and mechanism were conducted through in vivo experiments,such as water intake,spleen index,hematoxylin-eosin staining pathological changes,ELISA,Western Blot analysis,and immunofluorescence staining.Results:Nine active compounds were extracted from network pharmacology,including quercitrin,luteolin,kaempferol,β-sitosterol,isorhamnetin,galangin,hederagenin,diosmetin and gibberellin 7.Seven disease targets were identified:RELA,TP53,AKT1,interleukin(IL)6,MAPK1,ESR1,IL10;with RELA being the most core target.KEGG and GO enrichment analysis indicated that ZT may act through the TLR/NF-κB/RELA inflammatory mechanism process.preliminary results of molecular docking showed that ZT’s active compounds bind well to the RELA(p65)receptor.In vivo results demonstrated that a high dose of ZT significantly improved water intake and reduced lymphocytes infiltration in submandibular gland pathology in NOD mice.The expression content of AQP5 and vasoactive intestinal peptide in the submaxillary gland was significantly increased,while levels of inflammatory factors such as tumor necrosis factor-α,IL-6,and IL-1βalong with protein expressions including toll-like receptor4,p-p65 and p-IKKα/βin NF-κB pathway were reduced.Conclusions:The ZT treatment exhibits a promising efficacy in mitigating dryness symptoms of pSS,potentially attributed to its capacity for suppressing the TLR/NF-κB inflammatory signaling pathway.

Higher frequency of brain abnormalities in neuromyelitis optica spectrum disorder patients without primary Sjogren's syndrome

Neuromyelitis optica spectrum disorder often co-exists with primary Sjogreffs syndrome. We compared the clinical features of 16 neuro- myelitis optica spectrum disorder patients with （n = 6） or without primary Sjogreffs syndrome （n = 10）. All patients underwent extensive clinical, laboratory, and MRI evaluations. There were no statistical differences in demographics or first neurological involvement at onset between neuromyelitis optica spectrum disorder patients with and without primary Sjogren＇s syndrome. The laboratory findings of cerebrospinal fluid oligoclonal banding, serum C-reactive protein, antinudear autoantibody, anti-Sjogren＇s-syndrome-related antigen A an- tibodies, anti-Sjogren＇s-syndrome-related antigen B antibodies, and anti-Sm antibodies were significantly higher in patients with primary Sjogren＇s syndrome than those without. Anti-aquaporin 4 antibodies were detectable in 67% （4/6） of patients with primary Sjogren＇s syndrome and in 60% （6/10） of patients without primary Sj6gren＇s syndrome. More brain abnormalities were observed in patients without primary Sj6gren＇s syndrome than in those with primary Sj6gren＇s syndrome. Segments lesions （〉 3 centrum） were noted in 50% （5/10） of patients without primary Sj6gren＇s syndrome and in 67% （4/6） of patients with primary Sjogren＇s syndrome. These findings indicate that the clinical characteristics of neuromyelitis optica spectrum disorder patients with and without primary Sjogren＇s syndrome are similar. However, neu- romyelitis optica spectrum disorder patients without primary Sjogreffs syndrome have a high frequency of brain abnormalities.

Clinical Profile and Significance of Mucocutaneous Lesions of Primary Sjogren＇s Syndrome： A Large Cross-sectional Study with 874 Patients

Background： Mucocutaneous lesions are common features of primary Sjogren＇s syndrome （pSS）, but only a few studies have focused on them. To demonstrate the profile ofmucocutaneous lesions ofpSS and further explore their potential clinical significance, we perfermed a cross-sectional study on 874 patients. Methods： Demographic data, clinical manifestations, and laboratory results of 874 pSS patients were collected. Patients were divided into two groups according to the presence of mucocutaneous lesions. Differences in primary symptoms and systemic impairments between the two groups were analyzed. Results of laboratory tests were also compared alter excluding those who had taken corticosteroid from both groups. One-year follow-up was done, and occurrences of various new complications were compared. Results： Among the 874 pSS patients, 181 patients had mucocutaneous lesions, accounting for 20.7%. Multiple mucocutaneous manifestations were displayed, and the top four most common types of lesions were purpuric eruptions （39.8%）, urticaria （23.8%）, Raynaud＇s phenomenon （14.9%）, and angular stomatitis （9.9%）. Incidences of pulmonary interstitial fibrosis, pulmonary bullae, leukopenia, and anemia were significantly higher among patients with mucocutaneous lesions （P 〈 0.05）. Increase in lgG and decrease in C4 among patients with mucocutaneous lesions displayed statistical significance after excluding patients from both groups who had taken corticosteroid （P 〈 0.05）. Alter one-year follow-up, patients with mucocutaneous lesions presented a slightly higher incidence of new complications compared to those without. Conclusions： Mucocutaneous manifestations ofpSS patients were common and diverse. Patients with mucocutaneous manifestations had more systemic damages, higher level of IgG, and lower level of serum C4, suggesting a higher activity of the primary disease.

Visual resolution under photopic and mesopic conditions in patients with Sjogren's syndrome

AIM:To focus on different visual resolution tasks under photopic and mesopic conditions in Sjogren’s syndrome patients compared to age-matched healthy controls.METHODS:The visual resolution measurements included high and low visual acuities and contrast sensitivity functions.These tests were conducted under photopic and then mesopic conditions.Twenty-one Sjögren’s syndrome patients and 21 aged-matched healthy volunteers completed all the measurements in this study.RESULTS:Sjogren’s syndrome patients have greater impairment in contrast sensitivity than standardized visual acuity.This reduction was significant under the mesopic condition.Also,Sjogren’s syndrome patients treated with pilocarpine suffer more than patients without pilocarpine treatment under low light conditions.CONCLUSION:Sjogren’s syndrome patients shows greater impairment in different visual resolution tasks due to dry eye symptoms.

Persistent Xerophthalmia in a Patient with Rheumatological Disease and Priiviary Sjogren’s Syndrome: Case Report from Northern Brazil

This case study aims to contribute to the literature in order to highlight the importance of this collaboration between medical specialties. A female patient R.N.N. F, age 66, from the city of Manaus, with a previous diagnosis of Sjogren’s syndrome in regular follow-up by the Rheumatology team at the Araujo Lima outpatient clinic and referred to the Ophthalmology sector for complementary evaluation related to visual discomfort. The fundoscopy performed in the patient was within normal limits, but the symptoms experienced by her proved to be an important clinical finding, which has ratified the need for regular and multidisciplinary follow-up. This report unequivocally demonstrates that even in the face of tests considered within the expected limits for a given population, the clinical presentation can be specific and particular for each analyzed individual. Early screening exams should contemplate the patient in a holistic and individualized way whenever possible.

Traditional Chinese medicine compound ShengJinRunZaoYangXue granules for treatment of primary Sjogren＇s syndrome： a randomized, double-blind, placebo-controlled clinical trial

Background Traditional Chinese medical treatment of primary Sj（o）gren＇s syndrome has advantages over Western medicine in terms of fewer side effects and improved patient conditions.This study was a multicenter,randomized,doubleblind,placebo-controlled clinical trial of the efficacy and safety of ShengJinRunZaoYangXue granules for the treatment of primary Sj（o）gren＇s syndrome,including the symptoms of dry mouth and dry eye.Methods We undertook a 6-week,double-blind,randomized trial involving 240 patients with primary Sj（o）gren＇s syndrome at five centers in East China.A computer-generated randomization schedule assigned patients at a 2∶1 ratio to receive either ShengJinRunZaoYangXue granules or placebo once daily.Patients and investigators were blinded to treatment allocation.The primary endpoints were the salivary flow rate,Schirmer test results,and sugar test results.Intention-to-treat and per-protocol analyses were performed.Results All 240 patients were randomly allocated to either the treatment group （n=160,ShengJinRunZaoYangXue granules） or placebo group （n=80） and were included in the intention-to-treat analysis.After program violation and loss to follow-up,a total of 199 patients were included in the per-protocol analysis.At six week,intention-to-treat and per-protocol analyses of the left-eye Schirmer I test results showed an improved difference of 1.36 mm/5 min （95% CI：0.03 to 2.69 mm/5 min） and 1.35 mm/5 min （95% CI：0.04 to 2.73 mm/5 min）,respectively,and those of the right-eye Schirmer I test results showed an improved difference of 1.12 mm/5 min （95% CI：0.02 to 2.22 mm/5 min） and 1.12 mm/5 min （95% CI：-0.02 to 2.27 mm/5 min）,respectively.There was no significant difference between the two groups before treatment.After treatment,the between-group and within-group before-and-after paired comparison results were statistically significant （P 〈0.05）.Intention-to-treat and per-protocol analyses showed an improved salivary flow rate by 0.04 ml/15 min （95% CI：-0.49 to 0.58 ml/15 min） and 0.04 ml/15 min （95% CI：-0.52 to 0.60 ml/15 min）,respectively,but the differences were not significant.Intention-to-treat and per-protocol analyses showed that the sugar test results were improved by 1.77 minutes （95% CI：0.11 to 3.44 minutes） and 1.84 minutes （95% CI：0.12 to 3.55 minutes）,respectively,but the differences were not significant.For the secondary endpoint,intention-to-treat and per-protocol analyses showed significant improvement in the integrated evaluation of treated patients with dry eye and dry mouth after six weeks of treatment.The incidence of adverse events was 15.6% in the treatment group and 10.0% in the placebo group.Most （94%） adverse events were mild to moderate in the two groups,and only two cases of serious adverse events occurred in the treatment group; both were caused by autoimmune liver disease.Conclusions Six-week treatment with ShengJinRunZaoYangXue granules for primary Sj（o）gren＇s syndrome in this large-scale study improved the symptoms of dry mouth,dry eyes,and low tear flow rate with minimal adverse events.

Gastrointestinal microbiome and primary Sjogren’s syndrome: a review of the literature and conclusions

The recognition of the profound impact of the human gastrointestinal microbiome(GM) on human autoimmune diseases has gradually increased thanks to deeper research efforts. As a systemic autoimmune disease, primary Sjogren’s syndrome(pSS) cannot be completely cured. Human studies have revealed that GM species and diversity are altered in patients with p SS compared with healthy individuals. Animal studies have provided possible mechanisms for the association between pSS and GM. The potential role of GM in pSS is exerted through several mechanisms. GM dysbiosis leads to increased intestinal permeability, which increases the risk of GM antigen exposure and activates specific autoreactive T lymphocytes via “molecular mimicry”. In addition, GM antigen exposure and intestinal immune tolerance loss caused by GM dysbiosis together induce chronic local gut mucosal inflammation, which deteriorates to systemic chronic non-specific inflammation with the circulation of pro-inflammatory lymphocytes and cytokines. These factors eventually activate autoreactive B lymphocytes and lead to pSS. If GM plays a key role in the pathogenesis of pSS, clarifying the underlying mechanisms will be helpful for the development of new therapies targeting GM for dry eye associated with pSS. This review summarizes the latest knowledge about the relationship between GM and p SS,with the aim of contributing to future research and to the development of new clinical applications.

Primary Sjogren’s syndrome and focal proliferative and crescentric necrotizing glomerulonephritis

Intro: Primary Sjogren’s syndrome is a chronic systemic autoimmune disease. Although ocular and oral dryness are the hallmarks of this syndrome, renal involvement when present most commonly has the histological appearance of tubointerstitial nephritis and less often glomerular involvement. We report, a unique case of renal manifestations of primary Sjogren’s syndrome with a proven histological focal proliferative and crescentric necrotizing glomeru-lonephritis. Case: A 30 years old man with Kimura disease presented with four months of subjective fevers, migrating skin erythema, dry mouth, and dry eyes. On further workup the patient was found to have a positive ANA, positive anti-SSA, and was evaluated by ophthalmology who found keratoconjunctivitis sicca. The patient was started on hydroxychloroquine in combination with prednisone. On follow up, the patient was found to be hypertensive with acute kidney injury, a creatinine of 3.65, proteinuria and hematuria. Renal ultrasound was unremarkable. Cytoplasmic ANCA was positive and kidney biopsy was performed revealing non pauci-immune focal proliferative and necrotizing glomerulonephritis with cellular crescents and mesangial dense deposits by electron microscopy. Conclusion: To our knowledge, the association between biopsy proven focal proliferative and necrotizing glomerulonephritis with primary Sjogren’s syndrome has never been previously described in the literature, nor has there been a reported case of association between Kimura disease, ANCA serology and primary Sjogren’s syndrome.

Hypokalemic Paresis Revealing a Primary Sjogren’s Syndrome

Hypokalemic acidosis can complicate a primary Sjögren’s syndrome. The clinical feature is rarely revealed by manifestations due to hypokalemia. We report the case of a 46-year-old woman, admitted to explore a paresthesia and paresis of inferior limbs. The diagnosis of Sjögren’s syndrome was retained since there was the association of xerophthalmia, sialadenitis at the labial biopsy and positive immunological results (anti-SSA and anti-SSB). The absence of another auto-immune or systemic illness allowed us to consider that the Sjögren’s syndrome was primary. The biological explorations revealed a hyperchloremic and hypokalemic acidosis. The treatment was based on corticosteroid and potassium supplementation. The follow-up was marked by a clinical and biological amelioration.

A Case Report of Mesenteric Panniculitis and Primary Sjogren’s Syndrome

Mesenteric Panniculitis is a benign fibro-inflammatory process involving adipose tissue of the mesentery. It is characterised by fat necrosis, chronic inflammation and fibrosis, causing thickening and shortening of the mesentery. Patients may present with localised abdominal pain, abdominal mass, intestinal obstruction and ischaemic colitis. We report a case of mesenteric panniculitis causing abdominal pain in a patient with active Primary Sjogren’s Syndrome. The rarity of this case makes it of interest. We review the current literature on mesenteric panniculitis and its association with connective tissue disease and inflammatory conditions. A 64-year-old Caucasian male presented in 1994 with dry mouth. A diagnosis of Primary Sjogren’s Syndrome (PSS) was made on salivary gland biopsy. In 2010 he presented with an exacerbation of his eye symptoms, muscle pain and fatigue. He complained of abdominal pain and night sweating, but denied any weight loss or change in bowel habit. There was no significant past medical history other than PSS. On examination he had a small right submandibular node and mild synovitis at the right proximal interphalangeal joint and carpometacarpal joint. Examination of the abdomen showed marked umbilical tenderness but no organomegally. Blood tests at this time showed an active inflammation: CRP of 61 (NR 5 mg/L), ESR 39 (NR 20 mm/s), strongly positive ENA Ro and La. IgG was elevated at 18.6 (NR 5.8-15.4), Complement was low at 0.17 (NR 0.18-0.6). An abdominal ultrasound scan demonstrated a 6 × 3 ×3 cmarea of diffuse homogenous fat encasing some mesenteric vessels in the area of focal tenderness. CT abdomen and pelvis showed oedematous mesenteric fat and lymph nodes in the jejunal small bowel mesentery, consistent with mesenteric panniculitis. Laparoscopic biopsy was discussed with the surgical team, but was felt not indicated as risk outweighed potential benefit. The patient was treated with a 9-week reducing course of oral steroids. His abdominal symptoms resolved although CT abdomen showed little improvement in mesenteric panniculitis. A review of the literature suggests that currently there is no standard treatment and management should be guided by patient symptoms. Mesenteric Panniculitis is rare;as a result evidence for treatment is limited to individual case reports. There is no clear link between symptom improvement and radiological resolution of mesenteric panniculitis. It has, therefore been suggested that follow-up imaging should be limited to those with persistent symptoms. Overall the prognosis for mesenteric panniculitis is good, up to half of patients do not require treatment, and recurrence of symptoms is uncommon.

Tubulointerstitial Nephritis Complicated with Primary Sjogren’s Syndrome under Treatment for Type 2 Diabetes Mellitus

Tubulointerstitial nephritis complicated by primary Sj?gren’s syndrome in a patient under treatment for type 2 diabetes mellitus was diagnosed in the early stage of the disease by renal biopsy. The symptoms of primary Sj?gren’s syndrome, such as thirst and polydipsia, were masked by the characteristic symptoms of type 2 diabetes mellitus. An association between sicca symptoms and diabetes mellitus (types 1 and 2) has been previously reported. Hence, it is possible that there are common underlying immunological mechanisms between primary Sj?gren’s syndrome and diabetes mellitus of both types. Intervention with steroids in a timely manner appears to have prevented or slowed the progression of re- nal impairment.

Management of primary ciliary dyskinesia/Kartagener＇s syndrome in infertile male patients and current progress in defining the underlying genetic mechanism

Kartagener＇s syndrome （KS） is an autosomal recessive genetic disease accounting for approximately 50% of the cases of primary ciliary dyskinesia （PCD）. As it is accompanied by many complications, PCD/KS severely affects the patient＇s quality of life. Therapeutic approaches for PCD/KS aim to enhance prevention, facilitate rapid definitive diagnosis, avoid misdiagnosis, maintain active treatment, control infection and postpone the development of lesions. In male patients, sperm flagella may show impairment in or complete absence of the ability to swing, which ultimately results in male infertility. Assisted reproductive technology will certainly benefit such patients. For PCD/KS patients with completely immotile sperm, intracytoplasmic sperm injection may be very important and even indispensable. Considering the number of PCD/KS susceptibility genes and mutations that are being identified, more extensive genetic screening is indispensable in patients with these diseases. Moreover, further studies into the potential molecular mechanisms of these diseases are required. In this review, we summarize the available information on various aspects of this disease in order to delineate the therapeutic objectives more clearly, and clarify the efficacy of assisted reproductive technology as a means of treatment for patients with PCD/KS-associated infertility.

EVALUATION OF INTERNATIONAL CLASSIFICATION CRITERIA (2002) FOR PRIMARY SJGREN'S SYNDROME IN CHINESE PATIENTS

Objective To evaluate the sensitivity and specificity of international classification criteria (2002) for primary Sjgren’s syndrome (pSS) and the role of lower lip biopsy in diagnosis of pSS in Chinese patients. Methods Patients who were diagnosed by the experts/rheumatologists as pSS during 1990-2002 from the Depart- ment of Rheumatology, Peking Union Medical College Hospital were retrospectively collected as experimental group. Patients who were diagnosed as non-pSS connective tissue diseases or non-connective tissue diseases served as control group. Those with a history of head-neck radiation, hepatitis C virus infection, AIDS, lymphoma, sarcoidosis, graft versus host disease (GVHD), and anti-acetylcholine drug use were exempted. Both groups were required to complete question- naires about symptoms such as dry eyes and dry mouth, and complete the objective tests of keratoconjunctivitis and xero- stomia including Schirmer test, corneal staining, unstimulated salivary flow, sialography, lower lip biopsy, and antinuclear antibodies (including anti-SSA/SSB antibodies) test. Results A total of 330 pSS patients were included in experimental group and 185 non-pSS patients in control group. The mean age of both groups matched (47.8 ± 10.9 years vs. 46.2 ± 13.6 years, P > 0.05). The sensitivities of the criteria in pSS patients with lower lip biopsy and in pSS patients without lower lip biopsy were 89.2% and 87.2%, respectively; the overall sensitivity was 88.5%. The specificity was 97.3%. A total of 11.3% pSS patients with negative anti-SSA/SSB anti- bodies were diagnosed as pSS by lower lip biopsy. Conclusion The international classification criteria (2002) for pSS is feasible in Chinese patients. It has high sensitivity and specificity, and may serve as diagnosis criteria in routine clinical practice.

Comparison of corneal biomechanics in Sjogren's syndrome and non-Sjogren's syndrome dry eyes by Scheimpflug based device

AIM：To compare the corneal biomechanics of Sj？gren＇s syndrome（SS） and non-SS dry eyes with Corneal Visualization Scheimpflug Technology（CorV is ST）.METHODS：Corneal biomechanics and tear film parameters, namely the Schirmer I test value, tear film break-up time（TBUT） and corneal staining score（CSS） were detected in 34 eyes of 34 dry eye patients with SS（SSDE group） and 34 dry eye subjects without SS（NSSDE group） using CorV is ST. The differences of the above parameters between the two groups were examined, and the relationship between corneal biomechanics and tear film parameters were observed. RESULTS：The differences in age, sex, intraocular pressure（IOP） and central corneal thickness（CCT） were not significant between the two groups（P〉0.05）. The tear film parameters had significant differences between the SSDE group and NSSDE group（all P〈0.05）. Patients in the SSDE group had significantly lower A1-time and HC-time, but higher DA（P=0.01, 0.02, and 0.02, respectively） compared with the NSSDE group. In the SSDE group, DA was negatively correlated with TBUT（rho=-0.38, P=0.03）; HC-time was negatively correlated with CSS（rho=-0.43, P=0.02）. In the NSSDE group, HC-time was again negatively correlated with CSS（rho=-0.39, P=0.02）.CONCLUSION：There are differences in corneal biomechanical properties between SSDE and NSSDE. The cornea of SSDE tends to show less ＂stiffness＂, as seen by a significantly shorter A1-time and HC-time, but larger DA, compared with the cornea of NSSDE. Biomechanical parameters can be influenced by different tear film parameters in both groups.

Primary aldosteronism due to bilateral micronodular hyperplasia and concomitant subclinical Cushing’s syndrome:A case report

BACKGROUND Adrenal incidentaloma(AI)has been frequently encountered in the clinical setting.It has been shown that primary aldosteronism(PA)or subclinical Cushing’s syndrome(SCS)are the representative causative diseases of AI.However,the coexistence of PA and SCS has been reportedly observed.Recently,we encountered a case of AI,in which PA and SCS coexisted,confirmed by histopathological examinations after a laparoscopic adrenalectomy.We believe that there were some clinical implications in the diagnosis of the present case.CASE SUMMARY A 58-year-old man presented with lower right abdominal pain with a blood pressure of 170/100 mmHg.A subsequent computed tomography scan revealed right ureterolithiasis,which was the cause of right abdominal pain,and right AI measuring 22 mm×25 mm.After the disappearance of right abdominal pain,subsequent endocrinological examinations were performed.Aldosterone-related evaluations,including adrenal venous sampling,revealed the presence of bilateral PA.In addition,several cortisol-related evaluations showed the presence of SCS on the right adrenal adenoma.A laparoscopic right adrenalectomy was then performed.The histopathological examination of the resected right adrenal revealed the presence of a cortisol-producing adenoma,while CYP11B2 immunoreactivity was absent in this adenoma.However,in the adjacent nonneoplastic adrenal,multiple CYP11B2-positive adrenocortical micronodules were detected,showing the presence of aldosterone-producing adrenocortical micronodules.CONCLUSION Careful clinical and pathological examination should be performed when a patient harboring AI presents with concomitant SCS and PA.

Iguratimod in treatment of primary Sjögren’s syndrome concomitant with autoimmune hemolytic anemia:A case report

BACKGROUND Primary Sjögren's syndrome(pSS)concomitant with autoimmune hemolytic anemia(AIHA)but without eye and mouth dryness is exceedingly rare.Iguratimod(IGU)has been widely used in the treatment of pSS.However,there are few reports about the application of IGU in pSS concomitant with AIHA.CASE SUMMARY Here,we present the case of a patient with pSS concomitant with AIHA but without eye and mouth dryness.The patient was initially diagnosed with hyperplastic anemia and AIHA while pSS was missed,and was finally diagnosed with pSS concomitant with AIHA.The patient was treated with IGU along with prednisone and hydroxychloroquine,and her hemoglobin,reticulocytes and IgG returned to normal levels.CONCLUSION IGU was effective for and well tolerated by our patient with pSS concomitant with AIHA,and may be a promising therapy for the treatment of this disease.

Pulmonary amyloidosis and multiple myeloma mimicking lymphoma in a patient with Sjogren’s syndrome:A case report

BACKGROUND Sjogren’s syndrome(SS),which affect salivary gland function,is an autoimmune disease.SS may involve extraglandular organs.Approximately 10 to 20 percent of SS patients have clinically significant lung disease,but presentation of pulmonary amylodosis is extremly rare.The incidence of benign monoclonal gammopathy in SS patients is high,but multiple myeloma is rare.No case involving the simultaneous occurrence of two rare diseases,pulmonary amyloidosis and multiple myeloma,in the same patient with SS has been reported so far.CASE SUMMARY A 41-year-old male patient was referred to our hematology department due to incidentally detected gastric plasmacytoma.He had been diagnosed with SS four years earlier.Multiple miliary nodules,ground glass opacity in both lung fields,and enlargement of both inguinal lymph nodes was observed on chest and abdomen computer tomography.Based on the pathological findings of lung and lymph node biopsied specimens,the patient was diagnosed with pulmonary amyloidosis and multiple myeloma.Pulmonary amyloidosis and multiple myeloma associated with SS has rarely been reported.CONCLUSION This is an extremely rare case of simultaneous pulmonary amyloidosis and multiple myeloma in the same patient with SS.

NF-κB Controls Resistance of Human Salivary Gland (HSG) Cells to Apoptosis in an <i>in Vitro</i>Model of Sjogren’s Syndrome

Aim: To elucidate the anti-apoptotic properties of nuclear factor kappa light-chain-enhancer of activated B cells (NF-κB) and feedback regulation of NF-κB by nuclear factor of kappa light-chain-enhancer of activated B-cells inhibitor alpha (IκBα). Methods: We developed an in vitro model of Sjogren’s syndrome by transfecting human salivary gland (HSG) and acinar cells (NS-SV-AC) with a plasmid-encoding IκBαM (pCMV-IκBαM), a degradation-resistant IκBα (nuclear factor of kappa light polypeptide gene enhancer in B-cells inhibitor, alpha)-mutant, and examined TNF-induced apoptosis and anti-apoptotic properties of NF-κB. Apoptosis and induction of pro-apoptotic and anti-apoptotic genes were investigated by cDNA arrays, RT-PCR, electrophoretic mobility shift assays, and western blot. Results: In the presence of NF-κB inhibitors, TNF-induced apoptosis was markedly increased in both salivary gland and acinar cells. Increased caspase-3 activity was present in both HSG and NS-SV-AC cells. IκBαM-transfected salivary gland cells were more sensitive to TNF-induced apoptosis than IκBαM-transfected acinar cells. Transcription of pro-apoptotic genes was confirmed in both HSG and NS-SV-AC cells that were transfected with IκBαM. Results from caspase-3 activity assay confirmed previous experiments showing an apoptotic role for NF-κB. Conclusion: Data from gene expression arrays suggest that different mechanisms may operate during TNF-induced apoptosis in salivary gland ductal and acinar cells.

Hypergammaglobulinemic purpura of waldenstorm associated with sjogren’s syndrome in a young female responding to rituximab treatment

Hypergammaglobulinemic Purpura of Waldenstorm is one of the uncommon conditions with purpura and is often associated with collagen vascular disease. It is difficult to treat and sometimes needs anti CD 20 molecules for resistant cases.

A case of recurrent rhabdomyolysis associated with childhood Sjogren’s syndrome

We report a 9-year-old Japanese girl who presented with muscle weakness and elevated serum levels of muscle-derived enzymes following mycoplasma infection. Rhabdomyolysis or myositis was suggested by magnetic resonance imaging and repeated four times within 4 years. Each episode developed following respiratory infection and spontaneously recovered. The diagnosis of Sjogren’s syndrome was made by decreased salivary secretion, MR sialography, lip biopsy, and positive anti-SSA/Ro antibody. Given the rarity of rhabdomyolysis/myositis, recurrent episode could be induced by infectious disease on the basis of underlying Sjogren’s syndrome. Conclusion: Sjogren’s syndrome should be considered as an underlying disease of recurrent infection-induced rhabdomyolysis/ myositis.