Renal Vein Thrombosis Suggestive of Extramembranous Glomerulonephritis Associated with Sjögren’s Syndrome (Case Report)

Introduction: Glomerular damage during Gougerot-Sjgren syndrome is much rarer than interstitial damage, and is essentially extra-membranous and membrano-proliferative glomerulonephritis. Observation: We report the case of a 44-year-old woman with primary Sjgrens syndrome, confirmed by clinical dryness syndrome, positive anti-SSA and anti-SSB antibodies, and a salivary gland biopsy revealing grade 4 lymphocytic sialadenitis according to CHISHOLMs classification. Later, the patient developed nephrotic syndrome, along with hypertension. Renal function remained normal with a creatinine level of 9.3 mg/l, and hematuria was absent. Only antinuclear antibodies tested positive, while anti-PLA2R antibodies were negative. A renal biopsy was performed, which was complicated on the same day by hemodynamic instability with hematuria. Renal CT scan with contrast injection revealed a posterior perirenal hematoma without contrast extravasation. Additionally, bilateral renal vein thrombosis was incidentally discovered, suggesting extramembranous glomerulonephritis. The patients hemodynamic status stabilized after fluid resuscitation with isotonic saline solution (0.9%), without the need for blood transfusion. Renal biopsy confirmed extramembranous glomerulonephritis with interstitial fibrosis and minimal tubular atrophy. The initial etiological assessment was negative. The patient was started on oral corticosteroids, angiotensin-converting enzyme inhibitors, and therapeutic anticoagulation for renal vein thrombosis. The patients condition improved, with the disappearance of the syndrome and spontaneous regression of the hematoma. Discussion: The association of nephrotic syndrome and renal vein thrombosis primarily suggests glomerulopathy, in particular extra-membranous glomerulonephritis. Sjgrens syndrome can be associated with extra-membranous glomerulonephritis without being its direct cause. Like, it is possible that it is a cause of glomerulonephritis, essentially extra membranous and membrano-proliferative. Conclusion: Sjgrens syndrome is generally underestimated cause of glomerulonephritis, which should be considered in cases of extra-membranous glomerulonephritis.

Enzalutamide Associated with Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) Overlap: A Case Report

Enzalutamide is a hormonal therapy that blocks the action of androgens, such as testosterone in the treatment of metastatic castration-resistant prostate cancer. Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) overlap and are part of an adverse drug reaction continuum of disease, in which there is a 10% - 30% involvement of the skin surface with mucositis, blisters, skin slough, and a macular rash. A 66-year-old male was treated with enzalutamide for metastatic prostate cancer and developed SJS/TEN overlap with 25% total body surface area skin involvement. The patient received a seven-day course of cyclosporine to which he responded by re-epithelialization but succumbed to multi-organ failure. While SJS/TEN has been reported with apalutamide, to our knowledge, this is the first case of SJS/TEN overlap with enzalutamide.

Association of HLA-B*1502 and*1511 Allele with Antiepileptic Drug-induced Stevens-Johnson Syndrome in Central China

Previous studies have demonstrated a strong association between carbamazepine（CBZ）-induced Stevens-Johnson syndrome（SJS） and HLA-B 1502 in Han Chinese. Here, we extended the study of HLA-B 1502 susceptibility to two different antiepileptic drugs, oxcarbazepine（OXC） and phenobabital（PB）. In addition, we genotyped HLA-B 1511 in a case of CBZ-induced SJS with genotype negative for HLA-B 1502. The presence of HLA-B 1502 was determined using polymerase chain reaction with sequence-specific primers（PCR-SSP）. Moreover, we genotyped HLA-B 1502 in 17 cases of antiepileptic drugs（AEDs）-induced cutaneous adverse drug reactions（cADRs）, in comparison with AEDs-tolerant（n=32） and normal controls（n=38） in the central region of China. The data showed that HLA-B 1502 was positive in 5 of 6 cases of AEDs-induced SJS（4 CBZ, 1 OXC and 1 PB）, which was significantly more frequent than AEDs-tolerant（2/32, 18 CBZ, 6 PB and 8 OXC） and normal controls（3/38）. Compared with AEDs-tolerant and normal controls, the OR for patients carrying the HLA-B 1502 with AEDs-induced SJS was 6.25（95% CI： 1.06–36.74） and 4.86（95% CI： 1.01–23.47）. The sensitivity and specificity of HLA-B 1502 for prediction of AEDs-induced SJS were 71.4%. The sensitivity and specificity of HLA-B 1502 for prediction of CBZ-induced SJS were 60% and 94%. HLA-B 1502 was not found in 11 children with maculopapular exanthema（MPE）（n=9） and hypersensitivity syndrome（HSS）（n=2）. However, we also found one case of CBZ-induced SJS who was negative for HLA-B 1502 but carried HLA-B 1511. It was suggested that the association between the CBZ-induced SJS and HLA-B 1502 allele in Han Chinese children can extend to other aromatic AEDs including OXC and PB related SJS. HLA-B 1511 may be a risk factor for some patients with CBZ-induced SJS negative for HLA-B 1502.

Stevens-Johnson syndrome and concurrent hand foot syndrome during treatment with capecitabine:A case report

BACKGROUND Capecitabine is used in combination with lapatinib as palliative treatment for human epidermal growth factor receptor 2-positive metastatic breast cancer.The most frequently reported adverse events attributed to capecitabine include diarrhea,hyperbilirubinemia,and hand-foot syndrome(HFS).A number of cutaneous adverse events have been attributed to capecitabine,including Stevens-Johnson syndrome(SJS)as a rare and potentially life-threatening mucocutaneous condition.We report the first case involving concurrent SJS and HFS after capecitabine and lapatinib treatment.CASE SUMMARY A 70-year-old woman with a history of breast cancer treatment visited our hospital for evaluation of painful skin lesions.Six weeks earlier,she had been prescribed capecitabine plus lapatinib as treatment for metastatic breast cancer.She subsequently developed worsening erythema and bullae on her palms and soles,as well as reddish macules on her back and chest wall.Histopathological evaluation of the chest wall lesions revealed extensive eosinophilic epidermal necrosis and separation of the epidermis from the dermis.The capecitabine plus lapatinib treatment was discontinued immediately and treatment was started using systemic steroids.This treatment resolved most lesions,although the lesions on her palms and soles required Vaseline gauze dressings,which resulted in reepithelialization.Therefore,we determined that the patient had concurrent SJS and HFS.Although the dermatological problems resolved,the patient ultimately died because of multiple organ failure.CONCLUSION Oral capecitabine treatment carries a risk of both HFS and also life-threatening adverse cutaneous drug reactions,such as SJS.

Spectrum of gastrointestinal involvement in Stevens-Johnson syndrome

Stevens-Johnson syndrome(SJS) or toxic epidermal necrolysis(TEN) is a severe adverse drug reaction associated with involvement of skin and mucosal membranes, and carries significant risk of mortality and morbidity. Mucus membrane lesions usually involve the oral cavity, lips, bulbar conjunctiva and the anogenitalia. The oral/anal mucosa and liver are commonly involved in SJS or TEN. However, intestinal involvement is distinctly rare. We herein review the current literature regarding the gastrointestinal involvement in SJS or TEN. This review focuses mainly on the small bowel and colonic involvement in patients with SJS or TEN.

Celiac disease and Sjögren’s syndrome:A case report and review of literature

BACKGROUND Celiac disease(CD)is a systemic,chronic immune-mediated disease triggered by gluten ingestion in genetically-susceptible individuals,with a prevalence of 1%worldwide.Sjogren's syndrome(SS)is also a systemic autoimmune disease,mainly characterized by ocular and oral sicca symptoms and signs.Sharing a common genetic background,CD and SS are known associated autoimmune diseases,but currently available guidelines are not reporting it.CASE SUMMARY We report the case of a 39-year-old woman,who was in the care of her rheumatologist for 2 years with SS.On routine follow-up she was found to have iron deficiency,without anemia.She had no gastrointestinal complaints and denied any obvious source of blood loss.IgA tissue transglutaminase antibodies were positive and endoscopy with duodenal biopsies revealed crypt hyperplasia and villous atrophy.A diagnosis of CD was set and gluten-free diet was recommended.CONCLUSION We present a review of existing data in the literature regarding the association of the two diseases,summarizing prevalence studies of CD in SS patients and the other way around.Screening recommendations and future research perspectives are also discussed,highlighting clinically relevant unanswered questions with respect to the association of CD with SS.

Pembrolizumab-induced Stevens-Johnson syndrome in advanced squamous cell carcinoma of the lung:A case report and review of literature

BACKGROUND For advanced lung squamous cell carcinoma,immune checkpoint inhibitors(ICIs)have been regarded as one of the optimal therapies.While immune-related adverse events(ir AEs)are common in ICI treatment,cutaneous toxicities are among the most common ir AEs.Most immune-related skin toxicity grades are low,and the prognosis is good.However,Stevens-Johnson syndrome(SJS)is a rare but extremely severe cutaneous adverse drug reaction with high mortality.CASE SUMMARY We report a rare case of SJS induced by pembrolizumab.The case involved a 68-year-old female who was diagnosed with advanced squamous cell carcinoma of the lung.SJS appeared after one cycle of immunotherapy combined with chemotherapy.After treatment with prednisone hormone symptoms,antiinfection,gamma globulin,and antipruritic agents,the skin toxicity of the patients gradually decreased and eventually disappeared.Although the antitumor treatment was stopped due to serious adverse reactions,the tumor of the patient remained stable for nearly half a year after one cycle of immune therapy combined with chemotherapy,which also corroborates the delayed effect of immunotherapy.CONCLUSION We believe our report can provide some references for the treatment of SJS and the treatment of immune-related adverse reactions.

Role of ranulas in early diagnosis of Sjögren’s syndrome: A case report

BACKGROUND Although the presentations of Sjögren’s syndrome(SS)are variable,ranging from mild dryness to wider systemic involvement,ranulas as early clinical signs were scarcely reported.Here,we present an adult patient with SS,who developed a unilateral simple ranula and was diagnosed primary SS 3 years later.We also provide a review of cases of SS and ranulas from 1980 to 2020.CASE SUMMARY A 22-year-old girl was found to have a left painless floor-of-mouth lesion 3 years ago,without obvious trauma or inducement.The diagnosis of a unilateral(left)simple ranula was made,and the ranula was surgically treated.Within 3 years after the ranula surgery,she developed acute lymphadenectasis in unilateral parotid twice without inducement,and ultrasonic examination revealed diffuse lesions in bilateral parotids and submandibular glands,which strongly suggested SS.Serologic tests and the unstimulated whole saliva flow rate confirmed the SS diagnosis.CONCLUSION Our study underlines that ranulas are early clinical signs of SS.As early diagnosis and early intervention of SS are important to obtain better outcomes,our findings underline the need for histopathological test after sublingual adenectomy and imaging detection of exocrine glands for the patients with ranulas.

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.

Acute middle ear Aspergillus niger infection in a patient with Stevens-Johnson syndrome: a case report

Rationale: Stevens-Johnson syndrome is a disorder mostly attributed to drug use, which can cause different skin problems. Patient concerns: A 31-year-old woman presented with severe pain and pounding in the left ear. Diagnosis: Fluconazole-induced Stevens-Johnson syndrome with fungal infection in ear. Intervention: Surgical and systemic antibiotic therapy. Outcomes: The patient recovered and resumed her normal life. Lessons: The prescription of fluconazole for patients with underlying conditions should be done with caution.

Osteo-odonto keratoprosthesis in Stevens-Johnson syndrome:a case report

·AIM:To report a successful osteo-odonto keratoprosthesis(OOKP) procedure in a case of end stage of corneal blindness due to Stevens-Johnson syndrome (SJS).·METHODS:An interventional case report.·RESULTS:We describe a 35-year-old Indian woman,a known case of SJS with bilateral dry eyes and corneal blindness (failed corneal graft with vascularised total corneal opacity in the right eye and non-healing corneal ulcer in the left eye).Vision was hand movement only in both eyes.The corneal ulcer healed with medical treatment resulting in vascularised total corneal opacity with no improvement in vision.OOKP was performed in the right eye and the vision was improved from hand movement to 6/6.The same vision was maintained in the right eye at the last follow-up 5 years after surgery.·CONCLUSION:OOKP provides good visual rehabilitation with long-term anatomically stable prosthesis in patients with end-stage of ocular surface disorders and corneal blindness secondary to SJS.·

Cutaneous mucosa-associated lymphoid tissue lymphoma complicating Sjögren's syndrome:A case report and review of literature

BACKGROUND The association of Sjögren's syndrome(SS)and lymphoma is similar.Mucosaassociated lymphoid tissue(MALT)or extranodal marginal zone B-cell lymphoma was the most common lymphomatous histology in SS patients.MALT in SS patients is frequently located in the parotid gland,while MALT lymphoma of the skin with SS is an exceedingly rare entity that needs to be recognized.CASE SUMMARY A 60-year-old woman presented with a 3-year history of progressive dry mouth associated with a 1-year history of enlarging cutaneous nodules.Physical examination revealed two hard subcutaneous nodules on her right lower leg.The results of Schirmer’s test were positive,despite the absence of dry eyes.Labial salivary gland biopsy revealed lymphocytic infiltration and chronic inflammation with a focus score of 2.The patient was diagnosed with SS.She underwent resection of one cutaneous nodule,and histopathological analysis identified the nodule as MALT lymphoma.Her dry mouth symptoms improved,and the nodules decreased after 6 mo of treatment with hydroxychloroquine sulfate and chemotherapy(thalidomide,cyclophosphamide,and dexamethasone).CONCLUSION Lymphoma is a severe complication of SS,shown by the reported unique case of cutaneous MALT lymphoma with SS.

Significant impact of cryopreserved amniotic membrane on acute ocular Stevens-Johnson syndrome and toxic epidermal necrolysis

The ocular surface is covered by an epithelium encompassing an area including the cornea,the limbus and the conjunctiva bordered by the upper and lower lids.The healthy state of the ocular surface epithelium depends on a stable and protective preocular tear film when the eye is open.A stable preocular tear film is governed by sound ocular surface defense that involves effective

AA Amyloidosis Secondary to Primary Sjögren Syndrome: Can It Be Developed without Chronic Inflammation?

Background: The association of primary Sjögren syndrome (PSS) and AA amyloidosis is a rare occurrence. Objective: To describe the phenotype of patients with this association through our two cases and a literature review. Materials and methods: A report of two cases of AA amyloidosis complicating primary Sjögren syndrome with a literature review. Results: Eight patients of Primary Sjögren’s Syndrome complicated by AA amyloidosis were studies. Six cases were reported in the literature by consulting several databases. 50% of patients had a positive immunological assessment, three cases with kidney damage, and three cases lung damage. Conclusion: The immunological activity in the Primary Sjogren’s Syndrome requires the search not only a lymphoma but also AA amyloidosis apart from any clinical or biological chronic inflammation.

Peripheral Neuropathies Revealing Gougerot-Sjögren’s Syndrome: Description of 3 Cases

Introduction: Sjögren’s syndrome is an autoimmune epithelitis with various extraglandular signs, among which are neurological, with a variable frequency according to studies. We report three cases of peripheral neuropathy revealing Gougerot-Sjögren’s syndrome, collected in the Neurology Department of the Fann University Hospital in Dakar (Senegal). Observations: The first patient, aged 48 years, presented with a length-dependent sensitivomotor polyneuropathy associated with retrobulbar optic neuritis, with dry eyes and dry mouth noticed by the patient for several years. The second patient, aged 28 years, was admitted to the hospital with chronic generalized paresthesia in the context of xerostomia and xerophthalmia. The results of the clinical examination and the electroeneuromyogram were in favour of pure sensory neuronopathy. The third patient was 32 years old female, with a history of thyroidectomy and acute inflammatory demyelinating polyneuropathy (AIDP), who was seen for acute ascending flaccid tetraplegia with facial diplegia, preceded by diffuse paresthesia. The diagnosis of recurrence of acute demyelinating polyradiculonueropathy was retained in view of the rapidly increasing character of the deficit, the hyperproteinorachy at the lumbar puncture, and the signs of demyelination at the ENMG. The diagnosis of Gougerot-Sjögren’s syndrome in our three patients was established on the basis of the 2016 ACR/EULAR criteria. Indeed, the anti-SSA antibodies (Ro) were positive in our 3 patients with a biopsy of the salivary glands which showed stage 3 in the first patient and stage 4 in the two others. Corticosteroid therapy and immunosuppressive treatment resulted in a favourable clinical evolution on the neurological and general levels. Conclusion: Gougerot-Sjögren’s syndrome is an autoimmune exocrinopathy that may present with peripheral neuropathy, which may precede the diagnosis of Sjögren’s syndrome, be concomitant or occur during the course of the disease.

Sjögren’s Syndrome Revealed by Obstructive Renal Failure: A Case Report and Review of the Literature

Introduction: Primary Sjögren’s syndrome (SS) is the most common connective tissue disease after rheumatoid arthritis and affects mostly women between 30 and 40 years of age with an estimated prevalence between 0.1% and 0.6%. This observation illustrates an incidental finding of a case of SS in a young female patient in a context of obstructive renal failure (ARF) due to uterine fibroids. Observation: This was a 31-year-old woman hospitalized for anuric AKI (Acute Kidney Injury) with a creatinine level of 1247 μmol/l. Her history included sickle cell disease A/C and an unoperated uterine fibroid diagnosed 3 years ago. Approximately 2 months before her admission, her symptomatology was made of dizziness, physical asthenia, vomiting, poly-arthralgia, morning rash, pollakiuria and oral dryness. Abdominal examination showed a painless transverse mass in the pelvis. Biological examination showed a CRP (C-reactive protein) level of 488 mg/l. The cytobacteriological examination of the urine was normal and the proteinuria was 1.35 g/24 hours. The CT scan showed kidneys measuring 110 mm on the right and 113 mm on the left associated with bilateral pyelo-caliceal dilatation on a large polymyomatous uterus of interstitial and submucosal type. Immunologically, the anti-nuclear factor, the rheumatoid factor and the anti-SSA antibodies were positive. The resumption of the interrogation within the framework of the research of the subjective dry syndrome to find a notion of intermittent xerophthalmia 4 months ago. The Schirmer test was positive in the left eye. The initial management consisted of a polymyomectomy after 3 sessions of hemodialysis. Background treatment combining prednisone 5 mg/day and methotrexate 20 mg/week was started in parallel with the use of artificial tears. The evolution after twelve (12) months of treatment was favorable with a complete disappearance of the signs dry syndrome and full recovery of renal function. Conclusion: SS can have an insidious evolution and remain stable for many years, hence its fortuitous discovery in this case of obstructive ARF on uterine fibroid. In this context we insist on the interest of the immunological assessment in a patient in period of genital activity with a significant proteinuria and non-specific extrarenal signs.

实例探讨药物引起SJS或TEN的评分方法——Naranjo评分与ALDEN评分比较

药物引起的史蒂文斯-约翰逊综合征(SJS)和中毒性表皮坏死松解症(TEN)是一种罕见严重的不良反应,其特征是广泛性的皮肤表皮剥离和黏膜侵犯。当评估可疑药物和SJS或TEN之间的因果关系时,除了Naranjo评分外,最近根据两个大型的病例对照研究结果所衍生的ALDEN评分也提供了新思路。本文结合1例同时服用对乙酰氨基酚和头孢拉定导致中毒性表皮坏死综合征的案例,应用ALDEN评分及Naranjo评分分析两种药物与ADR间的关联性,并讨论此两种评分方法的差异性及适宜性。

亚洲人群HLA-B*1502等位基因与卡马西平引起SJS/TEN的关联性荟萃分析

目的：探讨亚洲人群人类白细胞抗原（HLA）-B＊1502等位基因对卡马西平引起Stevens-Johnson综合征和中毒性表皮坏死松解症（Stevens-Johnson syndrome/toxic epidermal necrolysis,SJS/TEN）的影响。方法：检索Pubmed、Embase、中国期刊网、万方和维普等数据库,按照纳入标准纳入有关HLA-B^＊1502等位基因与卡马西平引起SJS/TEN的病例对照研究,应用Review Manager4.2和Stata10.0进行荟萃分析。结果：共纳入10篇文献（10个病例对照研究）,共计卡马西平引起的SJS/TEN患者208例,对照者829例。荟萃分析显示合并RR（95%CI）为10.95（8.30-14.45）（Z=16.93,P〈0.00001）。结论：HLA-B^＊1502等位基因与卡马西平引起SJS/TEN存在明显关联。

原发性干燥综合征合并血细胞减少中医诊治研究进展

原发性干燥综合征(pSS)是一种慢性炎症性自身免疫病,除累及外分泌腺之外,还可累及多器官、系统,其中以血液系统受累最为常见,多表现为一系或多系血细胞减少。目前对于pSS合并血细胞减少缺乏统一公认的治疗方案,西医治疗多以糖皮质激素、免疫抑制剂为主,而中医药治疗本病具有较好疗效。通过总结pSS合并血细胞减少的中医诊治经验,并对相关研究进行综述后发现,现代医家认为pSS核心病机为气阴亏虚,久之则产生热、毒、瘀等多种病理产物,相互影响,导致全身气血津液失常,引起血细胞减少。本病病位多责之于肝、脾、肾,治以滋阴、益气、养血之法,辅以清热解毒、化瘀通络之法,并根据其合并血细胞减少类型的不同以及是否使用糖皮质激素用药各有侧重。中医药治疗本病较西药治疗能够更好地改善血细胞水平且不良事件发生率更低,具有一定优势。

五运六气与干燥综合征发病特点及中医证候的相关性研究

目的:探讨原发性干燥综合征(pSS)患者出生时的五运六气等相关信息与pSS发病所累及的系统损害及中医证候的相关性。方法:采用回顾性研究方法,从门诊及住院部收集2020年1月—2021年12月就诊的pSS患者,收集其出生时的运气学相关信息资料,统计运、气因素出现的频次,分析其与pSS发病所累及的系统损害及中医证候相关的运气因素,并运用中医理论进行分析、总结、探讨。结果:岁运因素中“土不及”是pSS患者的易患禀赋因素;岁运因素中“土不及”、六气因素中的“太阴司天、太阳在泉”及“厥阴司天、少阳在泉”是pSS气阴两虚证的易患禀赋因素;岁运因素中“土不及”“木太过”“金太过”“水太过”“水不及”,六气因素中“少阴司天、阳明在泉”及“少阳司天、厥阴在泉”为阴虚津亏证pSS的易患禀赋因素;岁运因素中“火太过”,六气因素中“太阴司天、太阳在泉”“少阴司天、阳明在泉”是阴虚热毒证pSS的易患禀赋因素;岁运因素中“木太过”“金不及”“木不及”,六气因素中“厥阴司天、少阳在泉”为阴虚血瘀证pSS的易患禀赋因素;岁运因素中“土不及”“金不及”,六气因素中“太阴司天、太阳在泉”为pSS患者关节受累的易患禀赋因素;岁运因素中“土不及”“金不及”为pSS患者肺受累的易患禀赋因素;岁运因素中“火太过”“土太过”“土不及”,六气因素中“厥阴司天、少阳在泉”为pSS患者血液系统受累的易患禀赋因素。结论:原发性干燥综合征的发病特点及其中医证候与五运六气具有一定的相关性。