Nongranulomatous Inflammation Research Articles

The Diagnostic Utility of Lip Biopsy in Paediatric Crohn's Disease: A 10-year Single-centre Retrospective Study

BackgroundOral manifestations of paediatric Crohn's disease (CD) are reported in up to 60% of cases. Lip biopsy can be used to histologically diagnose oral CD. We evaluated the utility of lip biopsy in children under initial investigation for potential CD. MethodsA 10-year retrospective review of electronic patient records at a single tertiary paediatric surgery centre was performed. All patients aged ≤16 years who underwent lip biopsy were included. Clinical features, histology, and diagnostic details were extracted. ResultsForty-two children underwent lip biopsy. Median age at biopsy was 13.3 years (11.0–14.9). Final diagnosis was CD in 21/42 (50%) children, indeterminant colitis in 3/42 (7%), orofacial granulomatosis (OFG) in 3/42 (7%), coeliac disease in 1/42 (2%), and eosinophilic oesophagitis in 1/42 (2%). Thirteen children (31%) received no formal diagnosis. The most common symptoms reported were oral ulceration (33/42, 79%), lip swelling (21/42, 50%), and abdominal pain (19/42, 45%). Lip biopsy histology was normal in 11/42 (26%). In 24/42 (57%), non-granulomatous inflammation was seen. In 7/42 (17%) lip biopsy identified granulomatous inflammation: three (7%) had endoscopic biopsies concordant for CD, three (7%) had negative endoscopic biopsies but were diagnosed with CD, and one was diagnosed with OFG (2%). Sensitivity was 29% and specificity was 95%. ConclusionLip biopsy has low sensitivity but high specificity for diagnosing CD. Lip biopsy diagnosed CD in 7% when endoscopic biopsies were negative, enabling treatment. LB is a useful diagnostic test for CD in children presenting with oral symptoms. Level of EvidenceIII.

Cessation of ocular hypertensive attacks following iStent trabecular micro-bypass stent implantation in a patient with Posner-Schlossman syndrome: a case report

Background: Posner-Schlossman syndrome (PSS) is an ocular condition with self-limiting recurrent episodes of markedly elevated intraocular pressure (IOP) and non-granulomatous anterior chamber inflammation. Surgical treatment is indicated if medical therapy fails to control IOP.
 Case presentation: A 58-year-old male presented with intermittent attacks of blurred vision and pain in the right eye for 6 months. Examination revealed keratic precipitates with IOP of 56 mmHg in the right eye. Gonioscopy revealed open angles in both eyes. Erythrocyte sedimentation rate was within normal limits, while aqueous tapfor viral PCR, rapid plasma reagent test, and Mantoux test were negative. IOP was in the high-teens (inappropriate) on 2 topical antiglaucoma medications and the patient developed ocular surface problems. iStent was implanted. IOP remained stable within the mid-teens with no further episodes of raised IOP in the following 6 months.
 Conclusion: This case showed cessation of ocular hypertensive attacks in PSS after iStent implantation.

Significance of non-granulomatous cytomorphology on fine needle aspirate in lymphadenitis cases classified as tuberculous by using a composite reference standard.

Fine needle aspiration cytology (FNAC) is established as a first line investigation for tuberculous lymphadenitis (TBLA). We aimed to describe the various cytomorphologic features of tuberculosis (TB) on FNAC and their contribution in the diagnostic decision-making in suspected TBLA cases. Patients with presumptive TBLA were prospectively enrolled (n = 266) and subjected to routine diagnostic work-up for TB, including FNAC samples, and followed until the end of treatment. Patients were categorized as TB or non-TB cases based on a composite reference standard of which the various cytomorphologic patterns were compared. Sensitivity, specificity, positive predictive value, negative predictive value and accuracy was calculated using cross-tabulation. Fifty-six patients were categorized as bacteriologically confirmed TB, 102 as clinically confirmed TB and 108 as non-TB. The most common cytomorphologic pattern among TB cases (59%) was granulomatous inflammation with necrosis, however, about one-third of tuberculous lymphadenitis patients presented with non-granulomatous inflammation, with 21% showing only necrosis and 13% presenting with a reactive pattern. The overall sensitivity and specificity of FNAC was 85% and 66%, respectively. We found that about one-third of TBLA patients presented without granulomas on FNA, highlighting the importance of considering TB in a wide spectrum of cytomorphology in a high TB burden setting. Our study supports the use of FNAC as a first-line investigation tool for diagnosing TBLA in a low-resource setting due to its relative simplicity and good sensitivity. However, the low specificity of FNAC, emphasizes the need for a second-tier confirmatory test with improved specificity.

Rapidly evolving gangrenous vasculitis in a critically ill patient with ulcerative colitis: a case report

Ulcerative colitis is a disease characterised by non-granulomatous submucosal inflammation ranging from isolated proctitis to colitis. Extra-intestinal manifestations of the condition occur in multiple organ systems, with dermatological complications occurring commonly. This case report aims to highlight an uncommon dermatological complication of ulcerative colitis with particular focus on patient care and management.

P024 Vasculitis of the female genital tract: is it a single organ or systemic disease?

Abstract Background/Aims Vasculitis of the female genital tract is rare. At one institution, incidence among all gynaecological surgeries was 0.15%. It is usually an incidental finding on histopathological examination after surgery done for another reason. Most commonly, it presents with vaginal bleeding, but can also present with pelvic pain, uterine prolapse, asymptomatic abdominal mass or atypical cervical smear. Constitutional and musculoskeletal symptoms are reported in about a quarter of cases. It can be localised to the female genital tract, most commonly the uterus and cervix. Less commonly, it can be part of a systemic vasculitis, including giant cell arteritis, polyarteritis nodosa, granulomatosis with polyangiitis, microscopic polyangiitis, cryoglobulinaemic vasculitis, lupus and rheumatoid vasculitis. Although data is limited, one review found 31.3% of cases to have systemic involvement. Approximately 60% of patients with vasculitis of the female genital tract and systemic vasculitis had GCA. In this group, about one third do not present with classical cranial, systemic or polymyalgia rheumatica symptoms. In localised disease, anaemia or raised inflammatory markers are unlikely. Histopathology mostly shows non-granulomatous inflammation. With systemic involvement, anaemia, raised inflammatory markers and granulomatous inflammation are more likely to be present. Localised vasculitis of the female genital tract tends not to require treatment. However, it can predate symptoms and signs of systemic disease. Therefore, follow-up for at least 12 months is recommended. Methods Written consent was obtained from the patient. Data was obtained from the patient's case notes, electronic records and histopathology results. Results We describe a 63-year-old lady with no past medical history and on no medications who presented with post-menopausal bleeding and abdominal cramping. Hysteroscopy showed two endometrial polyps. Biopsy showed grade 1A endometrial carcinoma. Total hysterectomy and bilateral salpingo-oophorectomy was performed. Histopathological analysis confirmed endometrial cancer but also showed some foci of granulomatous inflammation with multi-nucleating giant cells surrounding both small and medium calibre vessels. The vessels showed fibrinoid necrosis and disruption of the internal elastic lamina, suspicious for vasculitis. On review by the rheumatology team this lady was found to be well with no history of Raynaud’s; rashes; arthritis; ocular; ear, nose and throat; gastroenterological; respiratory; neurological; or constitutional symptoms. Examination was normal. C-reactive protein and immunoglobulins were normal. Anti-nuclear antibody, anti-neutrophil cytoplasmic antibody, rheumatoid factor and anti-CCP antibody were negative. Urine protein was raised at 0.29 (0-0.1) but protein/creatinine ratio was normal at 11 mg/mmol. Chest x-ray was clear. Conclusion In conclusion, female genital tract vasculitis is rare and is usually an incidental finding on histopathology. In this case, it appeared to be an isolated vasculitis of the female genital tract. It is important to recognise that uncommonly this condition can be associated with systemic involvement and therefore follow up in rheumatology clinic is recommended. Disclosure T. Nadin: None. N. Goadby: None. R. Smith: None.

Severe Ulcerative Colitis-like Pattern of Segmental Colitis Associated with Diverticulosis Complicated with a Colo-umbilical Fistula: Case Report

A 49-year-old African American male with history of diverticulitis presented to the ED with abdominal pain and feculent discharge from the umbilicus. CT scan of the abdomen showed a fistulous tract from the sigmoid colon to the umbilicus. Labs revealed a markedly elevated fecal calprotectin level and colonoscopy demonstrated friable-appearing mucosa of the sigmoid colon. Segmental colitis associated with diverticulosis (SCAD) refers to a chronic, localized, nongranulomatous inflammation, usually in the sigmoid colon. SCAD spares the rectum and the proximal colon endoscopically and histologically. We present a case of diverticular disease associated with an unusual fistula and features of SCAD.

Acquired nasolacrimal duct obstruction: clinical and histological findings of 275 cases

BackgroundAcquired nasolacrimal duct obstruction is a blockage of the lacrimal outflow system usually caused by local nonspecific inflammation of the lacrimal sac and the nasolacrimal duct. However, cases exist where the primary nasolacrimal system obstruction is caused by malignancies.Our aim was to investigate lacrimal sac pathologies in patients with acquired nasolacrimal duct obstruction and compare their clinical manifestations.MethodsThis retrospective clinical study included 275 patients with acquired nasolacrimal duct obstruction who underwent external dacryocystorhinostomy and lacrimal sac biopsy. Cases were classified into tumor or nonspecific pathology groups and subdivided according to the level of inflammation. Histological and clinical data were analyzed.ResultsThree tumors (1.1%) (an adenoid cystic carcinoma, an eccrine spiradenoma and small B cell lymphoma) were diagnosed. Chronic nongranulomatous inflammation was the most common histological finding, corresponding to 194 cases (70.5%). The other 81 (29.5%) were subacute, acute forms of nongranulomatous inflammation, tumors and fibrosis cases. Epiphora with continuous purulent discharge was the most common clinical sign reported by 144 (52.4%) patients, and two (0.7%) patients had a palpable mass near the medial canthal tendon, which was identified as an eccrine spiradenoma and small B cell lymphoma. There was no significant difference in the clinical symptoms, duration or case history between the nonspecific pathology and tumor groups (p = 0.292).ConclusionsChronic nongranulomatous inflammation of the lacrimal sac was the most common finding among acquired nasolacrimal duct obstruction cases. There were no associations between the histological findings and clinical presentation. The authors recommend a lacrimal sac biopsy only in cases when a tumor is clinically suspected.

Orbital Tumours in Northern Malaysia: A Five-Year Review.

Background: This study was conducted with the objective to examine demographic statistics and histopathological diagnoses of orbital biopsies from cases referred to the Oculoplastic subspecialty of the Ophthalmology department in Hospital Sultanah Bahiyah, Kedah, from 2016 to 2020.Method: This study is a case series of 28 patients who underwent orbital biopsy.Result: A total of 34 orbital biopsies from 28 patients were recorded. The mean age was 48.3 ± 19.1 years old. 22 (78.4%) cases manifested unilaterally and six (21.4%) manifested bilaterally. The commonest presentations were orbital mass (36.6%) and proptosis (24.4%). The mean duration of the presentation was 16.2 ± 19.5 months. Fourteen (50.0%) patients underwent orbital biopsy within six months of symptoms. 52.8% of the tumours are situated at supero-temporal region of the orbit. 53.0% (18) situated in extraconal space of orbit. Out of all, 23 (67.6%) cases were benign and 11 (32.4%) cases were malignant. All were primary in origin (100%). The commonest orbital tumours reported were malignant lymphoma (29.4%), reactive lymphoid hyperplasia (14.7%), non-caseating granulomatous inflammation (11.8%), non-granulomatous inflammation (5.9%) and cavernous haemangioma (5.9%). Of all 10 malignant lymphomas from eight patients (two were bilateral eyes) were all mucosa-associated lymphoid tissue (MALT) of primary non-Hodgkin lymphomas. None of the cases were reported to be metastasis from the systemic spread. The majority of patients' eyes (20, 58.8%) remained the same visual acuity post-biopsy while six (17.6%) eyes had improvement in visual acuity and eight (23.5%) eyes had reduced postoperative visual acuity.Conclusion: Malignant tumours are more common in the elder age group especially malignant lymphoma which is in contrast to Caucasian populations. Understanding the relative incidence of these various orbital tumours is essential to patient evaluation and management.

IgG4-related disease in patients with idiopathic orbital inflammation

BackgroundTo identify the prevalence of positive IgG4 immunostaining in orbital tissue among patients previously diagnosed with nongranulomatous idiopathic orbital inflammation (IOI) and to compare the clinical characteristics of patients with and without IgG4-positive cells.MethodsA retrospective review of all patients with a histopathologic diagnosis of IOI was performed. Immunohistochemical staining was performed to identify IgG-positive cells and IgG4-positive cells. Multivariate analysis was performed using likelihood ratio-test logistic regression on the differences between IgG4-related disease (IgG4-RD) and non-IgG4-RD.ResultsOf the 45 patients included, 21 patients (46.7%) had IgG4-positive cells, with 52.4% being male and a mean age of 55.9 ± 13.4 years. Bilateral ocular adnexal involvement (adjusted odds ratio [aOR] = 9.45; P = 0.016) and infraorbital nerve enlargement (aOR = 12.11; P = 0.008) were frequently found in IgG4-RD patients. Complete remission occurred in 23.8% of IgG4-RD patients and 41.7% of non-IgG4-RD patients. IgG4-RD patients had more frequent recurrent disease than non-IgG4-RD patients.ConclusionsNearly 50% of IgG4-RD patients were previously diagnosed with biopsy-proven IOI. IgG4-RD was more frequent in patients with bilateral disease and infraorbital nerve enlargement, showing the importance of tissue biopsy in these patients. Immunohistochemistry studies of all histopathology slides showing nongranulomatous IOI are highly recommended to evaluate for IgG4-RD.

АКТУАЛЬНЫЕ ПРЕДСТАВЛЕНИЯ ОБ ИДИОПАТИЧЕСКОМ НЕГРАНУЛЕМАТОЗНОМ ВОСПАЛЕНИИ ОРБИТЫ

Brief survey of the literature devoted to idiopathic non-granulomatous inflammation of the orbit which shows significant polymorphism of the clinical symptoms and absence of clear criteria of the diagnostics. Relevant publications are reviewed and latest data relating to epidemiology, classification and differential diagnosis of this disease is summarized.

Masses of the Lacrimal Gland: Evaluation and Treatment.

Lacrimal gland lesions account for approximately 9 to 10% of all biopsied orbital masses. Potential causes include nongranulomatous and granulomatous inflammation, autoimmune disease, lymphoproliferative disorders, benign epithelial proliferation, malignant neoplasia, and metastatic disease. Inflammatory lesions and lymphoproliferative disorders are the most common and may be unilateral or bilateral; they may also be localized to the orbit or associated with systemic disease. Both benign and malignant epithelial lacrimal gland masses tend to be unilateral and involve the orbital lobe, but a more rapid onset of symptoms and periorbital pain strongly suggest malignant disease. On orbital imaging, both inflammatory and lymphoproliferative lesions conform to the globe and surrounding structures, without changes in adjacent bone, whereas epithelial lacrimal gland masses often show scalloping of the lacrimal gland fossa. Malignant epithelial lacrimal gland tumors can also have radiographic evidence of bony invasion and destruction. Masses of the lacrimal gland may be due to a broad range of pathologies, and a good working knowledge of common clinical characteristics and radiographic imaging findings is essential for diagnosis and treatment. All patients with inflammatory, lymphoproliferative, and epithelial neoplastic lesions involving the lacrimal gland require long-term surveillance for disease recurrence and progression.

Case of Angular Blepharitis Caused by Demodex folliculorum

Angular blepharitis is inflammation of the eyelid skin at the lateral canthus, most commonly caused by Staphylococcus and Moraxella species. The Demodex mite is an external parasite that often infests the human eyelid margin and has been implicated in both anterior and posterior blepharitis. The authors describe the case of a 43-year-old female who presented for evaluation of a 3-month history of bilateral canthal eyelid irritation, consistent with angular blepharitis. This is a case report and review of relevant literature. Skin biopsy showed evidence of Demodex folliculorum infestation. Multiple D. folliculorum organisms were visualized within the hair follicles and on the surface, along with pockets of acute and chronic nongranulomatous inflammation surrounding hair follicles. The patient was treated with tea tree oil applied to her eyelids and eyelashes twice daily with complete resolution of symptoms. To our knowledge, this is the first report of angular blepharitis with histopathologic confirmation of Demodex infestation. In this discussion, we cover manifestations of the Demodex mite, angular blepharitis, and treatment options. Due to the lack of literature regarding this entity, we feel that it may be an underrecognized periocular and dermatologic condition that can lead to misdiagnosis, visits to multiple practitioners, and significant functional and cosmetic sequelae to the patient. Demodex infestation should be considered on the differential diagnosis for cases of refractory angular blepharitis, particularly when unresponsive to topical steroids. Based on results from treating Demodex anterior and posterior blepharitis and this case, treatment with tea tree oil should be considered.

Clinical profile of patients with anterior uveitis

The anterior uveitis can be categorized as iritis, anterior cyclitis and iridocyclitis. It often causes a painful red eye. Patients with anterior uveitis complain of redness, photophobia, tearing and blurred vision. Acute anterior uveitis causes mild vision loss but still contributes significantly to the total burden. It causes vision loss both directly through inflammation and via complications such as macular edema, glaucoma, cataract, and others. A prospective clinical study was conducted. The material for this study included, 50 patients between age 20 and 80 years, attending outpatient department, Department of Ophthalmology with signs and symptoms of anterior uveitis. In the present study it was observed that most common presentation was acute anterior uveitis, accounting for 76%, then chronic 18% and only 6% of the patients had recurrent anterior uveitis. In the present study 45 (90%) patients had non granulomatous inflammation and in 5 (10%) patients it was granulomatous inflammation. Thus nongranulomatous inflammation was more common than granulomatous inflammation.

The clinical profile and aetiological pattern of anterior uveitis- A hospital based study

Uveitis is a group of complex inflammatory disorder of the uveal tract with diverse aetiology. The most common formis anterioruveitis affecting the iris and pars plicata of the ciliary body which can lead to severe vision threatening complications if not treated appropriately. 1. To study the incidence, modes of presentation and etiological patternof anterior uveitis. 2. To evaluate the treatment, its outcome and the complications that could occur over the study period. A prospective cross sectional clinical study was conducted in the department of ophthalmology in 30 patients who were clinically diagnosed with anterior uveitis over a period of one year. All the patients underwent adetailed ophthalmic evaluation and relevant laboratory investigations. They were givenappropriate treatment according to the cause and the clinical clues and were also followed up for anycomplication during the study period. Anterior uveitis occurred most commonly in the 41 to 50 years age group. Majority of the cases had nongranulomatous inflammation (86%) but aetiology remained unknown in 30% of the cases. Herpetic infection (16%) was the commonest cause followed by phacolyticuveitis and tuberculosis. Most cases responded favourably to medical management. A balanced view of the disease needs to be taken while investigating and treating anterior uveitis. A thorough history and physical examination is required in each case to facilitate a final diagnosis. A timely diagnosis and appropriate treatment however can improve visual outcome.

Uveitis in juvenile idiopathic arthritis.

Inflammatory eye diseases comprise some of the most devastating complications of childhood rheumatic diseases, especially juvenile idiopathic arthritis (JIA). Chronic (initially asymptomatic) uveitis is the most common ocular complication of oligoarticular JIA. It is predominantly an insidious onset, anterior, nongranulomatous inflammation affecting the iris and ciliary body—an iridocyclitis (Fig. 20–1). Acute (symptomatic) anterior uveitis is characteristic of human leukocyte antigen-B27 (HLA-B27)– associated diseases such as enthesitis-related arthritis. The posterior uveal tract, the choroid, is rarely affected clinically.

Clinicopathologic spectrum of necrotizing lymphadenitis.

Necrotizing lymphadenitis represents a group of diseases characterized by non-granulomatous inflammation and necrosis of the lymph node, caused by a variety of infective and inflammatory diseases, most common being Kikuchi-Fujimoto disease, acute Epstein Barr viral infection and systemic lupus erythematosis (1). To study the morphological features in lymph nodes in cases of necrotizing lymphadenitis, to correlate them with specific etiological conditions.Materials and methods-58 cases of necrotizing lymphadenitis were reviewed and categorized into different etiological sub types, i.e. acute EBV lymphadenitis, lupus lymphadenitis and the rest as Kikuchis lymphadenitis. Morphological features studied were presence of vascular proliferation, periadenitis, foamy macrophage, neutrophil and plasma cell infiltrate. Clinical follow up was done. 62.2% of cases were Kikuchis lymphadenitis. Both lupus and Kikuchis had a female preponderance (78% and 62% respectively). Among the morphological parameters, plasma cell infiltration and vascular proliferation showed significant association with lupus lymphadenitis. Kikuchis and EBV lymphadenitis showed self-limiting course, with only 2 cases of Kikuchis developing recurrence .4 cases developed complications. All cases of lupus lymphadenitis needed long term therapy. Kikuchis lymphadenitis is the most common cause of necrotizing lymphadenitis, followed by lupus and acute EBV lyphadenitis.Young females were commonly affected in the first 2 groups. It is worthwhile to classify the cases of necrotizing lymphadenitis into etiological subgroups as the prognosis and treatment differ (2). Among the morphological features studied, plasma cell infiltrate and vascular proliferation were significantly associated with lupus lymphadenitis, hence can be used to predict etiology.

A Case of Sarcoidosis Presenting as Refractory Arrhythmias: Quieting the Storm

Introduction Sarcoidosis is a multisystem granulomatous disorder of unknown etiology. Cardiac sarcoidosis can manifest as conduction abnormality, ventricular tachycardia (VT), heart failure or sudden cardiac death. Although well studied in the literature, we present an uncommon case of arrhythmia as a presenting manifestation of this disease. Case 45-year-old female with no significant past medical history presented with acute onset shortness of breath and palpitations. Initial transthoracic echocardiogram revealed an ejection fraction of 50%. Electrophysiology (EP) study showed a chemically inducible accelerated idioventricular rhythm with right bundle morphology. Left heart catheterization showed angiographically normal vessels and cardiac MRI showed no regional wall motion abnormalities or abnormal delayed enhancement. Patient presented to the hospital again due to recurrence of arrhythmia, found to be VT. Nuclear medicine whole body PET scan showed perfusion defects consistent with cardiac sarcoidosis. A mediastinal lymph node biopsy demonstrated focal non-granulomatous inflammation. Patient was initiated on anti-arrhythmics and underwent a single-chamber ICD placement. She continued to have episodes of wide QRS tachycardia, and was evaluated by Advanced Heart Failure for refractory arrhythmias. Decision-making Patient was initially managed with Amiodarone and a beta-blocker. Due to unstable VT, she was taken to EP lab where a focal origin of the VT was successfully found in the basal anterolateral endocardial left ventricle and at the base of one of the papillary muscles. Ablations were performed in these areas with reduction in VT rate. Patient was also discovered to have complete atrioventricular block. Subsequently, she developed recurrence of arrhythmia and was admitted again for VT management. Patient was taken back to the EP lab for an endocardial and epicardial ablation. A focal VT source was found in the apical lateral left ventricle. Due to proximity of the phrenic nerve to the site, no ablations were performed. However, due to recurrence of arrhythmia, she was taken back to the lab for a third ablation in the epicardial area with resulting success. Antiarrhythmics were changed to Flecainide which has since suppressed her VT. Patient has been maintained on Methotrexate, Hydroxychloroquine, and Prednisone for continued sarcoid therapy. We are using periodic F-FDG PET imaging to monitor the severity of myocardial inflammation. Based on patient's clinical status and PET imaging, we have determined the time to wean immunosuppressant agents. Conclusion Cardiac sarcoidosis affects at least 25% of sarcoidosis patients and accounts for substantial mortality and morbidity.Ventricular arrhythmias may be the presenting symptom of cardiac sarcoidosis and are challenging to treat. Risk stratification through proposed techniques such as advanced cardiac imaging and EP studies is integral in this population. Recognition of sarcoidosis as a possible etiology of arrhythmias through integration of clinical and imaging findings can lead to appropriate lifesaving treatments.

Nasal and Lacrimal Sac Histopathology in Patients With Systemic Sarcoidosis Undergoing External Lacrimal Drainage Surgery.

To review the histological findings in the lacrimal sac and nasal mucosa from patients with sarcoidosis undergoing external lacrimal drainage surgery. All patients undergoing external dacryocystorhinostomy at Moorfields Eye Hospital with a known history of sarcoidosis had biopsies taken from the lacrimal sac and/or nasal mucosa during surgery. These patients were identified from databases at Moorfields Eye Hospital and the Institute of Ophthalmology, and their clinical notes were reviewed retrospectively for intraoperative findings with a view to identifying common trends. The histological findings of each biopsy were reviewed and classified as showing granulomas, nongranulomatous inflammation, or nonspecific fibrosis. Forty patients (29 females; 72%) were known to have systemic sarcoidosis prior to surgery, and they underwent 60 external dacryocystorhinostomies. Paired histological samples were available from 49/60 (82%) procedures, nasal biopsies alone in 3 dacryocystorhinostomies (5%), and solely lacrimal sac biopsies in 8 (13%). The main site of systemic sarcoidosis was pulmonary involvement (19 patients; 48%). Recorded operative findings included 9 large lacrimal sac mucoceles (29%), a "thick" (26%) or "inflamed" (9.7%) lacrimal sac mucosa, and "thick" (36%) or "friable" (32%) nasal mucosa. Noncaseating granulomas were identified in 34/57 (60%) sacs, and 45/52 (87%) nasal tissues-this being in 31/49 (63%) of paired tissues. Chronic inflammation, without granulomas, was present in 20/57 (35%) lacrimal sacs but only in 5/52 (9.6%) of nasal biopsies. In patients with sarcoidosis undergoing external dacryocystorhinostomy, the characteristic histological feature-noncaseating granulomas-is present in most patients' lacrimal sac mucosa and in almost all of their nasal mucosae. The lacrimal sac and nasal mucosa often appears abnormal-thickened or friable-during surgery.

Vancomycin-Associated Hemorrhagic Occlusive Retinal Vasculitis: A Clinical-Pathophysiological Analysis

To derive novel insights into the pathophysiology of vancomycin-related hemorrhagic occlusive retinal vasculopathy (HORV) through a careful clinicopathologic correlation. We retrospectively reviewed the clinical and pathologic course of 2 consecutive patients who developed HORV. The clinical history, multimodal imaging, ultrasound biomicroscopy (UBM), and intraoperative and histologic findings are reported. Both patients presented with decreased vision and eye pain within 1week following otherwise uncomplicated cataract extraction and were diagnosed with HORV after endophthalmitis was ruled out. Both patients presented with significant ocular discomfort that progressively worsened, and both experienced a dismal visual outcome despite early aggressive medical and surgical therapy. One patient requested enucleation for a blind and painful eye. Upon histologic examination of this eye, the iris and ciliary body appeared to be infarcted with separation of the iris and ciliary epithelia from their adjacent stromal components. These findings were corroborated by UBM of the second patient. Histologic examination of the posterior segment demonstrated severe hemorrhagic necrosis of the neurosensory retina and an occlusive nonarteritic vasculopathy of the retina and choroid. The choroid was thickened by prominent nongranulomatous chronic inflammation accompanied by a glomeruloid proliferation of small vessels. The inflammatory infiltrate was almost exclusively confined to the choroid and consisted of predominantly Tcells. There was conspicuous absence of inflammatory cells in the retina and no histologic evidence of leukocytoclastic vasculitis. HORV is a rare condition that can lead to profound vision loss. Significant ocular pain can be a presenting sign of HORV in cases with severe iris and ciliary body ischemia. Although it has been suggested that HORV is a form of leukocytoclastic retinal vasculitis, the histologic findings herein indicate that the pathophysiology is more complex. It is grounded in a necrotizing retinal vasculopathy in the absence of retinal vasculitis, chronic nongranulomatous choroiditis, and an unusual glomeruloid proliferation of endothelial cells in the choroid and elsewhere in the eye.

Autoimmune Hepatitis and Sarcoidosis

Abstract Background: Sarcoidosis is a systemic disorder of unknown etiology. It affects the organ systems in the body, and is characterized by the presence of noncaseating granulomas in the involved organs. The most common manifestation is bilateral hilar adenopathy along with skin, joint, and eye lesions. Here, we discuss an unusual manifestation of sarcoidosis along with autoimmune hepatitis (AIH). Case report: We report the case of a 33-year-old African American female presented with two-day history of progressive jaundice and deranged Liver function test (LFTs) the autoimmune work up was positive for anti-nuclear antibody (ANA) 1:640 and anti-smooth muscle antibody (SMA) 1:160, The serum Angiotensin converting enzyme (ACE) level was elevated 299 U/L; a right upper quadrant ultrasound revealed a mildly enlarged liver, subsequent CT chest and CT abdomen enlarged paratracheal and subcarinal adenopathy periportal edema in the liver and numerous small hypodense nodules in the spleen. Subsequent liver biopsy showed chronic, non-granulomatous, portal and parenchymal inflammation that was compatible with Autoimmune Hepatitis. Bronchoscopy with transbroncial biopsy revealed chronic, non-caseating granulomatous inflammation that was consistence with sarcoidosis. Thus the patient was diagnosed with sarcoidosis and AIH. The patient was started on Prednisone 60 mg daily, with subsequent reduction to 15 mg. The serum transaminase levels returned to normal level within three months. Conclusion: This case underscores the significance of association of Sarcoidosis that presented with Autoimmune Hepatitis. Physicians should be aware of this possibility so they may efficiently pick up the diagnosis after relevant investigations and start appropriate management. Timely intervention is key to treat the acute presentation and to prevent the further complications.