Obliterative Vasculitis Research Articles

Obliterative Vasculitis as the Presenting Sign of Systemic Lupus Erythematosus in a 12-Year-Old

Obliterative Vasculitis as the Presenting Sign of Systemic Lupus Erythematosus in a 12-Year-Old

A Comprehensive Narrative Review of the Impact of Pelvic Radiotherapy on Pelvic Bone Health: Pathophysiology, Early Diagnosis, and Prevention Strategies.

Radiotherapy is a commonly used modality in pelvic malignancies such as prostate, gastrointestinal, or gynecological, either as a primary treatment or an adjuvant post-surgery. Despite its positive impact on the prognosis of these patients, it was found in several studies that it contributes to insufficiency fractures in different sites of the pelvis, more commonly in the sacral ala. This is particularly true for elderly patients. There are several hypotheses on how radiotherapy affects bone health, as it destroys the bone matrix and causes obliterative vasculitis. Several imaging techniques, particularly magnetic resonance imaging (MRI), help detect the radiotherapy-induced fracture and distinguish it from metastases. Some modalities, such as intensity-modulated radiotherapy (IMRT) and brachytherapy, have decreased fracture risk by escaping the adjacent structures to the targeted organ. Pharmacological interventions such as amifostine and desferrioxamine are promising in terms of bone protection, which necessitates further studies to confirm their mechanism of action.

Bilateral Extensive Syphilitic Punctate Inner Retinitis Triggering Macular Ischemia after Intravitreal Triamcinolone Injections

Purpose: To report a case of extensive syphilitic punctate inner retinitis (SPIR) triggering bilateral macular ischemia after intravitreal triamcinolone injections, and the multimodal retinal imaging findings.Case summary: A 69-year-old male patient with nonproliferative diabetic retinopathy was transferred to our hospital because of bilateral visual deterioration (to counting fingers) after the first intravitreal triamcinolone injection. Fundus examination revealed numerous yellow punctate precipitates in the superficial retinae, retinal arteriolitis, and vitritis. The punctate lesions and surrounding retinal regions showed decreased vascular density on optical coherence tomography angiography, and focal hypofluorescence on fluorescein angiography. The patient was positive for all of the Venereal Disease Research Laboratory test, the fluorescent treponemal antibody-absorption test, and <i>Treponema pallidum</i> hemagglutination; we diagnosed bilateral SPIR. After treatment with aqueous crystalline penicillin solutions (24 million units per day for 14 days), the punctate lesions reduced but arteriolitis progressed to obliterative vasculitis. After 6 months, the bilateral SPIR and vitritis resolved, and the bilateral visual acuity improved to 20/100. However, inner retinal and macular ischemia persisted because of capillary nonperfusion attributable to obliterative vasculitis.Conclusions: Extensive SPIR can develop after an initial intravitreal steroid injection; the inner retinal ischemia and visual loss may persist after treatment because obliterative vasculitis develops. Therefore, patients scheduled for intravitreal steroid injections should be screened for syphilis infection.

Routine IgG4 staining in minor salivary gland biopsy in a cohort of Italian Caucasian patients suffering from xerostomia.

ObjectivesIgG4-related disease is a potentially systemic disease mimicking and overlapping with different autoimmune diseases, such as primary Sjögren’s syndrome (pSS). The involvement of salivary glands, previously called Mikulicz’s disease, has been reclassified as IgG4-related sialadenitis (SA). The aim of this study was to assess the prevalence of IgG4-SA in a cohort of Italian Caucasian patients presenting with xerostomia and to evaluate the eventual overlap between IgG4-SA and pSS.Material and methodsWe included 154 patients – 15 males and 139 females, mean age 54.18 ±14.24 years, who underwent minor salivary gland biopsy between March and December 2019 for xerostomia. Histopathology was evaluated using Chisholm-Mason (CM) and focus score (FS) for pSS and immunohistochemical study with IgG4 staining for IgG4-SA were performed. Serum autoantibodies (anti-SSa/RoAb, anti-SSB/LaAb, antinuclear antibodies, rheumatoid factor) were also assessed.ResultsIn 69 patients (44.8%) FS 0 was found, while FS ≥ 1 was presented in 85 (55.2%). Chisholm-Mason score < 3 and CM ≥ 3 was found in 73 (47.4%) and 81 (52.6%) cases, respectively. IgG4/high-power field level was 20 in 3 pSS patients (1.9%), but none of them had an IgG4/IgG ratio ≥ 40, as well as tissue fibrosis with storiform pattern, obliterative vasculitis, and tissue eosinophilia. The diagnosis of pSS, was confirmed in 92 patients (59.74%). No patient was definitively diagnosed with an IgG4-related disease.ConclusionsIn the case of xerostomia, the evaluation of the histopathological specimen for IgG4 should not be routinely performed, at least in an Italian-based Caucasian population. Moreover, immunohistochemistry should not be requested in the case of a negative result of biopsy for pSS.

P29 A case of paediatric ocular Behcet’s

Abstract Case report - Introduction Bechet’s is a rare chronic inflammatory multisystem disease of unknown cause. It is characterised by recurrent oral ulcerations, genital ulcerations, ocular inflammation and skin involvement. Bechet’s disease is rarely observed during childhood and the clinical picture can be incomplete, thus making the diagnosis challenging. Ocular involvement represents 10—20% of the initial presenting feature. Ocular involvement typically involves anterior and/or posterior uveitis and retinal vasculitis. Severe vision loss can be seen in patients. Prognosis is improving due to enhanced therapeutic options. The use of biologics, particularly anti-TNF drugs, are a positive advance, though more evidence in paediatrics is required. Case report - Case description This young man presented in 2014 at 12 years old to paediatric ophthalmology with a right panuveitis. He was treated with a weaning dose of oral steroids. His right eye became quiet. He reported some oral ulceration, not recurrent, and no genital ulcers. He did not fulfil Bechet’s criteria. Pathergy test and HLA B51 were negative. In 2017 he presented with right macular retinal infiltrate. Vision was reduced to counting fingers. Mycophenolate mofetil (MMF) was started with weaning prednisolone. Despite this, activity was ongoing with inflammation in the anterior chamber and vitreous. Humira was started in August 2017. There was resolution of the inflammation in the right eye but central vision remained poor due to irreversible macular damage. Left eye was normal. Oral steroids were weaned. In 2018, he developed acne and folliculitis type lesions. He reported sore joints and fatigue. He was felt to have probable Behcet’s. Quaternary review by the Bechet’s centre, Liverpool, agreed. A switch from MMF to azathoprine (AZA) was advised. Interferon alpha would have been suitable, but was no longer available. Skin lesions returned when switched to AZA; patient requested switch back to MMF. Over the next 2 years steroids were weaned. MMF and 2-weekly Humira continued. In November 2020 he presented with peripheral retinitis in his left, better-seeing eye, haemorrhaging, with patchy vision. MMF was increased and oral steroids recommenced. In December 2020 visual acuity was hand movements in his right eye and 6/7.5 in his left eye with ongoing inflammation. Adalimumab was increased to weekly. In January 21 there were 3 + of cells in his anterior chamber, extensive haemorrhages and retinal infiltrates in keeping with Behcet’s. He was admitted for pulsed IV methylprednisolone and switched from Humira to infliximab. Eyes are currently quiescent and prednisolone is weaning. Case report - Discussion Bechet’s disease is a multiorgan disease typified by an immune-mediated occlusive vasculitis. The aetiology of Bechet’s remains unclear. HLA-B51 is a major predisposing genetic factor. There is involvement of the innate immune system, propagated by the adaptive immune system. Ophthalmic involvement can be the presenting feature of Bechet’s. Eye disease in Bechet’s is most commonly a non-granulomatous uveitis with necrotising obliterative retinal vasculitis. This can be found in either the anterior or posterior segment or both. Recurrent inflammation in the posterior segment can lead to severe sight loss. Young males typically have the worst ocular prognosis. The treatment of ocular Bechet’s can be challenging as our case emphasises. Treatment follows a step-wise approach. Therapy consists of topical steroid, followed by intravenous or oral steroid. Maintenance therapy of either AZA 2mg/kg/day, MMF (max3 g daily) or ciclosporin max 5mg/kg/day should be commenced. The next step will typically be a TNF inhibitor; in our patient we used adalimumab initially. Infliximab is becoming a popular first choice anti-TNF. Certainly since January 2021 our patient’s eyes have remained quiescent on Infliximab. The alternative to infliximab is interferon alpha but unfortunately this is no longer commercially available. A further treatment that could be offered is rituximab – initially to be given as a one-off cycle. Optimum treatment of ocular Bechet’s involves good multidisciplinary team working between ophthalmology and rheumatology. By early initiation of systemic treatment and regular reviews secondary complications may be avoided/lessened. Case report - Key learning points

Coexistence of common gallstones and sinusoidal obstruction syndrome: Case report and review of the literature

Purpose: The sinusoidal obstruction syndrome (SOS) of the liver is an obliterative vasculitis that involves the terminal branches of the hepatic venules and sinusoids. When it is not treated, it will be a serious risk of mortality. Here, we aim to present our patient who has been associated with recurrent cholangitis attacks due to cholelithiasis and choledocholithiasis and is associated with sinusoidal obstruction syndrome with the literature. Description of the case: A 30 years old male patient had complaints of abdominal pain and nausea for a long time. The patient had a history of choledocholithiasis and cholangitis attacks. Although ERCP was performed and a stent was placed in the biliary tract, his jaundice did not disappear. Liver function tests were high. Tumor markers were negative. Methods: We could not make a definitive diagnosis with imaging methods and biopsy and we planned surgery. We performed segmental liver resection and biliary diversion in the surgery. Histopathological examination of the resected liver tissue was compatible with SOS. Conclusion: Many studies have been done on the etiology of SOS and different causes have been revealed. Accompanied with clinical findings, a definitive diagnosis is made with the exclusion of the presumptions considered. Surgery can be performed for both diagnostic and therapeutic purposes. If patients with elevated liver function tests and bilirubin have long-term abdominal pain, SOS should be bear in mind.

AN UNUSUAL CASE OF STEROID-RESISTANT IGG4-RD AFFECTING THE MAXILLARY ALVEOLAR PROCESS

<h3>Introduction</h3> Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune fibroinflammatory condition that is becoming increasingly recognized in the head and neck. I present a rare case of IgG4-RD originating in the alveolar process of the maxilla with orbital and maxillary sinus involvement that was unresponsive to traditional management. <h3>Case Presentation</h3> A 37-year-old Somalian woman presented with a 4-month history of progressive swelling of her right cheek and trismus. She had paresthesia and neuropathic pain affecting the V2 and V3 regions of the right trigeminal nerve. Testing of cranial nerve V revealed that she had decreased light-touch sensation over the site of swelling. Her hearing was reduced on the right side based on a whisper test. <h3>Investigations</h3> Ultrasound, magnetic resonance imaging, and computed tomography confirmed a mass centered on the right maxillary alveolus extending into the right buccal and masticator space, maxillary sinus, and orbit with involvement of the right infraorbital and greater palatine nerves. Histopathologic analysis revealed a storiform pattern of stromal fibrosis with a lymphoplasmacytic infiltrate and obliterative vasculitis. There was an increased number of IgG4⁺ plasma cells on immunostaining. <h3>Treatment</h3> She was started on corticosteroid but showed no response. Treatment with cyclophosphamide and rituximab provided improvement clinically and symptomatically. <h3>Discussion</h3> Clinically, IgG4-RD presents as a tumefactive, tissue-destructive lesion and often mimics malignant tumors, which can complicate diagnosis. IgG4-RD can be managed medically, and it is important to differentiate this disease from other pathologies in order to avoid invasive surgical management.

Pulmonary Manifestations of Plasma Cell Type Idiopathic Multicentric Castleman Disease: A Clinicopathological Study in Comparison with IgG4-Related Disease.

Plasma cell type idiopathic multicentric Castleman disease (PC-iMCD) occasionally manifests as parenchymal lung disease. This study aimed to elucidate the detailed clinicopathological features of lung lesions in PC-iMCD and compare the findings with those in immunoglobulin (Ig) G4-related disease (IgG4-RD), the most difficult differential diagnosis of PC-iMCD. We analyzed the clinicopathological findings and immunohistochemical expression patterns of interleukin-6 (IL-6) and Igs in lung specimens from 16 patients with PC-iMCD and 7 patients with IgG4-RD. Histologically, pulmonary PC-iMCD could not be differentiated from IgG4-RD based on lesion distribution patterns, the number of lymphoid follicles and obliterative vasculitis, or fibrosis types. The eosinophil count was higher in the IgG4-RD group than in the PC-iMCD group (p = 0.004). The IgG4/IgG-positive cell ratio was significantly higher in the IgG4-RD group (p < 0.001). The IgA-positive cell count and IL-6 expression intensity were higher in the PC-iMCD group than in the IgG4-RD group (p < 0.001). Based on these findings, we proposed a new diagnostic approach to differentiate lung lesions of PC-iMCD and IgG4-RD. Our approach can be utilized to stratify patients with suspected lung-dominant PC-iMCD to identify candidates for strong immunosuppressive treatment, including IL-6 blockade, at an early stage.

An Unusual Case of Steroid Resistant IgG4-RD Affecting the Maxillary Alveolar Process

Introduction Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune fibroinflammatory condition that is becoming increasingly recognized in the head and neck. I present a rare case of IgG4-RD originating in the alveolar process of the maxilla with orbital and maxillary sinus involvement that was unresponsive to traditional management. Case Presentation A 37-year-old Somalian woman presented with a 4-month history of progressive swelling of her right cheek and trismus. She had paresthesia and neuropathic pain affecting the V2 and V3 regions of the right trigeminal nerve. Testing of cranial nerve V revealed that she had decreased light-touch sensation over the site of swelling. Her hearing was reduced on the right side based on a whisper test. Investigations Ultrasound, magnetic resonance imaging, and computed tomography confirmed a mass centered on the right maxillary alveolus extending into the right buccal and masticator space, maxillary sinus, and orbit with involvement of the right infraorbital and greater palatine nerves. Histopathologic analysis revealed a storiform pattern of stromal fibrosis with a lymphoplasmacytic infiltrate and obliterative vasculitis. There was an increased number of IgG4+ plasma cells on immunostaining. Treatment She was started on corticosteroid but showed no response. Treatment with cyclophosphamide and rituximab provided improvement clinically and symptomatically. Discussion Clinically, IgG4-RD presents as a tumefactive, tissue-destructive lesion and often mimics malignant tumors, which can complicate diagnosis. IgG4-RD can be managed medically, and it is important to differentiate this disease from other pathologies in order to avoid invasive surgical management.

Rosai-Dorfman Disease of the Pancreas Shows Significant Histologic Overlap With IgG4-related Disease.

Rosai-Dorfman disease (RDD) is a rare entity characterized by proliferating S100-positive histiocytes. Originally described in lymph nodes, it can involve extranodal sites. Pancreatic involvement is rare, with <10 cases previously reported. Recent studies demonstrate a possible overlap between RDD and the more common IgG4-related disease (IRD), which could further complicate pathologic diagnosis. We describe distinct morphologic characteristics as well as overlapping histologic features of IRD in 5 cases of pancreatic RDD at our institution and compare these to a cohort of nonpancreatic extranodal RDD cases. All pancreatic cases were mass forming and had spindled patterns of elongated histiocytes with smaller areas of more classical appearing RDD; all cases had areas of storiform fibrosis and dense lymphoplasmacytic infiltrates with no increase in IgG4-positive plasma cells, and all cases had some degree of vasculitis (4 cases had obliterative vasculitis). Thirteen nonpancreatic extranodal RDD cases had dense lymphoplasmacytic infiltrates; most (85%) had some fibrosis with 46% showing storiform fibrosis, 85% had vasculitis with 31% demonstrating obliterative vasculitis and 2 cases had increased IgG4 staining. Extranodal (pancreatic and nonpancreatic) RDD often shows overlapping morphologic features with IRD, including lymphoplasmacytic inflammation, storiform fibrosis with elongated histiocytes and vasculitis. This can create a diagnostic challenge in the pancreas where IRD is more commonly encountered. Pathologists need to be aware that RDD can occur in the pancreas and should include RDD in the differential of any mass forming pancreatic lesion in which morphologic features of IRD are present.

Itʼs Not Recurrence?

Case presentation A 53-year-old man was evaluated for nausea, fatigue and progressive weakness. He had a history of a 20cm (figure A) stage IIIb gastric high-grade, predominantly epithelioid, GIST (figure B, note the epithelioid appearing GIST within the black arrows, the oxyntic mucosa within the blue arrows and the muscularis separating the two areas) extirpated by partial gastrectomy and Billroth II ˜18 months prior. A 3cm LUQ mass was found on surveillance imaging 12 months later while being treated with adjuvant tyrosine-kinase inhibitors. Repeat imaging demonstrated an enlarging 6.7x7.0cm mass within the abdominal mesentery anterior to the left kidney. He was referred for laparoscopic partial small bowel resection and excision of a 10 cm mesenteric mass at the level of the efferent jejunal limb, a 3cm nodule at the level of the proximal ileum and 6 smaller nodules within the small bowel mesentery. Histopathology of the resected masses demonstrated a vimentin positive, uniform, spindled cell lesion with minimal inflammation and a rare mitotic figure. The lack of inflammation, storiform spindled pattern, mucin and obliterative vasculitis, helped exclude inflammatory myofibroblastic tumor and IgG4 disease. CD117 (c-kit) and CD34 were negative, excluding residual or recurrent GIST. However, the spindled cell did demonstrate nuclear and cytoplasmic positivity for beta-catenin, supporting the diagnosis of intra-abdominal mesenteric fibromatosis (figure C). Discussion Mesenteric fibromatosis or desmoid tumor is group of rare benign tumors that can be locally aggressive but do not metastasize. They develop in ˜20% of patients with FAP but can appear spontaneously, with tissue trauma and radiation exposure as reported risk factors. To date, there are about 30 reported cases of mesenteric fibromatosis following GIST resection. Co-existence of mesenteric fibromatosis with GIST has been reported as well. Distinguishing mesenteric fibromatosis from recurrent GIST may be difficult given common similarities in radiologic appearance and c-kit expression. Accurate diagnosis can only be made after surgical removal and histopathologic examination. Unfortunately, surgery for mesenteric fibromatosis is not always curative and high rates of recurrence are common. Watchful waiting should be considered after resection, especially in patients with minimal symptoms.2701_A Figure 1. CT of the abdomen2701_B Figure 2. Epithelioid GIST, 10X2701_C Figure 3. Jejunal mesenteric mass, desmin stain, 20x

Biological therapies for the treatment of Behçet's disease-related uveitis beyond TNF-alpha blockade: a narrative review.

Intraocular inflammation is one of the more relevant complications of Behçet's disease (BD), which tends to respond poorly to different medications. The ocular histopathologic changes are basically identical to those occurring in other organs and consist in a necrotizing leukocytoclastic obliterative vasculitis, which is probably immune complex-mediated and affects both arteries and veins of all sizes. There are growing evidences showing the potential role of biologic agents other than anti-tumor necrosis factor (TNF)-α agents in the management of ocular-BD, which have been collected in this review, including interleukin-1 and interleukin-6 blockade, secukinumab, ustekinumab, daclizumab, rituximab, and alemtuzumab. Further large studies are needed to fully elucidate and establish the clinical efficacy of these different tools in the refractory ocular manifestations of BD.

CALCB splice region pathogenic variants leading to plasma cell neurotropic enrichment in type 1 autoimmune pancreatitis

Recently, we have demonstrated that PRSS1 mutations cause ectopic trypsinogen activation and thereby result in type 1 autoimmune pancreatitis (AIP). However, the molecules involved in inducing obliterative vasculitis and perineural inflammation in the pancreas are not well-described. The present study applied whole-exome sequencing (WES) to determine the underlying etiology and revealed novel missense splice region variants, CALCB c.88T>C (p.Ser30Pro) and IR [1]-mutants, in 2 of the 3 families and 2 of 26 unrelated patients with type 1 AIP. In vitro, both of the mutants displayed decreased βCGRP, ERK1/2 phosphorylation, and co-localized with endoplasmic reticulum and Golgi apparatus. The novel pathogenic variant identified in this case should contribute to our understanding of the expanding spectrum of AIP.

Retinal Ganglion Cell Layer Thinning Followed By Retinal Nerve Fiber Layer Thinning In Patients with Uveitis

In the model of experimentally induced ischemia- reperfusion injury, retinal ganglion cells (RGC) expressing the gene AP-1 result apoptosis. The inflammation mediators, such as TNF-α, IL-1β, etc. lead RGC to apoptosis, that may lead the thinning of the retinal ganglion cell layer (RGCL) followed by the optic nerve fiber layer (RNFL) thinning. In his study we observed retinal ganglion cell and optic nerve fiber layer thinning in patients with various uveitis, that the pathological features appear obliterative vasculitis, using the optical coherence tomography (OCT) imaging analyses. Subjects were 182 eyes of 91 uveitis patients without glaucoma. Comparison were patients with normal tension glaucoma (NTG). Image analyses were conducted with 3D OCT-2000. As a result average RGCL thickness values in the patients with uveitis were significantly(p&lt;0.01) thinner than those in healthies. Cycle scan findings of RNFL around the optic disc in the patients with uveitis showed significant thinning especially at nasal side. The retinal ganglion cell layer thinning followed by the retinal nerve fiber thinning in the patients with various uveitis was observed, and the thinning was similar to that in patients with glaucoma. The observation of RGCL and RNFL thickness may be useful for the diagnosis and the follow-up of uveitis.

Association Between Three SNPs and Thromboangiitis Obliterans in Xinjiang Uyghur Population

Thromboangiitis obliterans (TAO), also called Buerger's disease, is a chronic peripheral vascular occlusive disease. It is an obliterative vasculitis characterized by arterial thrombosis and strongly associated with tobacco exposure. The pathogenesis and etiology of TAO are not well understood, but genetic factors may be important in its development. A case-control study was undertaken to identify genetic factors potentially involved in the pathogenesis of TAO in a Xinjiang Uyghur population of China, where TAO is common. We ascertained 177 TAO patients by clinical screening and 86 healthy individuals from the HAPMAP database. The genotypes of single-nucleotide polymorphisms (SNPs) of the participants were identified using the Affymetrix Genome-Wide Human SNP Array 6.0 to perform a genome wide association study (GWAS). The association between the SNPs and incidence of TAO was quantified using race stratification exposure. Through a case-control GWAS study 26 SNPs were significantly associated with incidence of TAO following a Bonferroni correction. However, after genomic control correction for population stratification only three of these SNPS were highly significantly associated with TAO: rs376511 in IL17RC (OR = 24.4, 95% CI:8.68 - 68.62, p < 0.0001), rs7632505 in SEMA5B (OR = 29.47, 95% CI:7.16 - 121.3, p < 0.0001), and rs10178082 (OR = 18.09, 95% CI: 6.56 - 49.92, p < 0.0001) showed a significant risk of TAO in the Uyghur population. This study shows an association between these 3 SNPs and susceptibility to TAO in the Uyghur population, suggesting that polymorphisms in the IL-17RC and Sema 5B genes may pre-dispose individuals in this population to development of TAO. These findings require replication.

Antibodies to Type IV Collagen Induce Type 1 Autoimmune Pancreatitis.

Type 1 autoimmune pancreatitis (AIP) is prototypic autoantibody-mediated diseases. Sclerosis accompanied by fiber deposition is generally regarded as the primary lesion in the development of obliterative vasculitis. However, why collagens or their antibodies play a crucial role in the pathogenesis of AIP has not been demonstrated. This study was performed to investigate if anti-collagen type IV antibodies (ACIVAbs) are the key factor of fiber deposition and recruit leukocytes, resulting in obliterative vasculitis in pancreas. Enzyme-linked immunosorbent analyses (ELISA) were used to measure the expression of Col IV and ACIVAbs in serum of patients with and without AIP. In vitro, adhesion and proliferation were determined by human lymphocytes incubated with Col IV and ACIVAbs. In vivo, C57BL0/6 mice were immunized with IgG-ACIVAbs, followed by analysis of clinical phenotype. IgG-ACIVAbs were recognized by the serum specimens from 12 of 22 patients with type 1 AIP, 3 of 9 patients with Crohn's disease, and 2 of 18 patients with pancreatic cancer, but not in healthy controls and acute pancreatitis. In patient's biopsy, ACIVAb staining increased and co-localized with subepithelial IgG4 deposits along the capillary walls and surrounding nerve fibers. In vitro, recombinant IgG-ACIVAbs increased leukocyte adhesion and proliferation. What is more, AIP could be induced in mice by immunization with IgG-ACIVAbs into adult mice.

Inflammatory Fibroid Polyps of Gastrointestinal Tract Rarely Show Increased IgG4 Expression

Inflammatory Fibroid Polyp (IFP) of gastrointestinal tract is characterized by concentric perivascular fibrosis and a mixed inflammatory infiltrate rich in eosinophils and also few plasma cells. IgG4-related sclerosing diseases (IgG4-RSD) are a heterogenous group of disorders described in many organs, characterized by a significant increased of IgG4+ plasma cells in a context of storiform fibrosis, obliterative vasculitis and mixed inflammatory infiltrate containing eosinophils. The histological similarities between IFP and IgG4-RSD prompted the present study in the attempt to find a possible link between IgG4 over-expression and IFP. The expression of IgG4 and IgG was evaluated in a series of 23 cases of IFP belonging to 23 patients. All cases were reviewed by two pathologists and the histological diagnosis confirmed. Immunohistochemistry for antibodies anti-IgG, anti-IgG4, and anti-IgA was performed on all cases and the results were evaluated by two observers. One case of IFP out of 23 (4.3%) fulfilled the IgG4-RSD histological criteria. The case did not differ histologically from the others. The patient did not present either a raised serological level of IgG4, nor other sign of IgG4-RSD. IgG4 increased expression can be rarely observed in IFP. Steroid therapy in cases of IFP with abundant IgG4+ plasma cells, especially in patients with multiple tumors, could be considered as an alternative to surgical treatment.

Nodular Nongranulomatous Vasculitis of the Lung Detected by 18F-FDG PET/CT

We report a case of a nodular nongranulomatous vasculitis of the lung detected by (18)F-FDG PET/CT. A 51-year-old woman with fever of unknown origin underwent (18)F-FDG PET/CT, which showed multiple and bilateral pulmonary nodules at CT scan with increased radiopharmaceutical uptake in the largest one (20 mm in diameter). On the basis of the PET/CT findings, the patient was referred for excisional biopsy of the largest pulmonary nodules suspicious for neoplastic disease. Histological examination demonstrated the presence of an obliterative vasculitis with interstitial pulmonary perivascular inflammation.

Central Retinal Artery Occlusion and Recurrent Papillitis in a Patient With Incomplete Behçet Disease

A 40-year-old man presented with painless sudden visual loss due to a central retinal artery occlusion (CRAO) in his right eye. Three months later, he had painless acute visual loss in his left eye with visual acuity of 20/100 and a swollen optic disc. After oral prednisone treatment, the disc swelling resolved and acuity recovered to 20/20. Five months later, the patient experienced another episode of vision loss in the left eye associated with optic disc edema. With steroid therapy, he regained 20/20 acuity once again. With a history of recurrent oral ulcers, fluorescein angiography showing obliterative retinal vasculitis in the right eye, and steroid responsive optic neuropathy in the left eye, we made the diagnosis of incomplete Behçet disease.

Current trends in the management of ocular symptoms in Adamantiades-Beh&amp;ccedil;et&amp;rsquo;s disease

Adamantiades-Behçet's disease (ABD) is a multisystemic vasculitic disease. It is most prevalent in the Eastern Mediterranean countries and the Eastern region of Asia. Its effect on the eye can range from mild to debilitating, resulting in total blindness. A necrotizing and obliterative vasculitis affects both arteries and veins of organs. Recurrent attacks of uveitis, oral aphthous ulcers, skin lesions, and genital ulcers are common. Topical and systemic corticosteroids have been the mainstay in the treatment of ocular inflammation for many years; however, due to the several known side effects of corticosteroids and thanks to scientific advances, more novel approaches to ABD treatment have been emerging. Antimetabolites such as methotrexate and azathioprine have been utilized with the latter showing positive results. Chlorambucil has been utilized effectively for ocular manifestations of ABD. Interferon alpha has shown encouraging results in the management of refractory ocular inflammation associated with ABD, either alone or in combination with other immunosuppressive agents. Surgical interventions to deal with complications from ABD can be safely done if adequate control of inflammation is achieved peri-operatively. Early detection and aggressive treatment, when needed, have proven to be essential in the management of this relentlessly explosive disease.