scholarly journals The protean manifestations of central nervous system IgG4-related hypertrophic pachymeningitis: a report of two cases

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Peter Y. M. Woo ◽  
Ben C. F. Ng ◽  
June H. M. Wong ◽  
Oliver K. S. Ng ◽  
Timothy S. K. Chan ◽  
...  
Keyword(s):  
Autoimmune Disease ◽  
Tuberculous Meningitis ◽  
Immunosuppressive Treatment ◽  
Glucocorticoid Therapy ◽  
Low Grade ◽  
Hypertrophic Pachymeningitis ◽  
Gadolinium Contrast ◽  
Obliterative Phlebitis ◽  
Serum Igg4 ◽  
History Of

Abstract Background IgG4-related hypertrophic pachymeningitis is a relative newly recognized and rare manifestation of IgG4-related disease, an immune-mediated fibroinflammatory tumefactive disorder. Fewer than 80 patients have been reported in the literature, and it can mimic common neurosurgical conditions. We describe the clinical presentation of two patients that were initially considered to have a subdural collection, tuberculous meningitis, and a cervical spinal meningioma, but were eventually diagnosed with this disease. Case presentation Two ethnic Chinese men, 86 and 62 years old, experienced a 4-week history of headache. Both patients had a history of autoimmune disease, namely glomerulonephritis and Grave’s disease, respectively. Magnetic resonance brain imaging revealed diffuse dural thickening with the latter patient exhibiting homogeneous and intense gadolinium-contrast enhancement. Since the 86-year-old patient also had progressive bilateral visual loss, giant cell arteritis was suspected and a 2-week course of glucocorticoid therapy was prescribed, but his symptoms failed to improve. The 62-year-old patient also had accompanying low-grade fever and was treated empirically as having tuberculous meningitis although there were no confirmatory microbiological findings. This patient further developed right hemiparesis, and additional imaging revealed a C4/5 intradural-extramedullary contrast-enhancing lesion resembling a meningioma causing cord compression. Both patients underwent neurosurgical intervention with the former undergoing a dural biopsy and the latter having the cervical lesion resected. The final diagnosis was IgG4-related hypertrophic pachymeningitis with the hallmark histological features of lymphoplasmacytic infiltration of IgG4+ plasma cells, storiform fibrosis, and obliterative phlebitis. In addition, their serum IgG4 levels were elevated (i.e., > 135 mg/dL). Both patients received at least 6 months of glucocorticoid therapy while the latter also had azathioprine. Their symptoms improved significantly and recurrent lesions were not detected on follow-up imaging. Conclusions A high index of suspicion for this condition is suggested when a male patient with a history of autoimmune disease and compatible radiological findings, experiences subacute headache that is disproportionate to the degree of dural involvement. Neurosurgeons should consider early meningeal biopsy to establish a definitive histological diagnosis in order for early effective immunosuppressive treatment to be initiated and to avoid unnecessary morbidity.

scholarly journals Chronic sclerosing sialadenitis IgG4-related in a boy with recurrent parotitis: a case report

2021 ◽  
Author(s):  
Fabio Stefano Timeus ◽  
Mario Michele Calvo ◽  
Anna Maria Caci ◽  
Giogio Oliviero Gallone ◽  
Federico Vittone
Keyword(s):  
Plasma Cells ◽  
White Blood Cells ◽  
Anaerobic Bacteria ◽  
Final Diagnosis ◽  
Low Grade ◽  
Obliterative Phlebitis ◽  
Cervical Lymph Nodes ◽  
Chronic Sclerosing Sialadenitis ◽  
Igg4 Related Disease ◽  
Serum Igg4

Abstract BackgroundIgG4-related diseases are a group of immune-mediated diseases characterized by a lymphoplasmacytic infiltrate with a prevalence of IgG4-positive plasma cells, storiform fibrosis, obliterative phlebitis. Autoimmune pancreatitis, sialadenitis, dacryoadenitis and retroperitoneal fibrosis are the most frequent manifestations. IgG4-related sialadenitis usually affects submandibular glands and is very rare in children. We report here a case of an unexpected diagnosis of IgG4-related sialoadenitis in a boy previously diagnosed as affected by juvenile recurrent parotitis.Case presentationA six-year old boy presented to our centre with left parotid swelling about 4x3 cm, tender, soft in consistency, with the overlying skin red and warm. He had low-grade fever and a mild enlargement of left cervical lymph nodes. In the last two years he had five episodes of parotitis and a diagnosis of juvenile recurrent parotitis.. The general conditions were good, and the remainder of the physical examination was normal. At the ultrasonography left parotid was enlarged, inhomogeneous, with a colliquated intraparotid lymph node and no evidence of sialolithiasis. Laboratory tests were normal, except for an increase of white blood cells and positivity for IgM and IgG anti-VCA, with anti-EBNA e anti-EA I negative. The patient was treated with cephalosporins and azitromycin, but after 10 days the parotid became fluctuating and the patient underwent to drainage and biopsy of the gland. He was discharged after 3 days of observation, without any complications, and treated with a further course of cefpodoxime and betametasone, with a good clinical response.Investigations for aerobic and anaerobic bacteria, mycobacteria and Bartonella on the pus were negative. Histology showed marked fibrosis and histiocytic and lymphoplasmacellular infiltration. The plasma cells were polyclonal and mostly expressed IgG4 subclass immunoglobulins. The final diagnosis was chronic sclerosing sialadenitis IgG4-related in recurrent parotitis and recent EBV infection. ConclusionsThe interest of this case comes from the rarity of IgG4-related sialadenitis in children. It is not possible to argue with certainty that the previous episodes of parotitis were manifestations of a IgG4-related disease, due to the lack of histological data. Serum IgG4 in our patient were normal, as described in literature in 30% of cases.

Primary acquired melanosis (PAM) without atypia/WHO low-grade conjunctival melanocytic intraepithelial lesion over areas of oculodermal melanocytosis

2020 ◽  
Vol 13 (10) ◽  
pp. e236741
Author(s):  
Bashar M Bata ◽  
Sachin M Salvi ◽  
Hardeep Singh Mudhar
Keyword(s):  
Bulbar Conjunctiva ◽  
Low Grade ◽  
Primary Acquired Melanosis ◽  
Conjunctival Biopsy ◽  
History Of ◽  
Intraepithelial Lesion ◽  
The Relationship ◽  
Oculodermal Melanocytosis ◽  
Substantia Propria ◽  
New Onset

An elderly white man with a history of left oculodermal melanocytosis presented with new onset brown pigmentation of the left bulbar and inferior tarsal conjunctiva. The bulbar conjunctival pigmentation was at the level of the conjunctival epithelium and was overlying areas of typical slate-grey scleral pigmentation characteristic of oculodermal melanocytosis. Both areas of new pigmentation were biopsied. The bulbar conjunctiva revealed primary acquired melanosis (PAM) without atypia with increased melanin production and the tarsal conjunctival biopsy showed PAM without atypia sine pigmentio overlying areas of substantia propria spindle-shaped heavily pigmented melanocytes of oculodermal melanocytosis. The case report examines the relationship between the epithelial and substantia propria melanocytes and correlates the findings with what is known about this association from the dermatopathology literature.

scholarly journals IgG4-related gastric disease with plasma cell-rich obliterative arteritis accompanied by early-stage gastric cancer: a case report

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Masayoshi Obatake ◽  
Koichi Sato ◽  
Shigehiko Yagi ◽  
Hiromi Ohtani ◽  
Katsumi Kito
Keyword(s):  
Gastric Cancer ◽  
Plasma Cell ◽  
Early Stage ◽  
Vascular Lesions ◽  
Gastric Disease ◽  
Inflammatory Disorder ◽  
Obliterative Phlebitis ◽  
Regional Lymph Node Dissection ◽  
Serum Igg4 ◽  
Storiform Fibrosis

Abstract Background Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated inflammatory disorder that can involve multiple organs. It is characterized by IgG4-positive plasma cell-rich storiform fibrosis and obliterative phlebitis associated with a high serum IgG4 level. There are few reports of gastric IgG4-RD, especially those detected prior to systemic or other organ involvement. Case presentation: A 70-year-old man was diagnosed with type 0–IIc gastric cancer at the anterior wall of the gastric corpus by upper gastrointestinal endoscopy. In addition, a submucosal tumor (SMT) 7 mm in diameter was found at the greater curvature of the angulus. Laparoscopic distal gastrectomy with regional lymph node dissection was performed. Pathology revealed a poorly differentiated adenocarcinoma in the type 0–IIc lesion and storiform fibrosis with infiltration of a large number of IgG4-positive plasma cells in the SMT. Postoperative laboratory testing showed elevation of serum IgG4 levels; thus, we diagnosed the SMT as IgG4-RD. Intriguingly, the gastric IgG4-RD lesion demonstrated IgG4-positive plasma cell-rich arteritis as well as typical obstructive phlebitis. The patient has been followed for 2 years after surgery without recurrence of cancer, but skin lesions of IgG4-RD have appeared. Conclusion We report a rare case of IgG4-RD presenting as a gastric SMT, accompanied by early-stage gastric cancer. Our case may support a newly proposed relationship between IgG4-RD and malignancies. The gastric IgG4-RD lesion showed arteritis as well as obliterative phlebitis, potentially providing novel insight into IgG4-related vascular lesions.

scholarly journals Concomitant autoimmunity may be a predictor of more severe stages of endometriosis

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Vanni Valeria Stella ◽  
Villanacci Roberta ◽  
Salmeri Noemi ◽  
Papaleo Enrico ◽  
Delprato Diana ◽  
...  
Keyword(s):  
Autoimmune Disease ◽  
Adaptive Immune System ◽  
Stage Iv ◽  
Multivariate Logistic Regression Model ◽  
Medical Patients ◽  
Laparoscopic Procedures ◽  
Logistic Analysis ◽  
Increased Risk ◽  
History Of

AbstractPathogenesis of endometriosis is still unclear and a role of both innate and adaptive immune system has been postulated. Some recent findings have revealed an increased risk to have concomitant autoimmune disease in women with endometriosis, but no study so far has investigated whether this association could affect endometriosis severity and stage. We retrospectively reviewed medical patients’ notes of women with a confirmed diagnosis of endometriosis who referred to our endometriosis outpatient clinic between January 2015 and December 2019. Cases (endometriosis and an autoimmune disease) were matched in a 1:3 ratio by age and study period with controls (endometriosis without history of autoimmunity). At univariate logistic analysis, concomitant autoimmunity (OR 2.63, 95% CI 1.64–4.21, p < 0.001) and the number of laparoscopic procedures performed (OR 2.81, 95% CI 1.45–5.43, p = 0.002) emerged as factors significantly associated with the likelihood of stage IV endometriosis. In the multivariate logistic regression model, concomitant autoimmunity remained a significant predictor of stage IV endometriosis (OR 2.54, 95% CI 1.57–4.10, p = 0.004), whereas the association between the number of laparoscopic procedures performed and stage IV endometriosis was found to be of borderline-significance (OR 2.70, 95% 1.37–5.30, p = 0.050). Our findings suggest that endometriosis is more severe in patients who are also affected by autoimmune disturbances after controlling for relevant confounders.

scholarly journals P54 Development of ocular toxoplasmosis and panuveitis in a patient receiving infliximab for psoriatic arthritis

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Mariam Malik ◽  
Himashi Anver ◽  
Ernest Wong
Keyword(s):  
Psoriatic Arthritis ◽  
Past History ◽  
Acute Infection ◽  
Immunosuppressive Treatment ◽  
Ocular Toxoplasmosis ◽  
Close Monitoring ◽  
Horse Riding ◽  
Increased Risk ◽  
History Of ◽  
Acute Toxoplasmosis

Abstract Background Toxoplasma gondii is thought to infect up to a third of world’s population. Incidence rate of 0.4/100,000 has been calculated in Britain, culminating in a life-time risk of 18/100,000. Cats are primary hosts, but humans and warm-blooded animals can be infected by consumption of contaminated food/water. Although in most patients, it’s self-limiting, it can be devastating in immunosuppressed patients and may cause eye manifestations, cerebral abscesses or disseminated infection. Immunosuppressive therapies including treatment with biologics increases the risk and may also cause toxoplasmosis reactivation. Methods This is case of 57 year old lady with psoriatic arthritis. She has past history of congenital vision impairment in the left eye and is HLA B27 negative. She enjoyed horse-riding and had a pet dog. Initially she was started on methotrexate. Sulfasalazine was added later. Due to ongoing active disease, etanercept was used for 6 months, before being switched to cetrolizumab due to ineffectiveness. She had this for 5 months and then switched to infliximab, 3mg/kg, 8 weekly. In May 2019, she was seen by Ophthalmology for 2 weeks history of blurred vision and floaters in right eye. She was diagnosed to have panuveitis and had positive IgM for toxoplasma. Bloods revealed negative TB screen, HIV, Hep B&C, syphilis, lyme and anti-streptolysin antibody tests were negative. Infliximab levels were sub-therapeutic. She was commenced on 30mg prednisolone for possible inflammatory process secondary to seronegative arthropathy, but acute toxoplasmosis could not be excluded. Hence, she was started on azithromycin and had vitreous biopsy. Toxoplasma was detected in the sample, confirming acute infection. Methotrexate and infliximab were stopped. MRI head ruled out intracranial involvement. Following treatment of acute toxoplasmosis, adalimumab is now being considered for management of her inflammatory disease, with close monitoring by local infectious-disease team and specialist ophthalmology unit. Results This lady developed ocular toxoplasmosis and panuveitis, whilst on immunosuppression for psoriatic arthritis. She was a horse-rider and had exposure to dogs. Diagnosing toxoplasma in immunocompromised can be difficult. Isolation of T. gondii in tissue usually confirms diagnosis. Some forms of immunosuppressive treatment may be associated with increased risk of reactivation of toxoplasmosis but there is not much evidence to assess the relative risk of various therapies. Conclusion Ocular toxoplasmosis needs to be considered in patients receiving immunosupression and presenting with inflammatory eye symptoms. Management requires specialist input and close monitoring. Further research into diagnostic techniques, possibility of using prophylaxis in high-risk patients and management guidelines would be helpful. Disclosures M. Malik None. H. Anver None. E. Wong None.

Solitary Paraganglioma of the Hypoglossal Nerve: Case Report

Neurosurgery ◽  
2011 ◽  
Vol 68 (4) ◽  
pp. E1170-E1174 ◽  
Author(s):  
Kazim Raza ◽  
Chandrasekaran Kaliaperumal ◽  
Michael Farrell ◽  
John A. O'Dwyer ◽  
Christopher Pidgeon
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Hypoglossal Nerve ◽  
Jugular Foramen ◽  
Low Grade ◽  
Resonance Imaging ◽  
Stereotactic Radiation ◽  
Hypoglossal Canal ◽  
Suboccipital Craniotomy ◽  
History Of

Abstract BACKGROUND AND IMPORTANCE: We report the case history of solitary hypoglossal paraganglioma in a 64-year-old woman. The surgical difficulties encountered in the removal of this challenging tumor are discussed and as a literature review provided. CLINICAL PRESENTATION: A 64-year-old woman presented with a short history of dysphonia, occasional dysphagia, tinnitus, altered taste, and unilateral left-sided tongue wasting. On examination, there was left lower motor hypoglossal paralysis. Imaging showed a discrete enhancing lobulated mass, measuring 2 × 2 cm, in the region of the hypoglossal nerve extending into the hypoglossal canal suggestive of hypoglossal paraganglioma. A left dorsolateral suboccipital craniotomy was performed with the patient in the sitting position. The hypoglossal nerve appeared to be enlarged, and the jugular foramen was normal. Complete surgical debulking of the tumor was not attempted because of its vascular nature. The nerve was decompressed, and neuropathology confirmed a low-grade paraganglioma arising from the hypoglossal nerve. The patient was scheduled to receive stereotactic radiation for further management. CONCLUSION: When a case of solitary hypoglossal paraganglioma is encountered in clinical practice, the aim of management should be mainly focused on achieving a diagnosis and preserving the hypoglossal nerve function. If there is evidence of vascularity in the lesion noted on magnetic resonance imaging, a preoperative angiogram should be obtained with a view for embolization. We decompressed the hypoglossal canal and achieved good improvement in the patient's symptoms. We recommend stereotactic radiosurgery for remnant and small hypoglossal tumors and regular follow-up with magnetic resonance imaging scans.

Pressure, temperature, and composition history of syntectonic fluids in a low-grade metamorphic terrane

Geology ◽  
2005 ◽  
Vol 33 (5) ◽  
pp. 421 ◽  
Author(s):  
Arthur Goldstein ◽  
Bruce Selleck ◽  
John W. Valley
Keyword(s):  
Low Grade ◽  
History Of ◽  
Metamorphic Terrane ◽  
Composition History

scholarly journals Atypical Presentation of Atypical Teratoid Rhabdoid Tumor in a Child

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Y. T. Udaka ◽  
K. Shayan ◽  
N. A. Chuang ◽  
J. R. Crawford
Keyword(s):  
Rhabdoid Tumor ◽  
Low Grade Glioma ◽  
Low Grade ◽  
Atypical Presentation ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Resonance Imaging ◽  
Atypical Teratoid ◽  
History Of ◽  
Progressive Weight Loss ◽  
Intracranial Neoplasm

Atypical Teratoid Rhabdoid Tumor (ATRT) is a rare malignant intracranial neoplasm more commonly diagnosed in young children. The authors report the case of an 11-year-old boy with a long standing history of slowly progressive weight loss, fatigue, and weakness over 1.5 years whose magnetic resonance imaging revealed a large heterogeneous enhancing dorsally exophytic lower brainstem mass. Examination revealed extreme cachexia, gaze-evoked nystagmus, dysphagia, dysarthria, bilateral dysmetria, and global weakness without ambulation. The protracted history and neuroimaging features were most suggestive of a low grade glioma. However, pathology revealed a hypercellular tumor with large hyperchromatic nucleoli and loss of INI-1 staining on immunohistochemistry consistent with a diagnosis of an ATRT. The child died shortly after surgery due to complications from his brainstem infiltrative disease. This case illustrates the diverse presentation of ATRT in childhood that can clinically and radiographically mimic that of low grade glioma.

Dynamic history of low-grade gliomas before and after temozolomide treatment

2007 ◽  
Vol 61 (5) ◽  
pp. 484-490 ◽  
Author(s):  
Damien Ricard ◽  
Gentian Kaloshi ◽  
Alexandra Amiel-Benouaich ◽  
Julie Lejeune ◽  
Yannick Marie ◽  
...  
Keyword(s):  
Low Grade ◽  
Low Grade Gliomas ◽  
Before And After ◽  
History Of

scholarly journals P045 Nothing is too 'cagey' for the rheumatologist

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Muhammad F Kazmi
Keyword(s):  
Weight Loss ◽  
Inflammatory Markers ◽  
Low Grade ◽  
Careful Monitoring ◽  
Chronic Infections ◽  
Asian Origin ◽  
Travel History ◽  
Chest Clinic ◽  
History Of ◽  
Inflammatory Changes

Abstract Background/Aims  Rheumatological conditions can present with a number of non-specific features like arthralgia, fever, fatigue, weight loss along with raised inflammatory markers and positive antibodies. Due to this, when similar symptoms are referred for input it is very important to consider other ‘mimics’. We report a case of Pigeon fancier’s lung presenting with these symptoms which was referred as likely connective tissue disease. Methods  A 52-year-old lady of South Asian origin was referred by her GP with six month history of 3kg weight loss, arthralgia, fatigue, low grade fever and persistently raised inflammatory markers (ESR ranging from 50-64 mm/hr, CRP 10-14 mg/L, normal BMI). On further questioning there was history of mouth ulcers, non-specific rash, occasional cough but no Raynaud’s or joint swelling. Blood investigations showed weakly positive ANA and RF but negative ENA, DNA, antiCCP , CK, C3,C4. C-ANCA was positive but PR3 negative. CXR was clear and tests for chronic infections including TB were negative. Due to lack of objective CTD signs, plan was to take a careful monitoring approach to see if clinical features evolved. A month later due to worsening cough, a CT chest/abdomen arranged by GP showed ground-glass changes consistent with pneumonitis and hence her rheumatology appointment was expedited to see if there was an autoimmune unifying diagnosis. She was also referred by her GP to the chest clinic in view of CT report and mild shortness of breath. Results  On further review, again there were no objective CTD signs. On direct questioning there was history of travelling before worsening chest symptoms to South Asia. Also around a year before her symptoms started she was given an African grey parrot. Based on this, serology for Avian precipitin was checked which showed strongly positive IgG antibodies to avian antigens (Budgerigar droppings and feathers, Pigeon feathers IgG Abs) confirming the diagnosis of pigeon fanciers lung. She fulfilled the diagnostic criteria and was asked to avoid the trigger. Urgent respiratory input was arranged where diagnosis was agreed with and disease was deemed sub-acute in presentation. Due to PFTs showing low transfer factor of 38%, Prednisolone was started with significant improvement within few days. Review of CT chest only showed inflammatory changes and no established fibrosis predicting excellent prognosis as delay in treatment can cause irreversible pulmonary fibrosis. Conclusion  A number of conditions can mimic rheumatological conditions which usually turn out to be either infectious or malignant in origin. This case highlights the importance of considering other differentials and along with taking a travel history also asking for other possible triggers like pets. In similar scenarios the diagnosis may be ‘cagey’ but as rheumatologists we are expected to answers questions which others can’t. Disclosure  M.F. Kazmi: None.

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