Simultaneous presentation of IgG4-related chronic peri-aortitis and coeliac disease in a patient with Marfan's Syndrome Title: Simultaneous presentation of IgG4-related chronic peri-aortitis and coeliac disease in a patient with Marfan's Syndrome Authors [spiral.imperial.ac.uk]

Storiform fibrosis was also present. Obliterative phlebitis, depicted in this figure, was shown in a vein running adjacent to an artery (top of figure). [em-consulte.com]

To review the prevalence, mechanisms, presentations and clinical significance of aortic involvement in rheumatic inflammatory diseases. Methods. [scinapse.io]

The case presented was that of a 53-year-old woman who developed aortitis with no past history of inflammatory disease, presenting with severe aortic regurgitation. [heart.bmj.com]

She had received 1 month of oral prednisone (dose unclear) 4 months prior to presentation. Her vital signs were unremarkable, including symmetric blood pressures in all 4 limbs. [jrheum.org]

• Mediastinal Lymphadenopathy

  Four years before the diagnosis of his aortic dissection, the patient had been found to have mediastinal lymphadenopathy, with lymph nodes up to 3 cm in diameter. The patient had undergone biopsy of a subcarinal lymph node via mediastinoscopy. [docplayer.net]

• HELLP Syndrome

  The pregnancy wascomplicated by diabetes mellitus, fetal cardiac abnormality, and HELLP syndrome (hemolysis, elevated liver enzymes, and lowplatelets), necessitating emergency delivery at 34 weeks. [vdocuments.mx]

  The pregnancy was complicated by diabetes mellitus, fetal cardiac abnormality, and HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets), necessitating emergency delivery at 34 weeks. [docplayer.net]

• Inguinal Mass

  Khoan Vu, Richa Gupta, John Frater, John Atkinson and Prabha Ranganathan, A 55‐Year‐Old Man With Periorbital and Inguinal Masses, Pericarditis, and Pleuritis, Arthritis Care & Research, 69, 5, (730-736), (2017). [doi.org]

• Respiratory Disorders

  It is now widely believed that IgG4-RD characteristically manifests as Mikulicz’s disease [ 4 ], respiratory disorders [ 5 ], sclerosing cholangitis [ 6 ], retroperitoneal fibrosis [ 3 ], tubulointerstitial nephritis [ 7 ], and prostatitis [ 8 ]. [ncbi.nlm.nih.gov]

• Anosmia

  In this article, we report two patients of IgG4 syndrome: a 60-year-old male with complaints of nasal congestion, rhinitis, anosmia and hearing loss, and a 28-year-old female with bilateral orbital and parotid gland swelling. [scinapse.io]

• Nasal Congestion

  In this article, we report two patients of IgG4 syndrome: a 60-year-old male with complaints of nasal congestion, rhinitis, anosmia and hearing loss, and a 28-year-old female with bilateral orbital and parotid gland swelling. [scinapse.io]

• Bruxism

  […] headaches; The neck and headaches; Drug-induced headaches; Trigeminal neuralgia and glossopharyngeal neuralgia; Headaches and vasculitis; Tonsilar ectopia and headaches; Post-traumatic headaches; Metabolic headaches; Temporomandibular joint disorders, bruxism [books.google.com]

• Irregular Heart Rhythm

  He denied having head-aches, vision changes, jaw claudication, rash, weakness, orsymptoms of polymyalgia rheumatica.Physical examination on the sixth postoperativeday revealed an irregularly irregular heart rhythm. [vdocuments.mx]

  Physical examination on the sixth postoperative day revealed an irregularly irregular heart rhythm. The rate ranged from 80 to 123 beats per minute. [docplayer.net]

• Irregularly Irregular Heart Rhythm

  He denied having head-aches, vision changes, jaw claudication, rash, weakness, orsymptoms of polymyalgia rheumatica.Physical examination on the sixth postoperativeday revealed an irregularly irregular heart rhythm. [vdocuments.mx]

  Physical examination on the sixth postoperative day revealed an irregularly irregular heart rhythm. The rate ranged from 80 to 123 beats per minute. [docplayer.net]

• Eye Pain

  pain; Idiopathic intracranial hypertension (pseudotumor cerebri); Cough, exertional, and sex headaches; The neck and headaches; Drug-induced headaches; Trigeminal neuralgia and glossopharyngeal neuralgia; Headaches and vasculitis; Tonsilar ectopia and [books.google.com]

• Ankle Pain

  Between week 20of gestation and delivery, physical examination, laboratory testing, plain radiography, and magnetic resonance imaging (MRI) wereperformed to investigate the cause of the ankle pain. [vdocuments.mx]

  J Invest Dermatol 2003;120: DOI /art Clinical Images: Transient regional osteoporosis The patient, a 33-year-old woman, developed left ankle pain at week 20 of gestation during her first pregnancy. [docplayer.net]

• Osteopenia

  Plain radiography revealed generalized osteopenia of the bones of the left foot. MRI showedextensive bone marrow edema within the left talus, with similar patchy changes present in adjacent bones. [vdocuments.mx]

  Plain radiography revealed generalized osteopenia of the bones of the left foot. MRI showed extensive bone marrow edema within the left talus, with similar patchy changes present in adjacent bones. [docplayer.net]

• Thigh Pain

  A 54-year-old man presented with right lower abdominal and upper thigh pain. Cross-sectional imaging of the abdomen revealed right hydronephrosis caused by a mass adjacent to the bladder. [em-consulte.com]

• Dysuria

  Other presenting signs or symptoms include leg edema, varicocele, testicular pain, claudication, dysuria, frequency and, in cases with advanced bilateral ureteral stenosis, oligo-anuria. Thrombophlebitis or deep vein thrombosis may also occur. [omicsonline.org]

• Anuria

  Other presenting signs or symptoms include leg edema, varicocele, testicular pain, claudication, dysuria, frequency and, in cases with advanced bilateral ureteral stenosis, oligo-anuria. Thrombophlebitis or deep vein thrombosis may also occur. [omicsonline.org]

• Forgetful

  Chiara Tioni, Marco Antonutti, Concetta Di Nora and Alessandro Proclemer, Never forget the aorta: a case report of IgG4-related disease causing aortitis, European Heart Journal - Case Reports, 10.1093/ehjcr/yty111, (2018) . [doi.org]

• Neuralgia

  […] and glossopharyngeal neuralgia; Headaches and vasculitis; Tonsilar ectopia and headaches; Post-traumatic headaches; Metabolic headaches; Temporomandibular joint disorders, bruxism, and headaches. [books.google.com]

Despite extensive workup, no … View Full Text Request Permissions If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. [heart.bmj.com]

If the pathologic workup for this case had ceased with only two cassettes of aortic wall, then the aortitis would have been misclassified as lymphoplasmacytic, and would raise suspicion for IgG4-related aortitis [ 13 ]. [uscapknowledgehub.org]

[…] infiltration of IgG4-positive plasma cells, storiform fibrosis, obliterative phlebitis, and elevated concentration serum IgG4. 2 Tissue biopsy is the gold standard for diagnosis of IgG4-related disease; however, imaging is an important part of the diagnostic workup [circimaging.ahajournals.org]

Diagnostic workup for IgG4-RD requires a high index of suspicion, and further workup often includes the results of serological testing for elevated levels of IgG4. [readbyqxmd.com]

[…] fibrosis. [14] Chronic periaortitis had been recognized as a common manifestation of IgG4RD, and it commonly affects abdominal aorta; it often coexists with autoimmune pancreatitis, another prototype manifestation of this disease. [15] In the diagnostic workup [indianjrheumatol.com]

• Normocytic Anemia

  Prior to surgery, the patient hadhad mild normochromic, normocytic anemia, with ahematocrit value of 37.4% (normal 4153) and a meancorpuscular volume of 77 fl (normal 80100). The serumcreatinine level was 1.5 mg/dl (normal 0.81.2). [vdocuments.mx]

  Prior to surgery, the patient had had mild normochromic, normocytic anemia, with a hematocrit value of 37.4% (normal 41 53) and a mean corpuscular volume of 77 fl (normal ). The serum creatinine level was 1.5 mg/dl (normal ). Other Figure 3. [docplayer.net]

• Fibrinoid Necrosis

  Necrosis, especially fibrinoid necrosis, is not characteristic of IgG4-RD. Figure 1. Obliterative phlebitis in a case of retroperitoneal fibrosis. A 54-year-old man presented with right lower abdominal and upper thigh pain. [em-consulte.com]

At 1 month following treatment, her IgG4 levels had fallen to 31 mg/dL. [mdedge.com]

Treatment of the aortitis may prevent progression of the IgG4-related systemic disease to involvement of other organs. IgG4-related systemic disease should be considered in all patients with aortitis judged to be of unknown etiology. [ncbi.nlm.nih.gov]

Textbook of Systemic Vasculitis is an up-to-date and forward looking guide to the condition, featuring potential future developments, as well as reviewing advances in treatment over the past half-century. [books.google.com]

Further studies into diagnostic criteria, disease prevalence, prognosis, therapeutic interventions, and differentiating possible localized hypersensitivity reactions from systemic disease are areas of active investigation. [ncbi.nlm.nih.gov]

[…] of chapters throughout allows readers to easily scan through for the information required Useful summary boxes outline the main points of each condition including aetiology, pathology, epidemiology, clinical features, investigations, management and prognosis [books.google.com]

Female patients seem to have milder inflammation and a better visual prognosis. [medworm.com]

In conclusion the data show a rather good overall prognosis of CP and they question a central role of IgG4 in the pathogenesis of periaortitis. [rheuma-schweiz.ch]

Retroperitoneal fibrosis Periaortic and aortic inflammation associated with retroperitoneal and mediastinal fibrosis Behcet's disease Genital and oral aphthous ulcers, uveitis Sarcoidosis Interstitial lung diseases Relationship between postoperative prognosis [freethesaurus.com]

IgG4-related systemic disease should be considered in all patients with aortitis judged to be of unknown etiology. [Indexed for MEDLINE] Free full text [ncbi.nlm.nih.gov]

The secondary headaches include a diverse and fascinating array of etiologies which can mimic primary headache disorders ranging from the rare to the mundane and from the well established to the highly controversial. [books.google.com]

Stone and his colleagues concluded that IgG4-related systemic disease should be considered in all patients with aortitis of unknown etiology, and noted that treatment might prevent progression of the systemic disease to other organs. [mdedge.com]

Their etiology varies greatly based on patient’s age and other clinicopathologic determinants. [ijcep.com]

IgG4-related systemic disease should be considered in all patients with aortitis judged to be of unknown etiology. [docplayer.net]

Management of Acute Hemorrhage Platelets Plasma and Specialized Coagulation Concentrate New Approaches for the Therapy of Bleeding Disorders Overview of Venous Thromboembolism Thrombophilia Genetics Epidemiology and Risk Factors for Venous Clinical Manifestations [books.google.de]

Textbook of Systemic Vasculitis is an in-depth guide to the epidemiology, classification and genetics of systemic vasculitis as well as imaging, symptoms and management of the disease. [books.google.com]

Manifestations and Diagnosis of Venous Venous Thrombosis in Unusual Sites Victor J Marder Hylton V Joffe and Sam Schulman Prevention of Venous Thromboembolic Disease Treatment of Venous Thromboembolic Disease Overview of Arterial Thrombotic Disorders Pathophysiology [books.google.de]

Mediterr J Rheumatol 2016;27(4):191-3 58-year-old patient with IgG4-related thoracic aortitis Authors Information Department of Pathophysiology, Medical School, University of Athens, Athens, Greece Abstract We report a 58-year-old male with a one-month [mjrheum.org]

It should be regarded as a generalized disease with three different pathophysiological entities, specifically idiopathic retroperito... more Ref 78 Download PDF Cite this paper An elevated IgG4 response in chronic infectious aortitis is associated with [scinapse.io]

CONCLUSIONS: The results of this retrospective multicenter study highlight three important points: (1) the possibility of latent existence and progression of periaortic/periarterial lesions, (2) the efficacy of corticosteroid therapy in preventing new [ncbi.nlm.nih.gov]

[…] of Venous Thromboembolic Disease Treatment of Venous Thromboembolic Disease Overview of Arterial Thrombotic Disorders Pathophysiology of Atherothrombosis Prevention of Thromboembolic Stroke in Patients with Acute Thrombolytic Therapy Unusual Sites of [books.google.de]

Conclusions : The results of this retrospective multicenter study highlight three important points: (1) the possibility of latent existence and progression of periaortic/periarterial lesions, (2) the efficacy of corticosteroid therapy in preventing new [pubfacts.com]