Wegener's Granulomatosis Presenting Research Articles

INFLAMMATORY MONOARTHRITIS AS A RARE INITIAL PRESENTATION OF GRANULOMATOSIS WITH POLYANGIITIS

INFLAMMATORY MONOARTHRITIS AS A RARE INITIAL PRESENTATION OF GRANULOMATOSIS WITH POLYANGIITIS

GRANULOMATOSIS WITH POLYANGIITIS PRESENTING WITH PLEURAL AND PERICARDIAL EFFUSIONS

SESSION TITLE: Medical Student/Resident Pulmonary Manifestations of Systemic Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Granulomatosis with polyangiitis (GPA) is a type of ANCA-associated vasculitis affecting small and medium-size vessels. Usually associated with necrotizing granulomas, this disease commonly involves both the upper and lower respiratory tracts as well as the kidneys. Lung and cardiac involvement are common, however initial presentation with pleural effusion and pericardial effusion in the absence of uremic pericarditis has been reported in only a handful of cases. CASE PRESENTATION: Our patient was a 58-year-old woman with no prior medical history who presented to the emergency department with monocular vision loss, and shortness of breath. The initial evaluation was remarkable for normal renal function, lactic acidosis, troponin elevation as well as elevated CRP and ESR. In addition, CT chest showed a large pericardial effusion, bilateral pleural effusions(R>L), and right pulmonary infiltrates. MRI brain showed findings suspicious for acute or subacute lacunar infarcts within the mid pons and bilateral basal ganglia, and paranasal sinus disease. The myriad of symptoms that she described on admission raised the concern for a systemic process including unusual infections potentially related to her recent stay in Scotland, rheumatologic diseases like vasculitis or possible malignancy. Diagnostic thoracentesis revealed an exudative effusion. Infectious workup was negative including HIV test. cANCA was positive as well as antiproteinase 3 antibodies. Skin biopsy showed a medium-size vessel leukocytoclastic vasculitis and septal panniculitis. Given the findings, she was started on high dose steroids for suspicion of GCA, with a significant improvement of her symptoms, including resolution of dyspnea. DISCUSSION: In patients with GPA, lung involvement is frequent, as it will commonly present as parenchymatous lesions in 48-73% at presentation and 85-92% during the course of the disease. Pleural involvement is rarely seen usually present in 10% of this patient population, and it usually manifests as pleural thickening and pleural effusion. When a pleural effusion is present, usually unilateral, exudative, sterile, associated with predominant neutrophils and low glucose concentration. Pericardial effusion and cardiac tamponade have been linked to constrictive pericarditis due to uremia and not to the inflammatory process of the autoimmune disease per se. Our patient presented with pericardial effusion in the absence of uremia which raises the possibility of being linked to the autoimmune state. CONCLUSIONS: This case helps us illustrate the importance of keeping the differential diagnosis broad when approaching a patient with multisystem involvement. It is important to contemplate vasculitis in our differential diagnosis to be able to order the appropriate testing to avoid delay in the diagnosis and to be able to start treatment in a timely manner. Reference #1: Blundell AG, Roe S. Wegener's granulomatosis presenting as a pleural effusion. BMJ. 2003;327(7406):95-6. Reference #2: Chin M, Leblanc A, Souza C, et al. A severe pleural complication associated with granulomatosis with polyangiitis. Respir Med Case Rep. 2019;28:100933. Reference #3: Parmar MK, Alikhan M, Hsu VM, Borham A. Echocardiogram: The GPS to GPA's Heart (Granulomatosis with Polyangiitis). Case Rep Rheumatol. 2019;2019:7609386. DISCLOSURES: No relevant relationships by Daniel Colon Hidalgo, source=Web Response No relevant relationships by Sri Mahathi Kalipatnapu, source=Web Response No relevant relationships by Maria Jose Zabala Ramirez, source=Web Response

Wegener's Granulomatosis Presenting as a Diffuse Palatal Swelling, a Rare Presentation: Case Report

Wegener's Granulomatosis Presenting as a Diffuse Palatal Swelling, a Rare Presentation: Case Report

An Unusual Presentation of Wegener's Granulomatosis with Otologic Symptoms and Unilateral Parotid Gland Enlargement.

Wegener's granulomatosis is an autoimmune disease affecting the lungs, kidneys and upper respiratory organs. Major salivary gland enlargement is a rare presenting symptom. We are reporting a rare case of Wegener's granulomatosis presenting with pain in right ear with moderate hearing loss and unilateral painless parotid gland enlargement.

Wegener's granulomatosis presenting with multiple cranial nerve palsies and pachymeningitis

A previously healthy 27-year-old male professional singer presented with a 1-month history of difficulty in singing, fever and night sweats. He had been given a 1-week course of oral corticosteroids...

An Unusual Manifestation of Wegener's Granulomatosis Presenting with Multiple Cranial Neuropathies (P06.226)

An Unusual Manifestation of Wegener's Granulomatosis Presenting with Multiple Cranial Neuropathies (P06.226)

Wegener's Granulomatosis Presenting with Bilateral Facial Nerve Palsy and Limited to the Head and Neck

Objectives. To illustrate the only reported case of Wegener's granulomatosis limited to the head and neck, and to provide an update on current knowledge and treatment of Wegener's granulomatosis. Case report. A 50-year-old man presented with bilateral chronic otitis and mastoiditis, eventually developing bilateral facial palsies. While he progressed with other head and neck symptoms, his disease was limited entirely to the head and neck. Diagnosis of Wegener's granulomatosis was delayed for this reason, due to the lack of systemic manifestations and the inability to confirm the diagnosis with a tissue biopsy, despite an elevated c-ANCA level. Conclusion. The early diagnosis and initiation of appropriate treatment of Wegener's granulomatosis is essential to prevent progression of the disease to a severe and irreversible phase. This case emphasizes that an awareness of this rare presentation is important to prevent the unnecessary delay of appropriate treatment in order to optimize the patient's prognosis, particularly when classic therapy for otitis or facial palsy is ineffective.

Wegener's Granulomatosis Presenting as Bilateral Otalgia with Facial Palsy: A Case Report

Wegener's granulomatosis (WG) is defined as a granulomatous inflammation of the upper and lower respiratory tract due to a systemic vasculitis. Facial nerve palsy may occur during the course of any granulomatous or vasculitis disease. But very few cases have been reported in which facial nerve palsy is the presenting feature of WG. We recently experienced an interesting case of WG presenting as acute middle ear infection and subsequent bilateral facial palsy.

Wegener′s granulomatosis with subdural hematoma as the initial manifestation

Wegener's granulomatosis (WG) is a systemic vasculitis, which can involve any organ system in the body; however, involvement of central nervous system at presentation is very uncommon. Dural involvement in WG has been infrequently reported; however, presentation with subdural hematoma as the initial manifestation is extremely rare. We present a case of WG presenting as subdural hematoma, which resolved on cytotoxic therapy without surgical evacuation.

Diagnostic Value of Biopsies in Identifying Cytoplasmic Antineutrophil Cytoplasmic Antibody–negative Localized Wegener's Granulomatosis Presenting Primarily with Sinonasal Disease

A substantial proportion of Wegener's disease (WG) patients present with localized disease of the upper airways, i.e., sinonasal and other ear/nose/throat (ENT) symptoms. Because of the oligosymptomatic presentation a timely diagnosis of this potentially fatal disease is challenging. This study evaluates diagnostic peculiarities between WG in its localized and generalized form of the disease. Retrospective analysis was performed of 82 patients with suspected WG manifesting in the ENT region between 1989 and 2009. Comparison was performed of the clinical and laboratory results between patients with localized (n = 15) and generalized stage (n = 16) as well as non-WG patients (n = 50). ENT signs and symptoms were subtle, especially in the population presenting with localized disease. Therapy refractory rhinosinusitis or serous otitis media were the most frequent presentations of WG. In testing for localized WG, mucosal biopsy had the highest sensitivity (53%) compared with cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCAs) with a lower sensitivity (47%) but highest specificity (96%) and highest positive predictive value (PPV; 78% versus 73%). Patients with generalized WG typically revealed a pathological urine sediment, hemoptysis, or rheumatological symptoms. In the generalized stage, c-ANCA had the highest sensitivity (81%), specificity (96% versus 95%), and highest PPV (87%). Timely diagnosis and treatment of localized WG limited to the ENT region remains problematic. Even with adequate therapy, nearly one-half of patients with sinonasal localization suffer from relapse, at least 1 in 10 will progress to generalized disease, and up to two-thirds may develop permanent tissue damage. Unfortunately, the diagnostic usefulness of c-ANCA is significantly reduced at this early stage compared with cases with generalized disease (p = 0.04). Hence, the relative diagnostic value of mucosal biopsy increases especially for the significant proportion of c-ANCA(-) localized WG patients (47%). Sinonasal tissue sampling represents a cornerstone for diagnosis, which unlike c-ANCA testing can be repeated in short intervals and is associated with low morbidity.

Hemorrhagic Colitis as a Manifestation of Wegenerʼs Granulomatosis: Think Outside the Box

Case Report: A 37-year-old African American male presented to our institution with a 2 week history of diffuse crampy abdominal pain associated with frequent watery bowel movements and low grade fever. On presentation he was tachycardic, febrile and abdominal exam was unremarkable. Blood work revealed normocytic anemia, leucocytosis, coagulopathy, elevated ESR, CRP and hypokalemia and was started on intravenous antibiotics. Chest X-ray at admission revealed multiple cavitary lesions. Echocardiogram, stool studies, pancultures, serum Quantiferon, HIV test and urinalysis were unrevealing. CT chest showed lung opacities suspicious for septic emboli or neoplastic process and was started on anticoagulation that precipitated rectal bleeding while awaiting lung biopsy. Colonoscopy revealed large, deep, skipped, exudative ulcerations throughout the colon, pathology significant for chronic inflammation with crypt distortion. Due to the persistence of fever on antibiotics, steroids were started for possible autoimmune etiology. CT guided lung biopsy revealed suppurative granulomas with small vassel vasculitis, highly suspicious for Wegener's granulomatosis (WG) in the setting of elevated C-ANCA. Repeat colonoscopy for rectal bleeding revealed improvement in ulcerations with antibiotic and steroid use. Thoracoscopic lung biopsy revealed thrombotic arteriopathy, vascular intimal thickening indicative of vasculitis. He was discharged home on steroids having refused cyclophosphamide. He was asymptomatic at 6 months follow-up. Discussion: We present a unique case of a patient with pulmonary nodules and hemorrhagic colitis consistent with WG. Intestinal manifestations as an initial presentation of WG are rarely reported. As intestinal involvement is rare in WG, an important differential is inflammatory bowel disease (IBD), though lung manifestations are rare in IBD. Although our initial suspicion was Crohn's disease based on colonoscopic findings and pathology results, further investigation of the patient's lung pathology confirmed a systemic vasculitic process consistent with WG. Conclusion: Combined pulmonary and intestinal findings carry a small list of differentials, of which vasculitis including WG should be high on the differential.

Wegener granulomatosis presenting as refractory otitis media: A case report

Wegener's granulomatosis (WG) is a granulomatous widespread necrotizing vasculitis, sometimes progressing rapidly, characterized by a tendency to involve the upper and lower respiratory tracts, and in most cases, the kidneys. Otologic manifestations may be seen during the course of the disease, however, they are not frequently seen as the first and only presenting feature. The presence of anticytoplasmic antibodies versus neutrophil polymorphonucleate granules (c-ANCA) is highly specific for the diagnosis of WG, being positive in 97% of the cases. The early diagnosis and the timely medical treatment result in high rates of remission of this potentially lethal disease. In this article, we report a case of WG presenting with acute serous otitis media, progressing to suppurative otitis media and granulomas as the first and only symptom, which was refractory to medical therapy during follow-up and which was diagnosed with further examination.

Hypophyseal Wegener's granulomatosis presenting by visual field constriction without hypopituitarism

The authors report a 48-year-old woman with known sysemic Wegener’s granulomatosis and recent onset bitemporal uperior quadrantanopia in whom magnetic resonance imagng, transphenoidal adenohypophysectomy and histopathology onfirmed necrotising granulomatous hypophysitis causing optic hiasm compression. Pituitary gland involvement in Wegener’s ranulomatosis is rare, first manifesting most commonly with entral diabetes insipidus, sometimes with concomitant hypopiuitarism affecting other hormonal axes and extremely rarely ith visual symptoms. The previously unreported presentation f hypophyseal Wegener’s granulomatosis with bitemporal heminopia but neither diabetes insipidus nor hypopituitarism is iscussed.

Wegener's granulomatosis presenting as unhealing palatal ulceration – A case report

This article reports a case of 45-year-old female presented with pain and swelling in lower jaw with generalised burning sensation over the gums, mobility in upper anterior teeth and difficulty in feeding. On intra oral examination diffuse reddish inflamed and fragile gingiva was noticed with granular appearance. Painful whitish sloughed and indurated ulcerative lesion with erythematous margins was found over midpalate to buccal mucosa over anterior border of ramus. Consistent radiographic examination revealed cavitatory defect over right pleural base. List of various diagnostic possibilities has been given as differential diagnosis and finally findings of our case were most consistent with Wegener's granulomatosis which was confirmed by biopsy and serum testing for anti-neutrophilic cytoplasmic antibodies. Patient was cured with oral prednisolone and cyclophosphamide in the standard dose. After 3months cyclophosphamide was replaced with myecophenolate moeftil 500mg BD. At the follow up of 6months the patient was quiet normal and was relieved of all the symptoms. We feel that the clinicians should be aware of the possible, occurrence of oral lesions of WG and consider them in the differential diagnosis of any oral ulcer of unknown etiology. On further assessment, it is imperative for clinicians to conduct further laboratory and radiographic and histopathological investigations to rule out the disease.

Two cases of Wegener’s granulomatosis with pancreatic pseudocyst treated by endoscopic ultrasonography (EUS)-guided drainage

Wegener's granulomatosis is a systemic vasculitis with prominent involvement of the respiratory tract and kidney. There are 10 patients with Wegener's granulomatosis in the literature who were documented as acute pancreatitis. We present two cases with Wegener's granulomatosis presenting with acute pancreatitis and pancreatic pseudocyst. Endosonography-guided drainage of the pancreatic pseudocyst led to rapid clinical improvement. Pancreatic pseudocyst in Wegener's granulomatosis is not reported in the literature, and these are the first cases of Wegener's granulomatosis to be managed by endosonography-guided cyst drainage. The safety of endosonography-guided pancreatic pseudocyst drainage and the clinical features of the previous Wegener's granulomatosis cases with acute pancreatitis are discussed.

A Case of Wegener's Granulomatosis Presenting as a Diplopia

Wegener’s granulomatosis (WG) is a multisystemic granulomatous inflammatory disorder which is presumably caused by an autoimmune response. It typically targets the upper and lower respiratory tract and the kidney. Ocular involvement occurs in 50 to 60% of WG patients. However, orbital myositis and diplopia as a presenting symptom is a rare ocular manifestation. We present the case of a 49-year-old man who was admitted with bilateral diplopia due to orbital myositis under a diagnosis of WG. He was successfully treated with high dose corticosteroid therapy.

Wegener's granulomatosis with bilateral facial nerve paresis and bilateral profound sensorineural hearing loss

Wegener's granulomatosis (WG) is a fatal disease. Facial nerve paralysis in association with WG is rare, being present in about 5% of patients, alone or in combination with hearing loss and rarely may be the only presenting feature. We are reporting a case of WG presenting with bilateral facial paresis and its peculiarity consists in its acute onset, lack of a significant previous history of ear disease and its association with bilateral profound sensorineural hearing loss. A Medline search revealed that there has been no reported case of WG presenting with bilateral facial involvement with bilateral profound sensorineural hearing loss.

Limited Wegener's Granulomatosis Presenting as a Solitary Cavitary Mass

Limited Wegener's Granulomatosis Presenting as a Solitary Cavitary Mass

An Unusual Presentation of Wegener Disease of the Cervix

: Wegener granulomatosis is a rare autoimmune disease of unknown cause. It commonly affects the respiratory tracts, lungs, and kidneys.Involvement of the uterine cervix is rare, with only 4 previously published cases in the literature. : This is a case report. : We describe the unusual presentation of Wegener granulomatosis involving the uterine cervix, presenting as cervical cancer. : A review of previously published cases of Wegener granulomatosis involving the uterine cervix is presented.We report the first case of Wegener granulomatosis involving the cervix without antecedent diagnosis or treatment of Wegener granuloma in any other organ.

Wegener's Granulomatosis Initially Presenting as Pleural Effusion

Pulmonary involvement is very common in the patients with Wegener's granulomatosis (WG). However, pleural effusion as the initial and major thoracic presentation of WG has not yet been reported before. We reported a 52-year-old male presenting with an acute onset of low-grade fever, left chest pain and generalized musculoskeletal soreness. Serial imaging studies showed progression of bilateral pleural effusions. The results of pleural fluid analysis revealed an exudate with neutrophil predominance. WG was diagnosed based on the findings of necrotizing granulomatous vasculitis on the closed pleural biopsy specimens and high levels of cytoplasmic antineutrophil cytoplasmic antibody detected in both serum and pleural fluid. The pleural effusion resolved almost completely 6 weeks after corticosteroid treatment. This reported case provides evidencehighlights that WG may be added into the list of different diagnoses ofin the patients with exudative pleural effusion with neutrophil predominance, even in those without concomitant renal and/or pulmonary lesions.