Polyangiitis Presenting Research Articles

Ocular Manifestation of Granulomatosis with Polyangiitis Presenting as Serous Retinal Detachment: A Case Report

Background: Ocular involvement is relatively common in granulomatosis with polyangiitis (GPA); however, choroidal involvement is rare. We present a case of serous retinal detachment resulting from choroidal involvement in GPA. Case presentation: A 55-year-old male presented to our clinic with bilateral eye redness and pain. Ocular examination revealed bilateral conjunctival injection, and dilation of the episcleral and scleral vessels. Slit-lamp examination revealed anterior chamber cells. Optical coherence tomography (OCT) confirmed serous retinal detachment (SRD) in the left eye. The patient had recently been diagnosed with GPA following a lung biopsy and had received immunosuppressive therapy, including systemic steroids, cyclosporine, mycophenolate mofetil, and rituximab. Five weeks after treatment, the clinical symptoms of the patient, including SRD, improved with alleviation of systemic symptoms. However, tapering systemic steroids and immunosuppressants and discontinuing rituximab led to disease reactivation. OCT demonstrated a recurrence of subretinal fluid, which had previously resolved, and slit-lamp examination showed mild bilateral engorged scleral vessels. Conclusions: Choroidal involvement can present as SRD and may indicate disease activity in patients with GPA.

A Rare Case of Granulomatosis with Polyangiitis Presenting with Esophagitis

A Rare Case of Granulomatosis with Polyangiitis Presenting with Esophagitis

Eosinophilic granulomatosis with polyangiitis presenting with eosinophilic myositis: A case report and review of literature

Eosinophilic granulomatosis with polyangiitis presenting with eosinophilic myositis: A case report and review of literature

Granulomatosis with polyangiitis presenting with dyspnea and chronic urticaria

Granulomatosis with polyangiitis presenting with dyspnea and chronic urticaria

A case of granulomatosis with polyangiitis presenting as cerebritis

Background: Isolated Central Nervous System (CNS) involvement in Granulomatosis with Polyangiitis (GPA) is rare. There are limited accounts of such a presentation in the literature. Case presentation: A 61-year-old gentleman presented with right upper limb and lower limb weakness, slurred speech and confusion following two instances of mechanical falls while at home. A cerebrovascular event was suspected. He underwent Computed Tomography (CT) brain and Magnetic Resonance Imaging (MRI) brain which demonstrated leptomeningeal thickening of left frontal region with vasogenic oedema. The differential diagnosis included malignancy, infection and contusion. He initially commenced on Dexamethasone 8 mg, Keppra 250 mg and a combination of Ceftriaxone, Linezolid, Metronidazole to treat vasogenic edema and possible infectious cerebritis, respectively. His vasculitis screen demonstrated a positive p-ANCA, positive for high titer PR3-ANCA. He was diagnosed with cerebral vasculitis secondary to GPA and commenced on immunosuppressant treatment.

Eosinophilic granulomatosis with polyangiitis presenting as an endobronchial nodule and atelectasis: A case report

Eosinophilic granulomatosis with polyangiitis presenting as an endobronchial nodule and atelectasis: A case report

Granulomatosis with polyangiitis presenting as acute ischemic stroke- A case of an unusual presentation

Ischemic stroke can be a rare atypical presentation of granulomatosis with polyangiitis (GPA). Awareness of this entity is vital when a clinician evaluates a patient for the aetiology of the ischemic stroke. We report a case of a 48-year-old female who presented with acute ischemic stroke and the etiology was found to be related to GPA. She was diagnosed according to the serological markers and MRI brain findings. Correct disease diagnosis is essential, as immunosuppressive therapy can improve the prognosis.

A case of granulomatosis of polyangiitis presenting with COVID-19 infection: False-positivity or co-existence?

Coronavirus disease 2019 (COVID-19) was declared a global pandemic and a public health emergency worldwide in March 2020.
 COVID-19 presents with non-specific symptoms of the upper airway and pulmonary system, which can overlap with other diseases
 involving the respiratory system as granulomatosis with polyangiitis (GPA). Both diseases have high morbidity and mortality rates
 and it is important to promptly differentiate and treat them. Real-time reverse transcriptase polymerase chain reaction (RT-PCR)
 is currently the recommended method for diagnosing COVID-19. Antibody-based tests are used to diagnose both pat and current
 COVID-19 infections.
 We present a previously healthy thirteen-year-old girl who was admitted with upper airway symptoms and pulmonary involvement,
 and progressed to acute kidney failure. Laboratory findings showed leukocytosis, anemia, elevated kidney function tests and 2+
 proteinuria. Computed tomography (CT) of the lungs showed multiple nodules, cavities, and ground-glass opacities (GGOs). We
 performed RT-PCR tests for COVID-19 for three times. Results were all negative, but the COVID-19 immunoglobulin (Ig)M test
 sent simultaneously was positive. Based on the cytoplasmic antineutrophilic cytoplasmic antibody (c-ANCA) positivity, upper airway,
 pulmonary, and renal involvement, she was diagnosed as GPA.
 This report highlights that COVID-19 antibody tests can be false-positive in patients with autoimmune diseases including GPA.

Eosinophilic Granulomatosis with Polyangiitis presenting as Vasculitis in the Temporal Artery

Eosinophilic Granulomatosis with Polyangiitis presenting as Vasculitis in the Temporal Artery

Eosinophilic Granulomatosis with Polyangiitis Presenting as Acute Non-segment Elevation Myocardial Infarction: A Case Report

Eosinophilic Granulomatosis with Polyangiitis Presenting as Acute Non-segment Elevation Myocardial Infarction: A Case Report

Granulomatosis with polyangiitis presenting as a solitary renal mass: a case report

BackgroundGranulomatosis with polyangiitis (GPA) is characterized by necrotizing granulomatous vasculitis involving small-sized vessels in the upper airways, lower airways, and kidneys. Renal pathology is usually characterized by focal segmental necrotizing glomerulonephritis, which often leads to rapidly progressive renal failure. This type of renal involvement is usually unapparent on radiography. The presence of a renal mass in a patient with GPA, although extremely rare, is recognizable. Herein, we report a rare case of GPA presenting as a solitary renal mass and present a review of the literature.Case presentationA 75-year-old woman presented with a solitary right kidney mass measuring 4 × 3.5 cm detected by enhanced computed tomography. There was no history of sinusitis, rhinitis, cough, or pneumonia suggestive of systemic GPA. Nephrectomy was performed based on the suspicion of renal cell carcinoma or malignant lymphoma. Three months later, she was admitted because her serum creatinine levels increased from 54.81 μmol/L to 405.76 μmol/L accompanied by a high C-reactive protein level of 159 mg/L. Anti-neutrophil cytoplasmic antibodies against myeloperoxidase and anti-proteinase 3 were negative. Histological examinations of the solitary renal mass revealed necrotizing granulomatous arteritis in the cortex and medullary vasa recta, surrounded by interstitial fibrosis, and focal segmental necrotizing glomerulonephritis in the localized lesion; however, signs of vasculitis were not observed in areas other than the solitary mass. Therefore, the patient was diagnosed with granulomatosis with polyangiitis (GPA). Despite treatment with prednisolone (30 mg/day), the patient developed oliguria with an elevation of her serum creatinine level to 583.44 μmol/L, which required hemodialysis within one month after the initiation of steroid therapy. The patient could successfully discontinue hemodialysis 21 months later, following a decrease in her serum creatinine level to 251.06 μmol/L.ConclusionsGPA should be considered as one of the differential diagnoses of a solitary renal mass. Furthermore, patients with solitary renal masses associated with GPA may exhibit a favorable response to steroid or immunosuppressive treatment.

Eosinophilic granulomatosis with polyangiitis presenting as aspirin–exacerbated respiratory disease

Eosinophilic granulomatosis with polyangiitis presenting as aspirin–exacerbated respiratory disease

Granulomatosis with polyangiitis presenting as exudative pleural effusion: A rare case report.

Granulomatosis with polyangiitis presenting as exudative pleural effusion: A rare case report.

Eosinophilic granulomatosis with polyangiitis presenting as recurrent myocardial infarction.

Eosinophilic granulomatosis with polyangiitis presenting as recurrent myocardial infarction.

Granulomatosis with Polyangiitis Presenting as a Renal Mass: A Scarce Case Report with a Review of the Literature

Wegener granulomatosis (WG) now known as granulomatosis with polyangiitis (GPA) is an uncommon autoimmune disorder of undivulged etiology affecting the respiratory tract including paranasal sinuses, nasal cavity, lungs, and kidneys predominantly. GPA presenting as a solitary renal mass is rarely seen. We present a case report of a 27-year-old female presenting with a right renal mass along with pain, low-grade fever, and arthralgia. Computed tomography scan of the abdomen revealed a hypodense low attenuated renal mass with indistinct margins. Ultrasound-guided biopsy revealed features typical of GPA. She was started on oral steroids (prednisolone 40 mg) and azathioprine. She developed pain, vomiting, and diarrhea after starting treatment with azathioprine. Azathioprine was stopped and rituximab 1 g weekly was started for 4 weeks followed by 500 mg 6 monthly injections. She got symptomatic relief at 4 weeks with a diminution of renal mass at 6 months follow-up. We report this rare entity of WG presenting as renal mass. Suspecting and diagnosing renal mass as a part of GPA prevented us from undertaking unnecessary surgical treatment in this patient. Medical treatment with steroids and rituximab is effective in inducing remission and maintenance.

Granulomatosis With Polyangiitis Presenting as an Infratemporal Fossa Mass

Granulomatosis with polyangiitis is a rare autoimmune disease that affects small to medium-sized blood vessels throughout the body. Here, we present a case of an infratemporal mass that was the result of granulomatosis with polyangiitis. A 51-year-old male presented to the emergency department due to right cheek and facial pain that he had been experiencing for 2 to 3 months. An MRI revealed a mass within the right infratemporal and pterygopalatine fossae extending into the inferior right orbital fissure along the maxillary division of the trigeminal nerve (V2) and the vidian nerve causing concern for malignancy. Histology from an endoscopic biopsy demonstrated multiple arteries with luminal obliteration with non-necrotizing granulomas. The patient was started on steroids and immunosuppressive therapy, which improved his symptoms and decreased the size of the residual mass. This case illustrates the need for laboratory testing, imaging, and biopsy of the involved tissue in cases where GPA is suspected to prevent treatment delays that could lead to the destruction of vital organs.

A Case of Granulomatosis with Polyangiitis Presenting as Necrotizing Pneumonia

Systemic vascular diseases frequently involve the respiratory system leading to inflammation of varying-sized vessels in the lungs by various immunological mechanisms. Granulomatosis with polyangiitis (Wegner’s granulomatosis) is one of the rare vascular diseases involving medium and small vessels in various organs. In typical cases, the upper and lower respiratory tract and kidneys are involved producing granulomatous inflammation of small arteries leading to necrotizing vasculitis usually associated with antineutrophil cytoplasmic antibodies (ANCA). Abnormal chest X-rays are seen in almost 70% of the patients during the course of the disease which includes nodules, infiltrate, and necrosis with subsequent cavitation. In rare cases, it may mimic the radiological features of pneumonia thus making the diagnosis difficult. So, here we present a case of a 53-year-old male who presented with necrotizing pneumonia with parapneumonic effusion later found out to be a case of C ANCA positive granulomatosis with polyangiitis.

Microscopic Polyangiitis Presenting with Acute Abdominal Pain Due to Rare Small Intestine Involvement

Microscopic Polyangiitis Presenting with Acute Abdominal Pain Due to Rare Small Intestine Involvement

Granulomatosis with polyangiitis presenting with multisystem impairment

Rationale: Granulomatosis with polyangiitis (GPA) mostly affects the upper and lower respiratory tracts and the kidneys. Although it may also affect other systems, multiorgan damage to the upper respiratory tract, lungs, kidneys, skin, hearing, and vision is rare. Patient concerns: A 52-year-old man was admitted to our hospital with fever, epistaxis, anosmia, blood in sputum, proteinuria, limb rashes, hearing loss, diplopia, night sweats, and chest pain. The patient had no history of any illness. Diagnoses: GPA was established based on multisystem clinical manifestations, nasal tissue biopsy, and positive serum antibodies. Interventions: Medical treatment with glucocorticoid and immunosuppressants was given to the patient. Outcomes: The patient’s condition improved; computed tomography scan of the lung indicated shrinkage of the lesion, and there were no complications or recurrence within the 5-month follow-up period. Lessons: For GPA patients with multisystem involvement, detailed physical examination is required to improve the survival rate and prognosis.

MICROSCOPIC POLYANGIITIS PRESENTING WITH CARDIAC TAMPONADE

MICROSCOPIC POLYANGIITIS PRESENTING WITH CARDIAC TAMPONADE