Novel pediatric granulomatosis with polyangiitis with a marked bloody pericardial effusion and bloody stool: a case report

Abstract Background Granulomatosis with polyangiitis (GPA) is a syndrome of refractory vasculitis involving the upper respiratory tract, lungs, kidneys, and systemic small and medium-sized arteries that affects all age groups. No pediatric case with a bloody pericardial effusion resulting in cardiac tamponade and co-existing hematochezia has been reported. Case presentation A 14-year-old boy was referred for evaluation of prolonged fever, chest pain, and intermittent hematochezia. Diagnostic imaging showed a prominent pericardial effusion. Immediately after admission, his systolic blood pressure decreased. Emergent pericardiocentesis resulted in aspiration of a massive amount of bloody pericardial fluid. This was diagnosed as cardiac tamponade because his blood pressure recovered immediately after the drainage. The patient had an elevated serine proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA) level on serological examination. Head MRI showed thickening of nasal and sinusoidal mucosa and a cystic mass in the left sphenoid sinus. After ruling out malignancy based on the cytology of the effusion, chest MRI, and gallium scintigraphy, total colonoscopy showed multiple irregular-shaped aphthae from the right transverse colon to the cecum on the contralateral side of the mesenteric attachments. Biopsy specimens of aphthous lesions confirmed necrotizing granulomatous inflammation. A diagnosis of GPA was made based on these findings, and oral prednisolone (PSL) and azathioprine were started. The hematochezia disappeared rapidly, and no recurrence of pericardial effusion was seen after PSL tapering was completed. The PR3-ANCA level decreased into the normal range immediately after the initial therapy. Conclusions Pericarditis is a common cardiac complication of GPA, but there have been no reports of resultant cardiac tamponade. This is the first case of pediatric GPA with cardiac and gastrointestinal complications preceding the common symptoms such as respiratory or renal symptoms. A case of pediatric GPA with hematochezia is also extremely rare. In conclusion, serial measurement of ANCA levels is important in patients with persistent fever and bloody stool, such as in inflammatory bowel disease, to make the diagnosis of a vasculitic syndrome.

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Novel Pediatric Granulomatosis With Polyangiitis With a Marked Bloody Pericardial Effusion and Bloody Stool: A Case Report

10.21203/rs.3.rs-269101/v1 ◽

2021 ◽

Author(s):

Memi Kato ◽

Keisuke Jimbo ◽

Masumi Nagata ◽

Yoshiko Endo ◽

Kosuke Kashiwagi ◽

...

Keyword(s):

Blood Pressure ◽

Pericardial Effusion ◽

Respiratory Tract ◽

Cardiac Tamponade ◽

Granulomatosis With Polyangiitis ◽

Pericardial Fluid ◽

Bloody Stool ◽

Upper Respiratory Tract ◽

Gastrointestinal Complications ◽

Bloody Stools

Abstract Background Granulomatosis with polyangiitis (GPA) is a syndrome of refractory vasculitis in the upper respiratory tract, lungs, kidneys, and systemic small and medium-sized arteries; adults and children have similar courses. A pediatric GPA case with a preceding bloody pericardial effusion, which caused cardiac tamponade, and bloody stool has not been reported. Case presentation: A 14-year-old boy was referred for the evaluation of prolonged fever. He had chest pain and bloody stools, and diagnostic imaging showed a pericardial effusion. Immediately after admission, his systolic blood pressure decreased (85/70 mmHg), and pericardiocentesis was performed, with aspiration of approximately 500 mL of bloody pericardial fluid. Because pericardiocentesis increased the blood pressure (115/65 mmHg), the cause of the blood pressure decrease was diagnosed as cardiac tamponade. Because the pericardial fluid cytology was negative for malignant disease, as were chest MRI and gallium scintigraphy, colonoscopy was performed and showed multiple irregular-shaped aphthae from the right transverse colon to the cecum on the contralateral side of the mesenteric attachments, and a biopsy of the aphthous region showed necrotizing granulomatous inflammation. The patient also had an elevated serine proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA) level (141 IU/mL) on serological examination. Head MRI showed thickening of nasal and sinus mucosa and a cystic mass in the left sphenoid sinus. Therefore, GPA was diagnosed based on these findings, and treatment with oral prednisolone (PSL) and azathioprine was started. After the treatment, the bloody stool disappeared, and there was no recurrence of pericardial effusion even after completing PSL tapering, and the PR3-ANCA level was maintained in the normal range. Conclusions Although pericarditis is a common cardiac complication of GPA, there have been no previous reports of cardiac tamponade due to pericarditis. This is the first case of a pediatric patient with cardiac and gastrointestinal complications preceding the main symptoms, including upper and lower respiratory tract and renal symptoms, although cases of GPA with bloody stools are extremely rare. In conclusion, the sequencing of measuring ANCA levels appears important assuming the vasculitic syndrome as one of differential diseases causing persistent fever and bloody stool including such as the inflammatory bowel disease.

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Malignant pericardial effusion complicated by cardiac tamponade under atezolizumab

SAGE Open Medical Case Reports ◽

10.1177/2050313x211036005 ◽

2021 ◽

Vol 9 ◽

pp. 2050313X2110360

Author(s):

Lardinois Benjamin ◽

Goeminne Jean-Charles ◽

Miller Laurence ◽

Randazzo Adrien ◽

Laurent Terry ◽

...

Keyword(s):

Pericardial Effusion ◽

Cardiac Tamponade ◽

Cardiac Toxicity ◽

Checkpoint Inhibitors ◽

Final Diagnosis ◽

Pericardial Fluid ◽

Malignant Pericardial Effusion ◽

Typical Cell ◽

High Fluorescence ◽

Tomography Scan

Immune-related adverse events including cardiac toxicity are increasingly described in patients receiving immune checkpoint inhibitors. We described a malignant pericardial effusion complicated by a cardiac tamponade in an advanced non-small cell lung cancer patient who had received five infusions of atezolizumab, a PDL-1 monoclonal antibody, in combination with cabozantinib. The definitive diagnosis was quickly made by cytology examination showing typical cell abnormalities and high fluorescence cell information provided by the hematology analyzer. The administration of atezolizumab and cabozantinib was temporarily discontinued due to cardiogenic hepatic failure following cardiac tamponade. After the re-initiation of the treatment, pericardial effusion relapsed. In this patient, the analysis of the pericardial fluid led to the final diagnosis of pericardial tumor progression. This was afterwards confirmed by the finding of proliferating intrapericardial tissue by computed tomography scan and ultrasound. This report emphasizes the value of cytology analysis performed in a hematology laboratory as an accurate and immediate tool for malignancy detection in pericardial effusions.

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Severe course of quickly progressing glomerulonephritis in a patient with ANTSA-associated vasculitis: review and case report

Translational Medicine ◽

10.18705/2311-4495-2020-7-3-55-62 ◽

2020 ◽

Vol 7 (3) ◽

pp. 55-62

Author(s):

Iu. V. Lavrishcheva ◽

Y. S. Kaledinova ◽

A. A. Yakovenko ◽

I. A. Artemev

Keyword(s):

Granulomatosis With Polyangiitis ◽

Rapidly Progressive Glomerulonephritis ◽

Granulomatous Inflammation ◽

Clinical Work ◽

Chronic Hemodialysis ◽

Complete Loss ◽

Vascular Lesions ◽

Upper Respiratory Tract ◽

Delay In Diagnosis ◽

Necrotizing Glomerulonephritis

Granulomatosis with polyangiitis is characterized by necrotizing granulomatous inflammation, vasculitis with vascular lesions of small and medium caliber and focal necrotizing glomerulonephritis. A frequent and one of the most formidable complications is kidney damage, which in a large number of cases leads to a complete loss of organ function and a switch to renal replacement therapy. Given the rare occurrence of this disease in the clinical work of practitioners, and their low awareness of this pathology, problems often arise with the diagnosis and treatment of patients with HPA. Due to the diversity and non-specific nature of the manifestations of the disease, a delay in diagnosis may occur. The presented case illustrates the manifestations of granulomatosis with polyangiitis in the form of severe damage to the upper respiratory tract and kidneys, the diagnosis of which was difficult due to the rarity of the disease and the multiple organ pathology. This article presents a clinical case of severe progression of rapidly progressive glomerulonephritis in a patient with ANCA-associated vasculitis, a brief review of the literature is given. Despite adequate therapy, the disease progressed mainly due to deterioration of renal function, which subsequently led to a complete loss of kidney function and the transition to treatment with chronic hemodialysis.

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Graves’ disease inducing a massive cardiac tamponade

BMJ Case Reports ◽

10.1136/bcr-2020-239772 ◽

2021 ◽

Vol 14 (3) ◽

pp. e239772

Author(s):

Elisabeth Martinez Fonseca ◽

Igor Schonhofen ◽

Maria Pereira Toralles ◽

Jozelio Freire de Carvalho

Keyword(s):

Pericardial Effusion ◽

Cardiac Tamponade ◽

Nyha Class ◽

Clinical Manifestations ◽

Emergency Admission ◽

Heart Association ◽

Pericardial Fluid ◽

Graves Disease ◽

Class Iii ◽

Filling Time

A 23-year-old woman was diagnosed with Graves’ disease 5 months ago with decompensated thyroid function, for which she is taking thiamazole and propranolol. She developed progressive respiratory dyspnoea [New York Heart Association (NYHA) class III] and frequent palpitations. On emergency admission, the patient was tachypnoeic, hypotensive (77/54 mm Hg) and tachycardic (120 beats per minute), with an oxygen saturation of 94%. She also presented with cold, swollen and shaky extremities, with extended capillary filling time, and a significant reduction in heart sounds. Echocardiogram showed massive pericardial effusion compatible with cardiac tamponade. Pericardiocentesis was performed, with a drainage of 1420 mL serosanguinolent fluid, with prompt haemodynamic recovery. Analysis of the pericardial fluid showed exudates. A diagnosis of pericardial effusion secondary to Graves’ disease was determined and corticotherapy, lithium carbonate, cholestyramine and phenobarbital were prescribed. An oral iodine-131 was performed and the patient showed reasonable control of the clinical manifestations of hyperthyroidism. After 3 months, the patient showed no symptoms of hyperthyroidism and a new echocardiogram revealed a significant reduction in pericardial effusion.

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GRANULOMATOSIS WITH POLYANGIITIS: TREAT THE PATIENT NOT SYMPTOMS

The Journal of V. N. Karazin Kharkiv National University, Series "Medicine" ◽

10.26565/2313-6693-2018-35-17 ◽

2018 ◽

Keyword(s):

Granulomatosis With Polyangiitis ◽

Treatment Strategies ◽

Granulomatous Inflammation ◽

Organ Damage ◽

Upper Respiratory Tract ◽

Childbearing Age ◽

Immunological Mechanisms ◽

Upper Respiratory ◽

Tract Infections ◽

In Pregnancy

Immunological mechanisms of appearance and therapeutic treatment strategies were discussed on example of the rare granulomatosis with polyangiitis clinical case in young patient. This vasculitis, formerly known as Wegener’s granulomatosis, is a rare multisystem autoimmune disease with necrotizing granulomatous inflammation and pauci-immune vasculitis in small- and medium-sized blood vessels. Autoimmune diseases affect 5 to 7% of people, are commoner in women of childbearing age, and are frequently encountered in pregnancy. They may remit or improve during pregnancy, butcan flare or present in pregnancy with disastrous consequences. Otorhinolaryngologist is the first physician to contact for the majority of patients with GPA. This diagnosis must always be taken into consideration in patients with recurrent upper respiratory tract infections, otitis, mucosal ulcers and laryngitis. Proper and early diagnosis is crucial for imminent therapy implementation and allows avoiding irreversible organ damage.

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Intrapericardial tetracycline sclerosis in the treatment of malignant pericardial effusion: an analysis of thirty-three cases.

Journal of Clinical Oncology ◽

10.1200/jco.1984.2.6.631 ◽

1984 ◽

Vol 2 (6) ◽

pp. 631-636 ◽

Cited By ~ 67

Author(s):

S Davis ◽

P Rambotti ◽

F Grignani

Keyword(s):

Pericardial Effusion ◽

Cardiac Tamponade ◽

Performance Status ◽

Signs And Symptoms ◽

Pericardial Fluid ◽

Complete Control ◽

Malignant Pericardial Effusion ◽

Efficacious Treatment ◽

Clinically Significant ◽

Premature Removal

Thirty-three unselected patients with cardiac tamponade secondary to malignant pericardial effusion were treated by intrapericardial instillation of tetracycline hydrochloride. Complete control of the initial signs and symptoms of tamponade was obtained in 30 patients without concomitant chemotherapy or radiotherapy. The procedure did not result in clinically significant complications. Failure of the technique was related to premature removal of the catheter by the patient (one patient) or the inability to totally remove hemorrhagic, clot-filled pericardial fluid (two patients). Survival ranged between 28-704 days and extended survival was related to the performance status and/or chemoradiosensitivity of the primary cancer. No patient successfully treated subsequently developed recurrent cardiac tamponade or alternatively, constrictive pericarditis. Tetracycline pericardial instillation remains a safe, simple, and efficacious treatment of tamponade secondary to malignant disease.

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Cardiac Tamponade - A Hypothyroidism Associated Emergency: A Report of 2 Cases

Austin Journal of Clinical Case Reports ◽

10.26420/austinjclincaserep.2021.1194 ◽

2021 ◽

Vol 8 (2) ◽

Author(s):

Tripathi S ◽

◽

Sharma JB ◽

Vijayvergia P ◽

Khichar S ◽

...

Keyword(s):

Emergency Department ◽

Pericardial Effusion ◽

Cardiac Tamponade ◽

Young Female ◽

Medical Emergency ◽

Myxedema Coma ◽

First Case ◽

History Of ◽

Medical Emergencies ◽

Recurrent Pericardial Effusion

Pericardial effusion in commonly seen in-patient with hypothyroidism but effusion large enough to cause cardiac tamponade is not a common presenting feature whereas myxedema coma is a commonly defined medical emergency in-patient with hypothyroidism. We report 2 cases of hypothyroid associated medical emergencies. First case is a young female with history of recurrent pericardial effusion presenting to the emergency department with cardiac tamponade and later on diagnosed as having hypothyroidism. The second patient is a known case of hypothyroidism non-compliant to thyroid supplement and presented with lethargy, fatigue, decreased talking and breathlessness who was later diagnosed as having myxedema coma and impending cardiac tamponade. Both the patient required percutaneous pericardiocentesis and improved with medical management.

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An Unusual Presentation of Tuberculous Pericardial Effusion

Journal of SAFOG with DVD ◽

10.5005/jp-journals-10006-1014 ◽

2009 ◽

Vol 1 (3) ◽

pp. 71-72

Author(s):

Shaheen LNU ◽

Rajyashri Sharma ◽

Parvez Anjum ◽

Pathak Jayshree

Keyword(s):

Pericardial Effusion ◽

Cardiac Tamponade ◽

Pericardial Fluid ◽

Accurate Diagnosis ◽

Unusual Presentation ◽

Severe Bleeding ◽

Acute Pericarditis ◽

Prompt Treatment ◽

Antitubercular Treatment ◽

Life Saving

ABSTRACT Tuberculosis accounts for upto 4% of acute pericarditis and 7% cases of cardiac tamponade. 19% of women with tuberculosis can present with menorrhagia. Prompt treatment can be life saving but requires accurate diagnosis. We report a case of 25-year-old women who presented with severe bleeding per vaginum for four days. She was in shock. Echocardiography showed moderate pericardial effusion with features of cardiac tamponade. ADA was positive in aspirated pericardial fluid. The patient responded well to antitubercular treatment.

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Cardiac tamponade due to malignant pericardial effusion in breast cancer: A case report

Bangabandhu Sheikh Mujib Medical University Journal ◽

10.3329/bsmmuj.v9i1.28946 ◽

2016 ◽

Vol 9 (1) ◽

pp. 53

Author(s):

Sultan Mahmud ◽

Omar Sadeque Khan ◽

Md. Aftabuddin ◽

Asit Baran Adhikary

Keyword(s):

Breast Cancer ◽

Pericardial Effusion ◽

Cardiac Tamponade ◽

Duct Cell ◽

Pericardial Fluid ◽

Pericardial Window ◽

Severe Respiratory Distress ◽

Malignant Pericardial Effusion ◽

History Of ◽

Subxiphoid Pericardial Window

We present a case of 35 years old women who presented to our institution with a history of bilateral infiltrating duct cell carcinoma of breast, chest pain with heaviness, severe respiratory distress and hypotension. Echocardiography revealed massive pericardial effusion with features of cardiac tamponade. The patient was treated with urgent pericardiocentesis followed by subxiphoid pericardial window drainage of 500ml of haemorrhagic pericardial fluid. Cytological examination confirmed the previous suspicious of malignancy. The patient tolerated the procedure very well, immediate symptomatic relief was observed.

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Nocardia beijingensis pericarditis presenting with cardiac tamponade: A case report

International Journal of STD & AIDS ◽

10.1177/0956462417736986 ◽

2017 ◽

Vol 29 (5) ◽

pp. 515-519 ◽

Cited By ~ 1

Author(s):

Nattinee Laksananun ◽

Parichat Salee ◽

Quanhathai Kaewpoowat

Keyword(s):

Cardiac Tamponade ◽

Correct Diagnosis ◽

Pericardial Fluid ◽

Nocardia Asteroides ◽

Cd4 Cell Count ◽

First Case ◽

Count Range ◽

Rrna Sequencing ◽

Smear Negative ◽

Cd4 Cell

We report the first case of Nocardia beijingensis pericarditis in a 32-year-old HIV-infected patient. He presented with cardiac tamponade after failing to respond to treatment for smear-negative pulmonary and pericardial tuberculosis (TB). The pericardial fluid was examined several times before it eventually revealed filamentous branching organisms in Gram and modified acid-fast bacilli stain. The culture grew Nocardia spp. and was identified by 16s rRNA sequencing as N. beijingensis. Eight previously reported cases of Nocardia pericarditis in HIV-infected patients were caused by Nocardia asteroides. All patients had low CD4 cell count (range: 17–239 cells/mm3) and 50% of patients were treated for tuberculous pericarditis prior to making the correct diagnosis of Nocardia pericarditis. This report revisits the issue of nocardiosis as a great TB mimicker. It should always be considered in the differential diagnosis among HIV-infected patients suspected of having pericardial TB that is failing treatment.

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