Dynamically changing antineutrophil cytoplasmic antibodies in granulomatosis with polyangiitis:A case report

BACKGROUND Granulomatosis with polyangiitis(GPA)is one of the most prevalent forms of the antineutrophil cytoplasmic antibody(ANCA)-associated vasculitis.GPA is characterized histologically by necrotizing granulomatous inflammation in addition to vasculitis.The diagnosis of GPA depends on clinical presentation,serological evidence of a positive ANCA,and/or histological evidence of necrotizing vasculitis or granulomatous destructive parenchymal inflammation.Cytoplasmic ANCA(c-ANCA)is positive in 65%-75% of GPA patients,accompanied by proteinase 3(PR3),the main target antigen of c-ANCA,another 5% of GPA patients had negative ANCA.CASE SUMMARY The patient,a 52-year-old male,presented with unexplained nasal congestion,tinnitus,and hearing loss.After a duration of 4 months experiencing these symptoms,the patient subsequently developed fever and headache.The imaging examination revealed the presence of bilateral auricular mastoiditis and partial paranasal sinusitis,and the ANCA results were negative.The anti-infective therapy proved to be ineffective,but the patient's symptoms and fever were quickly relieved after 1 wk of treatment with methylprednisolone 40 mg once a day.However,after continuous use of methylprednisolone tablets for 3 months,the patient experienced a recurrence of fever accompanied by right-sided migraine,positive c-ANCA and PR3,and increased total protein in cerebrospinal fluid.The and cyclophosphamide 0.8 g monthly,the patient experienced alleviation of fever and headache.Additionally,the ANCA levels became negative and there has been no recurrence.CONCLUSION For GPA patients with negative ANCA,there is a potential for early missed diagnosis.The integration of histopathological results and multidisciplinary communication plays a crucial role in facilitating ANCA-negative GPA.

A Bronchopulmonary Onset of Candidemia Revealing a Granulomatosis with Polyangiitis

Candidemia is defined as being a yeast infection confirmed by the presence of at least one positive Candida blood culture. It is a life threatening infection causing high mortality. The clinical signs are generally compatible with the causative agent (whether there is a deep venous catheter or not). On the other hand and according to the 2012 Revised Chapel Hill Classification, granulomatosis with polyangiitis GPA is classified as a vasculitis associated with antineutrophil cytoplasmic antibodies ANCA. It is a systemic disease characterized by the anatomopathological aspect of granuloma. We report the case of a patient who presented an atypical and a very rare revealing mode of GPA which was a bronchopulmonary candidiasis complicated by candidemia. Despite its controversy, the combination in the acute phase of antifungal treatment based on intravenous voriconazole and glucocorticoid therapy has made it possible to control candidemia and calm vasculitis.

Successful treatment of granulomatosis with polyangiitis using tocilizumab combined with glucocorticoids:A case report

BACKGROUND Tocilizumab is a humanized monoclonal antibody against the interleukin-6(IL-6)receptor that is commonly used to treat large vessel vasculitis and antineutrophil cytoplasmic antibody-related small vessel vasculitis.However,tocilizumab in combination with glucocorticoids for successfully treating granulomatosis with polyangiitis(GPA)has rarely been reported.CASE SUMMARY Here,we report a 40-year-old male patient who suffered GPA for 4 years.He was treated with multiple rounds of drugs,including cyclophosphamide,Tripterygium wilfordii,mycophenolate mofetil,and belimumab,with no improvement.In addition,he exhibited persistently high IL-6 levels.After tocilizumab treatment,his symptoms improved,and his inflammatory marker levels returned to normal.CONCLUSION Tocilizumab may be effective for treating GPA.

Ruptured gastric artery aneurysm:An uncommon manifestation of microscopic polyangiitis

Gastric artery aneurysm is a rare and lethal condition,and is caused by inflammatory or degenerative vasculopathies.We describe herein the clinical course of a patient with a ruptured gastric artery aneurysm associated with microscopic polyangiitis.Absence of vasculitic changes in the aneurysm resected and negative results of autoantibodies interfered with our diagnostic process.We should have adopted an interventional radiology and initiated steroid therapy promptly to rescue the patient.

Cyclophosphamide-associated enteritis presenting with severe protein-losing enteropathy in granulomatosis with polyangiitis:A case report

BACKGROUND Although cyclophosphamide(CPA)is the key drug for the treatment of autoimmune diseases including vasculitides,it has some well-known adverse effects,such as myelosuppression,hemorrhagic cystitis,infertility,and infection.However,CPA-associated severe enteritis is a rare adverse effect,and only one case with a lethal clinical course has been reported.Therefore,the appropriate management of patients with CPA-associated severe enteritis is unclear.CASE SUMMARY We present the case of a 61-year-old woman diagnosed with granulomatosis with polyangiitis based on the presence of symptoms in ear,lung,and,kidney with positive myeloperoxidase-antineutrophil cytoplasmic antibody.She received pulsed methylprednisolone followed by prednisolone 55 mg/d and intravenous CPA at a dose of 500 mg/mo.Ten days after the second course of intravenous CPA,she developed nausea,vomiting,and diarrhea,and was admitted to the hospital.Laboratory testing revealed hypoalbuminemia,suggesting proteinlosing enteropathy.Computed tomography revealed wall thickening of the stomach,small intestine,and colon with contrast enhancement on the lumen side.Antibiotics and immunosuppressive therapy were not effective,and the patient’s enteritis did not improve for>4 mo.Because her condition became seriously exhausted,corticosteroids were tapered and supportive therapies including intravenous hyperalimentation,replenishment of albumin and gamma globulin,plasma exchange,and infection control were continued.These supportive therapies improved her condition,and her enteritis gradually regressed.She was finally discharged 7 mo later.CONCLUSION Immediate discontinuation of CPA and intensive supportive therapy are crucial for the survival of patients with CPA-associated severe enteritis.

Characteristics and Prognosis of Microscopic Polyangiitis Patients with Diffuse Alveolar Hemorrhage and Interstitial Lung Disease

Objective To evaluate the clinical characteristics and prognostic predictors of patients with diffuse alveolar hemorrhage(DAH)and/or interstitial lung disease(ILD)secondary to microscopic polyangiitis(MPA)in a Chinese general hospital.Methods We retrospectively reviewed the medical records of MPA patients admitted to internal medicine departments between the year 2002 and 2012.The patients were divided into the ILD,DAH,DAH combined with ILD(DAHILD),and no pulmonary involvement(NPI)groups according to pulmonary involvement patterns.The clinical characteristics at diagnosis were analyzed.The risk factors associated with short-term death and long-term death were identified with Logistic regression and Cox analysis.Results Of 193 newly diagnosed MPA patients,181 patients were enrolled in the research,of which 19 had DAH alone,96 had ILD alone,18 had DAH and DAH concurrently,and 48 had NPI.The median of serum creatine level in the DAH group was 449μmol/L,significantly higher than that in the ILD group(123μmol/L,Nemenyi=-35.215,P=0.045)and DAHILD group(359μmol/L,Nemenyi=-43.609,P=0.007).The median follow-up time was 67(range:1-199)months.Patients in the ILD group were older than those in the DAH group(median:69 years vs.57 years,Nemenyi=43.853,P=0.005).The patients with both DAH and ILD had combined features of the two subtypes,and the highest mortality(72.2%at the end of follow-up).The elevated white blood cell count was a risk factor for short-term death(OR=1.103,95%CI:1.008-1.207,P=0.032 for one month;OR=1.103,95%CI:1.026-1.186,P=0.008 for one year).Old age(HR=1.044,95%CI:1.023-1.066,P<0.001),cardiovascular system involvement(HR=2.093,95%CI:1.195-3.665,P=0.010),poor renal function(HR=1.001,95%CI:1.000-1.002,P=0.032)were risk factors for long-term death.Pulmonary infections(38/54)were the leading causes of death,especially for the patients with ILD.Besides,49 patients suffered from pulmonary infections in the first year after diagnosis.Conclusions MPA patients who presented with different pulmonary involvement patterns have completely different clinical features.These subtypes probably have different pathogenesis and should be studied separately.

Granulomatosis with polyangiitis presenting as high fever with diffuse alveolar hemorrhage and otitis media: A case report

BACKGROUND Granulomatosis with polyangiitis is a necrotizing inflammation of small andmedium-sized vessels accompanied by formation of granuloma, involvement ofprimary granulomatous upper and lower respiratory tracts, glomerulonephritis,and vasculitis of small vessels.CASE SUMMARY Herein, we described a case of a 52-year-old man admitted with pulmonarynodules and high fever. Autoantibody workup revealed that the patient waspositive for c-anti-neutrophil cytoplasmic antibodies and proteinase-3 antineutrophilcytoplasmic antibodies. Pulmonary biopsies revealed a localgranulomatous structure. The patient received therapy with methylprednisoloneand intravenous immunoglobulin, and his clinical symptoms improved.CONCLUSION Intravenous immunoglobulin may act on granulomatosis with polyangiitis similarto immunosuppressants.

Acute myocardial infarction induced by eosinophilic granulomatosis with polyangiitis:A case report

BACKGROUND Eosinophilic granulomatosis with polyangiitis(EGPA)is a multisystem disease characterized by allergic rhinitis,asthma,and a significantly high eosinophil count in the peripheral blood.It mainly involves the arterioles and venules.When the coronary arteries are invaded,it can lead to acute myocardial infarction(AMI),acute heart failure,and other manifestations that often lead to death in the absence of timely treatment.CASE SUMMARY A 69-year-old man was admitted to the emergency department due to chest pain for more than 1 h.He had a past history of bronchial asthma and chronic obstructive pulmonary disease and was diagnosed with AMI and heart failure.Thrombus aspiration of the left circumflex artery and percutaneous transluminal coronary angioplasty were performed immediately.After surgery,the patient was admitted to the intensive care unit.The patient developed eosinophilia,and medical history taking revealed fatigue of both thighs 1 mo prior.Local skin numbness and manifestations of peripheral nerve involvement were found on the lateral side of the right thigh.Skin biopsy of the lower limbs pathologically confirmed EGPA.The patient was treated with methylprednisolone combined with intravenous immunoglobulin and was discharged after 21 d.On follow-up at 7 d after discharge,heart failure recurred.The condition improved after cardiotonic and diuretic treatment,and the patient was discharged.CONCLUSION Asthma,impaired cardiac function,and eosinophilia are indicative of EGPA.Delayed diagnosis often leads to heart involvement and death.

Pericardial effusion with tamponade – an uncommon presentation leading to the diagnosis of eosinophilic granulomatosis polyangiitis: A case report

BACKGROUND Eosinophilic granulomatosis polyangiitis(EGPA)is a small vessel necrotizing vasculitis that commonly presents as peripheral eosinophilia and asthma;however,it can rarely manifest with cardiac involvement such as pericarditis and cardiac tamponade.Isolated pericardial tamponade presenting as the initial symptom of EGPA is exceedingly rare.Early diagnosis and appropriate treatment are crucial to prevent life-threatening outcomes.CASE SUMMARY 52-year-old woman with no past medical history presented with progressive dyspnea and dry cough.On physical exam she had a pericardial friction rub and bilateral rales.Vital signs were notable for tachycardia at 119 beats per minute and hypoxia with 89%oxygen saturation.On laboratory exam,she had 45%peripheral eosinophilia,troponin elevation of 1.1 ng/mL and N-terminal prohormone of brain natriuretic peptide of 2101 pg/mL.TTE confirmed a large pericardial effusion and tamponade physiology.She underwent urgent pericardial window procedure.Pericardial and lung biopsy demonstrated eosinophilic infiltration.Based on the American College of Radiology guidelines,the patient was diagnosed with EGPA which manifested in its rare form of cardiac tamponade.She was treated with steroid taper and mepolizumab.CONCLUSION This case highlights that when isolated pericardial involvement occurs in EGPA,diagnosis is recognized by performing pericardial biopsy demonstrating histopathologic evidence of eosinophilic infiltration.

Familial Eosinophilic Granulomatosis with Polyangiitis

Eosinophilic granulomatosis with polyangiitis (EGPA) is an uncommon ANCA associated vasculitic disorder characterized by systemic necrotizing vasculitis of small vessels occurring exclusively among patients with bronchial asthma and tissue eosinophilia. Familial EGPA is extremely rare. Only two case reports have been published so far. We present a Saudi family with 3 cases of EGPA and almost three-fourths of family members affected by asthma. We explored genetic basis of EGPA in this family and found that genes were mutated in four affected siblings suggesting genetic involvement in susceptibility to EGPA.

A Fatal Case of Treatment-Related Adverse Effects in Granulomatosis with Polyangiitis (Wegener’s Granulomatosis)

Well-studied therapies have proven to be effective in treating Granulomatosis with polyangiitis (formerly Wegener’s granulomatosis) (GPA). There has been considerable improvement in survival of patients with GPA but treatment related morbidity and mortality remains still high, particularly in patients with renal disease. We describe a case of 64-year old woman with recent onset GPA, who responds well to the initial cyclophosphamide based therapy but latter develops a fatal stroke. Infectious complications should be considered in patients with GPA who are on adequate immunosuppression but develop symptoms that may mimic a relapse. Aggressive diagnostic interventions should be undertaken to discriminate between an infection and a relapse of GPA.

Eosinophilic Granulomatosis with Polyangiitis and Bowel Perforation: Case Report

The small and medium sized vessels vasculitis included in the group of vasculitis associated with anti-neutrophil cytoplasmic antibodies,is a rare disease characterized by the presence of vasculitis in association with asthma and eosinophilia.The objective of this report is to describe a case with a typical initial presentation of eosinophilic granulomatosis with polyangiitis-EGPA,that evolved with atypical clinical manifestations and findings in complementary exams.Justifying the clinical evolution and comprehending the therapeutic response of the patient in question,with scientific embasement on published medical literature.

Ocular Manifestations in Granulomatosis with Polyangiitis Patients from Saudi Arabia

Eye and/or orbit involvement occurs in Granulomatosis with polyangiitis (GPA) patients frequently. The aim of our study was to describe the clinical manifestations, therapy and outcome of ocular involvement in our GPA patients. A retrospective study was conducted including patients with GPA who followed up in Rheumatology clinics during 1990-2016 at King Khalid University Hospital, Riyadh. Information relating to demographics, ocular manifestations, laboratory findings, therapy and outcome of GPA patients were noted. Ocular involvement was detected in 9 (39.1%) of the 23 GPA cases identified. The mean age of ocular GPA patients was 51.8 (range 27 - 62) years, the mean age at onset of disease was 39.6 (range 11 - 57) years and the mean duration of disease was 9.0 (range 2 - 19) years. Concomitant ear, nose, throat and sino-nasal manifestations occurred with ocular symptoms in 77.8% GPA patients. The most frequent manifestations were, eye pain (66.7%), scleritis/episcleritis (55.6%), eye redness and itching (55.6% each). Antineutrophil cytoplasmic antibodies (ANCA) were positive in 88.9% patients, 55.6% had c-ANCA and 33.3% had p-ANCA. Infections were observed in 22.2% of patients, which included pneumonia in one patient and esophageal candidiasis and bacterial meningitis in another. All patients received oral prednisolone, 44.4% received intravenous cyclophosphamide, 22.2% refractory cases received rituximab doses and the disease outcome was good. Comparison of ocular GPA with non-ocular GPA patients showed that 77.8% of ocular GPA patients had concomitant sino-nasal symptoms compared to 42.9% in non-ocular GPA patients and 22.2% of the ocular GPA patients had renal involvement compared to 64.3% in non-ocular GPA patients (p = 0.049). We found that the frequency of ocular manifestations in our GPA patients was similar to reports elsewhere, and the most frequent symptom was eye pain and scleritis/episcleritis.

Prolonged conjunctivitis mimicking nodular episcleritis as a manifestation of granulomatosis with polyangiitis-a case report

Red eye is common in our daily practice.It ranges from non-inflammatory to inflammatory causes.An extended course of disease should prompt suspicion and the possibility of diagnosis revision.A prolonged conjunctivitis mimicking nodular episcleritis can be presented as a manifestation of granulomatosis with polyangiitis(GPA).A 57-year-old woman complained of eye redness and tearing for two weeks which partially resolved with antibiotics.She was subsequently commenced on topical and oral non-steroidal anti-inflammatory drugs(NSAIDs)and topical anti-allergic.However,in the following reviews she developed cornea thinning and her systemic examination revealed an injected uvula with absence of upper respiratory tract infection.She was investigated for connective tissue disease and found to have raised anti-inflammatory markers and her antinuclear antibody and C-ANCA tests were positive.She was diagnosed with GPA.Her conditions improved followed by the commencement of topical corticosteroid with high dose of systemic corticosteroid,which followed by a tapering regime with oral corticosteroid.Although red eye is common,it is associated with a variety of diseases.GPA manifestation can be as subtle as a red eye.Any prolonged partially treated red eye should prompt suspicion of a more sinister cause.Sensitive detection of other subtle systemic signs is very important.

Periungual Capillaroscopy Findings in a Series of Patients with Granulomatosis with Polyangiitis(Wegener):An Observational Study

Introduction: Periungual capillaroscopy studies skin microcirculation. Its main indication is in the differential diagnosis of Raynaud’s phenomenon as well as diagnosis and prognosis of systemic sclerosis (SS). Many rheumatic diseases present characteristic capillaroscopic alterations, but little is known at present about the alterations in skin microcirculation of systemic vasculitis. Objective: Our main objective was to study the alterations in skin microcirculation by capillaroscopy in patients with granulomatosis with polyangiitis (GPA). The secondary objective was to evaluate the association of capillaroscopic alterations with systemic alteration. Material and Methods: We studied patients in our unit with GPA, and we collected demographic and clinical variables. Periungual capillaroscopy was performed on all patients, and always by the same investigator. Results: We reviewed 10 patients with a mean age of 55.7. They were predominantly women. We found capillaroscopic alterations in 8 of the 10 patients. Morphological alteration was the most common finding (62.5%), followed by bleeding (50%). We found no mega-capillaries or neovessels. These findings were not related to any specific organ system involvement. Discussion: The tortuous pattern with bleeding was predominant in our series, with lower capillary density, findings which were unrelated to any specific organ system involvement. A single study describes capillaroscopic alterations in GPA, detecting a high percentage (92%) of avascular areas, in contrast with the predominance of a twisting pattern and bleeding found in our study. Further studies are warranted to confirm these results.

Efficacy of borneol-gypsum in skin regeneration and pain control in toxic epidermal necrolysis:A case report

BACKGROUND Toxic epidermal necrolysis(TEN)is a life-threatening dermatological emergency mainly induced by drug hypersensitivity reactions.Standard management includes discontinuation of culprit drug and application of immunomodulatory therapy.However,mortality remains high due to complications like septic shock and multiorgan failures.Innovative approaches for skin care are crucial.This report introduces borneol-gypsum,a traditional Chinese drug but a novel dressing serving as an adjuvant of TEN therapy,might significantly improve skin conditions and patient outcomes in TEN.CASE SUMMARY A 38-year-old woman diagnosed with eosinophilic granulomatosis with polyangiitis experienced gangrenous complications and motor nerve involvement.After initial treatment of high-dose corticosteroids and cyclophosphamide,symptom of foot drop improved,absolute eosinophil counts decreased,while limb pain sustained.Duloxetine was added to alleviate her symptom.Subsequently,TEN developed.Additional topical application of borneol-gypsum dressing not only protected the skin lesions from infection but also significantly eased localized pain.This approach demonstrated its merit in TEN management by promoting skin healing and potentially reducing infection risks.CONCLUSION Borneol-gypsum dressing is a promising adjuvant that could significantly improve TEN management,skin regeneration,and patient comfort.

肉芽肿性多血管炎累及会厌及杓区1例

1临床资料患者,女,21岁,因咽痛1周,加重伴呼吸困难1 d于2021-11-26入院。患者感冒后出现咽痛,逐渐加重伴活动后呼吸困难,就诊于承德医学院附属医院耳鼻咽喉科急诊。喉镜示:会厌及杓充血肿胀(图1A),以“急性会厌炎”收入院。查体:会厌及杓区充血、肿胀,声门区窥视不清。尿常规:隐血(1+),葡萄糖(2+),蛋白(+-),白细胞65.30μl,白细胞高视野11.80 HPF,尿沉渣白细胞23,尿沉渣红细胞4,胸部CT未见明确异常,肝肾功未见明确异常。入院后给予激素及足量抗生素静点治疗1周后肿胀未见明显好转,遂考虑全身系统疾病.

Prognosis of microscopic polyangiitis with renal involvement:report of 60 Chinese patients

With the widespread clinical application of renal biopsy and serum antineutrophil cytoplasmicantibody （ANCA） testing, the percentage of renal injuries caused by systemic small vessel vasculitis （ SVV ） including microscopic polyangiitis （ MPA ） and Wegener＇ s granulomatosis （WG） is on the rise in China. According to our previous report, SVV constituted 1.09% of diagnoses from all renal biopsies （5. 8% in secondary nephritis） and 14.4% of that from renal biopsies from patients with chronic renal failure,

Extracorporeal membrane oxygenation for pulmonary hemorrhage in microscopic polyangiitis

Microscopic polyangiitis （MPA） is characterized by an inflammatory process of the vessel walls and is classified according to the smallest vessels involved. Diagnosis is based on clinical manifestations, anti-neutrophil cytoplasmic autoantibody （ANCA） testing, and histology. In this disease, pulmonary hemorrhage is seen in 30% of patients and is associated with an eight-fold increase in mortality. Classically, MPA involves the kidneys and upper and lower respiratory tracts. Due to the perceived risk of increased bleeding secondary to the use of systemic anticoagulation, extra corporeal membrane oxygenation （ECMO） is often not used to treat patients with hemorrhage suffered from MPA. Here we report a case of a 50-year-old woman with MPA that was successfully supported with ECMO.

耳鼻受累首发的肉芽肿性血管炎1例

1临床资料患者,女,57岁,既往体健,因“右耳不适1个月”于2022-01-03收治入院。1个月前患者因右耳耳闷伴轻度听力下降反复就诊于当地医院,考虑分泌性中耳炎,经口服抗生素、激素等药物保守治疗后未能完全缓解,收治于我科。专科查体:右耳耳道湿润可见淡黄色脓液,稍黏稠,量稍多,鼓膜充血肿胀,标志欠清,鼓室可见积液(图1A)。