scholarly journals POS1230 OUTCOME FOLLOWING COVID-19 INFECTION IN ANTI-NEUTROPHIL CYTOPLASMIC ANTIBODY (ANCA)-ASSOCIATED VASCULITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 898.2-898
Author(s):  
A. Antovic ◽  
B. Lövström ◽  
A. Hugelius ◽  
O. Borjesson ◽  
A. Bruchfeld ◽  
...  
Keyword(s):  
Maintenance Therapy ◽  
Immunosuppressive Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Data Retrieval ◽  
Induction Treatment ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Upper Airways ◽  
Anca Associated Vasculitis

Background:Patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) require immunosuppressive therapy for disease control and reduction of disease relapse and may be at risk for complications during Sars-CoV-2 (COVID-19) infection.Objectives:To analyze the consequences of COVID-19 in a large cohort of AAV patients regarding occurrence, need of hospitalization, treatment at the intensive care units (ICU), or death.Methods:Data were retrieved from March 2020 to mid-January 2021 from medical records from the AAV cohort (n=233). Patients diagnosed with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) or eosinophilic granulomatosis with polyangiitis (EGPA) were included. Data included age, gender, diagnosis, ongoing immunosuppressive medication at onset of COVID-19 or at last follow-up in non-COVID individuals. Renal involvement (ever) and estimated glomerular filtration rate (eGFR) were included. COVID-19 was confirmed either by a positive PCR test in the upper airways or by serology. Severe COVID-19 was defined as need of non-invasive ventilation, ICU care, and/or death.Results:The cohort comprised of 172 patients with GPA, 50 with MPA and 11 with EGPA. There were 121 females (52%). During the study period, 20 patients (8.6%) were diagnosed with COVID-19. The median age at data retrieval in all patients was 68 years (21-93), in the COVID-19 group 63 (29-93) and 68.5 (21-90) years in the non-COVID patients.Fourty-three patients in all (18%) were hospitalized during the study period of which 11 (4.7%) due to COVID-19 infection. In all, 8 deaths occurred of which 3 were related to COVID-19.At data retrieval, 110 (47%) patients were on prednisolone treatment, 10/20 (50%) in the COVID-19 group and 100 (47%) in the non-COVID-19 group (p=0.5), with significantly higher doses in COVID-19 patients (p<0.001). In patients hospitalized with COVID-19, 6/11 (54.5%) were on prednisolone, median dose 5 mg/day (0-50). In the total group 112 (48%) were on disease modifying anti-rheumatic drugs (DMARD) and 64 (27.5%) on rituximab as maintenance therapy. Eight patients were on induction treatment with either cyclophosphamide or rituximab.Of the 20 COVID-19 cases, 8 had severe COVID-19. Of these, 2 were inactive without immunosuppressive treatment, 4 had stable disease with prednisolone (5-7.5 mg/day) in combination with DMARDs, and 2 were active treated with high dose prednisolone (25-50 mg/day) in combination with cyclophosphamide and rituximab (n=1) or rituximab (n=1).A higher proportion of patients had active AAV (p=0.03) in the severe COVID-19 then in the non-COVID group (10/213 patients).In the group with the severe COVID-19, 1/8 (12%) patient had rituximab as maintenance therapy, compared to 61/213 (28.6%) in the group of non-COVID-19 patients (p=0.5).Renal involvement (ever) was present in 144 patients (62%), in 6 patients (30%) with COVID- 19, from which 5 (62%) were in the group of severe COVID-19 patients. Median eGFR did not differ between severe COVID-19 and remaining patients with renal involvement independently of COVID-19 infection.Conclusion:We found a high rate of severe COVID-19 infection in our cohort of AAV patients which indicates risk for serious complications, especially in patients with active disease and intense immunosuppressive therapy. Maintenance therapy with rituximab did not seem to increase the risk for severe COVID-19. The findings stress the need for continued shielding and early vaccination in AAV patients.Disclosure of Interests:None declared

scholarly journals A Rare Case of Digital Ischemia and Gangrene in ANCA-Associated Vasculitis with Review of the Literature

2017 ◽  
Vol 2017 ◽  
pp. 1-7 ◽  
Author(s):  
Richard A. Lau ◽  
Ramandeep Bains ◽  
Duminda Suraweera ◽  
Jane Ma ◽  
Emil R. Heinze ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
English Literature ◽  
Renal Involvement ◽  
Antineutrophil Cytoplasmic Antibody ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Proteinase 3 ◽  
Anca Associated Vasculitis ◽  
Digital Ischemia

This paper describes one patient with Antineutrophil Cytoplasmic Antibody- (ANCA-) associated vasculitis who initially presented with multiple ischemic fingers and toes. On further evaluation, the patient was also found to have pulmonary-renal involvement and episcleritis. The diagnosis was supported with a positive cANCA (anti-proteinase 3) and a bronchoscopy consistent with diffuse alveolar hemorrhage. Although the patient refused a tissue biopsy, clinical presentation including nasal ulceration, sinus congestion, and epistaxis and anti-proteinase 3 antibody were more consistent with Granulomatosis with Polyangiitis (GPA) rather than Microscopic Polyangiitis (MPA) or Eosinophilic Granulomatosis with Polyangiitis (EGPA) based on the recently presented ACR/EULAR Provisional 2017 Classification Criteria for GPA (Luqmani et al., 2016). The patient responded well to therapy including high dose steroids and cyclophosphamide, with improvement of all organs involved and had no further digital ischemia or gangrene on follow-up. We include a review of the English literature summarizing presentation, management, and outcome of 16 similar cases.

scholarly journals ANCA-associated vasculitis in a 14 years-old patient: a clinical case

2020 ◽  
Vol 27 (5) ◽  
pp. 184-194
Author(s):  
A. V. Burlutskaya ◽  
N. V. Savelyeva ◽  
N. S. Тaran
Keyword(s):  
Respiratory Failure ◽  
Immunosuppressive Therapy ◽  
Clinical Case ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Pulse Therapy ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Anca Associated Vasculitis ◽  
Nasal Bleeding ◽  
Diagnostic Research

Background. ANCA-associated systemic vasculitis is a rare childhood disease. Antineutrophil cytoplasmic autoantibodies (ANCA)-related vasculitises include microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis. Their rarity often leads to a late diagnosis, rapid disability and high mortality in patients due to aggressive respiratory, pulmonary lesion and renal failure.Clinical Case Description. The patient suffered from a recurrent bronchoobstructive syndrome with signs of respiratory failure, obscure origin fever and chronic rhinitis with nasal bleeding for 6 months. The patient was diagnosed with obstructive bronchitis (putative bronchial asthma debut), received antibacterial therapy and inhalation bronchodilators without stable improvement during the entire period. Skin haemorrhages and arthralgia stimulated diagnostic research to establish ANCA-associated systemic vasculitis (presence of proteinase 3-specifi c ANCAs in titre 1/80). CT lung scanning revealed frosted glass foci of reduced pulmonary pneumatisation and signs of bilateral bronchoobstruction. Immunosuppressive therapy with glucocorticosteroids (methylprednisolone pulse therapy No. 3, 1000 mg intravenously on alternate days, subsequent per os administration of 1 mg/kg/day) and cyclophosphamide (500 mg intravenously once per 28 days) was prescribed. This led to the positive dynamics with eliminated fever and skin haemorrhages, as well as essentially reduced signs of respiratory failure.Conclusion. Diagnosis of systemic vasculitis is often complicated and long-term due to commonly non-specifi c debut symptoms of autoimmune disorders. In the described case, the fi rst 6 months of illness displayed intoxication and bronchoobstruction with signs of respiratory failure. Haemorrhagic rashes, arthralgias and the presence of ANCAs are proxy to vasculitis. Standard immunosuppressive therapy for ANCA-associated vasculitis improved the patient’s condition.

scholarly journals ANCA-Associated Vasculitis with Cardiac Valve Vegetations in Two Teenage Males: A Case Report and Literature Review

2022 ◽  
Author(s):  
Alexandra Theisen ◽  
Martha Rodriguez
Keyword(s):  
Cardiac Involvement ◽  
Granulomatosis With Polyangiitis ◽  
Pediatric Population ◽  
Adult Population ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Newly Diagnosed ◽  
Cardiac Morbidity ◽  
Anca Associated Vasculitis ◽  
Cardiac Manifestations

Abstract Background: Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis is a term used to describe systemic vasculitides that affect small and medium-sized blood vessels. The three types of ANCA-associated vasculitis (AAV) are Granulomatosis with Polyangiitis (GPA), formerly Wegener’s granulomatosis , Microscopic Polyangiitis (MPA), and Eosinophilic Granulomatosis with Polyangiitis (EGPA), formerly Churg-Strauss, with clinical presentation most frequently involving the respiratory tract, the kidney, the skin and the joints. ANCA-associated vasculitis is rare in children, with estimated prevalence of 3-4 per million, and even more rare is the manifestation of cardiac abnormalities secondary to ANCA-associated vasculitis in the pediatric population. Case Presentation: We present the cases of two teenage males who presented with cardiac valvular lesions secondary to Granulomatosis with Polyangiitis in addition to sinus, pulmonary, renal, and cutaneous involvement. These findings of cardiac valvular abnormalities in GPA have rarely been described in the literature in pediatrics. Both patients were treated with rituximab, high-dose methylprednisolone, and plasma exchange (PLEX) and showed improvement in their disease manifestations. Conclusions: A review of the literature revealed only five pediatric cases of ANCA-associated vasculitis with cardiac manifestations, and interestingly, three of the five had valvular involvement. Subsequent valvular involvement makes obtaining the diagnosis of ANCA-Associated Vasculitis very difficult due to concern for underlying infectious endocarditis and can lead to misdiagnosis given the rarity of cardiac involvement in ANCA-associated vasculitis. Routine echocardiogram is not always completed in newly diagnosed GPA, yet cardiac involvement can lead to severe consequences as was seen with our first patient in the form of thromboembolic stroke. We discuss the importance of keeping AAV on the differential when cardiac lesions are present as well as the importance of regular cardiac screening in newly diagnosed patients with AAV, as it is a major factor of cardiac morbidity and mortality in the adult population and can contribute substantially to management decisions.

scholarly journals AB0472 INFECTIOUS PROFILE IN PATIENTS WITH ANCA-ASSOCIATED VASCULITIS: RETROSPECTIVE ANALYSIS IN A REFERRAL CENTRE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1534.3-1534
Author(s):  
M. Estévez Gil ◽  
B. Maure ◽  
A. Argibay ◽  
C. Vazquez-Triñanes ◽  
B. Gimena ◽  
...  
Keyword(s):  
Autoimmune Diseases ◽  
Immunosuppressive Therapy ◽  
Bacterial Infections ◽  
Granulomatosis With Polyangiitis ◽  
Response To Therapy ◽  
Upper Airways ◽  
Factors Associated ◽  
Icu Admission ◽  
Anca Associated Vasculitis ◽  
Increased Risk

Background:The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are rare multisystem autoimmune diseases of unknown cause, characterised by inflammatory cell infiltration causing necrosis of blood vessels. The treatment of AAV requires prolonged immunosuppressive therapy. Infections remain a major cause of morbidity and mortality.Objectives:The aim of our study was to investigate the prevalence and characteristics of infection, and analyse the factors associated with infection in patients with AAV from Northern of Spain.Methods:Retrospective, descriptive study of patients with AAV followed in a specific Systemic Autoimmune Diseases and Thrombosis Unit from January 2000 to December 2019. Demographic, laboratory, microbiology, treatment and clinical data were collected from the medical records. AAV was diagnosed according to the definitions of the Chapel Hill nomenclature and designated as granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis (MPA) or pauci-immune necrotizing and/or crescentic glomerulonephritis without systemic vasculitis (renal-limited vasculitis, RLV). Disease activity of AAV was evaluated by Birmingham Vasculitis Activity score (BVAS). The infection episode was considered on the basis of clinical, laboratory, microbiology, radiology information, and response to therapy. Different episodes of infection in one patient were independently reflected. Data were analysed using SPSS 25.0Results:Thirty-six patients of which 20 (55.6%) were males. Median follow-up was 42 months. The mean age at the diagnosis was 61.14 ± 17.49 years and mean BVAS was 18.81 ± 5.96. 15 patients were diagnosed of GPA, 13 of MPA, 5 of EGPA and 3 of RLV. 72.2% MPO, 11.1% PR3. Lung involvement occurred in 75% of patients, upper airways was detected in 41.7%, skin involvement in 16.7%, Nervous system affectation occurred in 33.3%. 30 patients (83.3%) had renal affectation with a mean of 1.93± 1.66 gr/dl of Proteinuria and 2.9±2.17mg/dl of creatinine. We detected hypocomplementemia in 27.8% of patients (C3 in 19.4% and C4 in 16.7%). Regarding induction treatments, all patients received corticoids at high doses, 21 (58.3%) Cyclophosphamide, 3 (20%) Rituximab and 2 (13.3%) patients, Azathioprine. When we analyse infections, we detected 15 patients (41.66%) who presented any infection after the diagnosis of AAV, with a total of 71 episodes of infection. The most frequent were bacterial infections (29 episodes), specifically gram negative pathogens. The most frequent location was the respiratory (56.3%) followed by urinary (22.5%) and Skin (8.5%). Also opportunistic infections were described: 3 patients with Aspergillus fumigatus and one patient with Cryptococcus neoformans. 41 of these episodes needed hospitalisation with a median stay of 11 days. 6 episodes warranted intensive care unit (ICU) admission. Infection related mortality was 2.82%. We made latent tuberculosis screening and Pneumocystis prophylaxis in all our patients. No cases of Tuberculosis or Pneumocystis were recorded. Factors associated with increased risk of hospitalisation with statistical signification in univariated study were MPA, Hypocomplementemia and increased BVAS. But in the logistical regression study, only the value of the BVAS maintained statistical significance. The only factor associated with elevated risk of ICU admission was IgG deficit in the multivariate analysis. Neither immunosuppressive therapy nor age was associated with increased risk of infection in our study.Conclusion:More than 50% of the episodes of infection needed hospitalisation in patients with AAV. Risk factors for hospitalisation and ICU admission were BVAS and IgG deficit respectively. Bacterial infections were the most frequent but fungal infections were the most severe.Disclosure of Interests:None declared

Alveolar Hemorrhage in Vasculitis (Primary and Secondary)

2018 ◽  
Vol 39 (04) ◽  
pp. 482-493 ◽  
Author(s):  
Mouhamad Nasser ◽  
Vincent Cottin
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Granulomatosis With Polyangiitis ◽  
Alveolar Hemorrhage ◽  
Antithyroid Drugs ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Systemic Lupus ◽  
Anca Associated Vasculitis ◽  
Pulmonary Capillaritis

AbstractDefined by the accumulation of red blood cells into the alveolar space, diffuse alveolar hemorrhage (DAH) is a severe and potentially fatal medical condition requiring careful attention. In contrast to simple extravasation of erythrocytes facilitated by impaired hemostasis or hemodynamic causes, DAH in vasculitis is due to capillaritis, that is, inflammation of capillaries. Dyspnea, hemoptysis, chest infiltrates, and abrupt fall of blood hemoglobin level represent the cardinal features of DAH; yet, hemoptysis is lacking in one-third of cases. Bronchoalveolar lavage, retrieving bright red fluid, is the best diagnostic clue, also excluding infection and other causes of hemoptysis. Although not recommended, lung biopsy is the gold standard for the diagnosis of DAH and pulmonary capillaritis. Pulmonary capillaritis may be primary as in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis or secondary to drugs (especially antithyroid drugs such as propylthiouracil), infections, connective tissue diseases especially systemic lupus erythematosus, or other small vessel vasculitides. Newer toxic causes of drugs of abuse may be difficult to diagnose. Granulomatosis with polyangiitis and microscopic polyangiitis are the most common causes of capillaritis and DAH, whereas DAH is extremely rare in eosinophilic granulomatosis with polyangiitis. When pulmonary capillaritis is not secondary to underlying systemic vasculitis, idiopathic pauci-immune pulmonary capillaritis may be considered, with or without ANCA. Supportive treatment strategy is mandatory in all cases of DAH. Mechanical ventilation and extracorporeal membrane oxygenation may be used in severe cases. Early identification and removal of the putative drug is crucial in drug-induced vasculitis/DAH and may obviate the need for immunosuppressive therapy. High-dose corticosteroids, intravenous cyclophosphamide, and recently rituximab are the mainstay of treatment in vasculitis. Plasma exchange is recommended in anti–glomerular basement membrane disease and in severe DAH associated with systemic lupus erythematosus and is used in selected cases in ANCA-associated vasculitis.

P0410SIGNIFICANT BURDEN OF DISEASE DURING MAINTENANCE TREATMENT OF ANCA-ASSOCIATED VASCULITIS (AAV) PATIENTS IN REAL WORLD PRACTICE IN EUROPE

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Peter Rutherford ◽  
Dieter Götte
Keyword(s):  
Adverse Events ◽  
Maintenance Therapy ◽  
Induction Therapy ◽  
Remission Induction ◽  
Induction Treatment ◽  
High Dose ◽  
Full Remission ◽  
Anca Associated Vasculitis ◽  
Active Disease

Abstract Background and Aims ANCA associated vasculitis (AAV) is a relapsing remitting long term condition and patients are at risk of organ damage from both active AAV and its therapy in particular high dose and/or prolonged glucocorticoids (GC). The remission maintenance phase of AAV is critical for good long term outcomes including renal preservation as well as preventing AAV relapse. This retrospective study aimed to examine the definition of maintenance, therapy and clinical outcomes in patients managed in routine practice. Method 1478 AAV patients (France, Germany, Italy, Spain and UK) managed by 493 physicians (61% Nephrologists) who completed induction therapy for organ or life threatening AAV and initiated maintenance therapy between 2014-16 were studied. Data were collected retrospectively at the time maintenance was determined to begin by the physician and then at 6, 12, 18 and 36 months. Results 49% had granulomatosis with polyangiitis,; mean age 54.2 years with 56% male. 49% had incident AAV and 51% were studied from the time of a relapse requiring remission induction therapy. 70% received cyclophosphamide and GC and 30% received rituximab and GC as induction treatment with 28% receiving plasma exchange. Physicians defined time of the start of maintenance from induction treatment start with mean of 5.7 months on the basis of fixed time point 40%, starting new drug for maintenance 26%, reaching full remission 26% and no specific criteria 8%. At this time of maintenance start 43% were in full remission vs 50% in partial and 7% refractory. Various maintenance regimes were used, 21% received rituximab (88% 6 monthly and 8% 12 monthly, 4% other) at varying planned doses 34% 1g, 40% 500 mg and 23% 375 mg/m2, 4% other regime. Remission rates varied with patients experiencing disease relapse and many patients experienced adverse events (AE) and infections with prolonged GC use being common. Renal function was relatively unchanged and some patients had worsening eGFR, protein excretion or blood pressure. At the most recent clinical review patients had been followed for a mean of 50.7 months – 6% had died, 38% had relapsed at least once, and 11% required renal replacement therapy. 54% had no vasculitis activity, 26% were ANCA positive without active disease and 19% were still experiencing active disease. 32% were still receiving GCs - 22% of them receiving &gt; 5mg/ day. Conclusion Maintenance therapy is variably defined but typically at 6 months from start of remission induction therapy. Achieving full remission and preventing relapse are still clinical problems and many patients require ongoing GC therapy to maintain remission. Infectious complications and adverse events are common and renal disease remains an ongoing clinical problem.

scholarly journals AB0464 THE ROLE OF CYCLOPHOSPHAMIDE CHEMOTHERAPY IN THE TREATMENT OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1530.2-1531
Author(s):  
B. Bitik ◽  
M. Aydin ◽  
G. Sahin Dalgic ◽  
D. Kaskari ◽  
A. E. Yucel
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Treatment Modalities ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Level Of Evidence ◽  
Rare Type ◽  
The Third ◽  
Anca Associated Vasculitis ◽  
Bowel Involvement ◽  
Eosinophilic Granulomatosis

Background:Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare type of ANCA associated Vasculitis (AAVs). Cyclophosphamide (CYC) is generally recommended for the induction of remission in life/organ threatening AAVs in combination with glucocorticoids. However, due to its rarity, randomized controlled trials regarding the efficacy of treatment modalities in EGPA are hard to perform. Therefore, the level of evidence for the use of CYC in the treatment of EGPA is lower when compared to other AAVs (1).Objectives:The aim of this study is to investigate common therapeutic agents used for the treatment of patients with EGPA.Methods:Medical records of patients who were followed-up with the diagnosis of EGPA between 2007-2020, in rheumatology clinics of Ankara and Adana Hospitals of Başkent University, were analyzed retrospectively. Treatment outcomes were assessed.Results:Records of 11 patients (six females) were analyzed. The median age was 47 (19-77) years. The median follow-up time of the patients was 24 (9-156) months. Six patients were diagnosed with asthma. The median time between the diagnosis of asthma and EGPA was 4.5 (1-3) years. Five patients had tissue biopsies. Biopsy locations were terminal ileum, lung, myocardium and nerve. The most common forms of involvement were asthma, eosinophilic pneumonia and / or nodule, cardiovascular involvement, mononoritis multiplex, vasculitic skin rash, arthritis and bowel involvement, respectively. P-ANCA was positive in 8 patients. Three patients had myocarditis and cardiomyopathy, and two patients had isolated valve problems. The median BVAS value at the time of diagnosis and the third month of treatment was 17 (6-27) and 4 (2-7), respectively.Nine patients used oral 1mg/ kg methylprednisolone (MP) and 500mg CYC every two weeks as an induction therapy. The cumulative median CYC dose was 4.5 g (1.5-8). Neither of the patients developed CYC related side effects. MP was tapered to 2 mg in five patients, and was quited in two patients. Azathioprine (AZA) was used in remission treatment following CYC therapy. Rituximab (RTX) therapy 1 g twice, 2 weeks apart was initiated in two patients due to unresponsiveness to CYC. While RTX was effective in one patient, newly developed renal involvement was detected after the third cycle of RTX therapy in other patient. Two patients had pregnancy plan therefore they used AZA plus MP as induction. A patient had mycophenolate mofetil plus MP due to AZA allergy. All patients are currently in remission except one patient.Conclusion:In seven out of 11 EGPA patients, long-term remission was achieved with CYC treatment. CYC appears to be an effective and inexpensive method of first-line treatment for organ threatening EGPA.References:[1]Yates M, Watts RA, Bajema IM, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016 Sep;75(9):1583-94.Disclosure of Interests:None declared

scholarly journals POS0119 RENAL INVOLVEMENT AND NEED OF RENAL REPLACEMENT THERAPY IN ANCA ASSOCIATED VASCULITIS IN A SPANISH SINGLE-CENTER STUDY

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 270.2-271
Author(s):  
J. Álvarez Troncoso ◽  
J. C. Santacruz Mancheno ◽  
A. Díez Vidal ◽  
S. Afonso Ramos ◽  
A. Noblejas Mozo ◽  
...  
Keyword(s):  
Renal Replacement Therapy ◽  
Replacement Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Age At Diagnosis ◽  
Systemic Involvement ◽  
Risk Of Death ◽  
Anca Associated Vasculitis ◽  
The Mean

Background:Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EPGA). Renal involvement is frequent in AAV and is an important factor for morbidity and mortality.Objectives:The main objective of this study was to analyze the demographic, clinical, histological and therapeutic characteristics of renal involvement in patients with AAV and the risk of renal replacement therapy (RRT) or death.Methods:Retrospective observational study of 56 patients with AAV fulfilling classificatory criteria and renal involvement diagnosed between 1995 and 2020 from a Spanish tertiary centre. We studied the histological involvement (according to the 2010 classification in focal, crescentic, mixed or sclerotic), immunofluorescence (IF) and the treatment received with the risk of RRT or death.Results:We included 56 patients diagnosed with AAV and renal involvement. The mean age was 61.08±4.05 years; 58.9% were women. The mean follow-up time of these patients was 16.14± 8.80 years. Only 57.1% of patients presented systemic involvement.Most frequent non-renal AAV manifestations were lung involvement (39.3%), central nervous system (30.4%), otorhinolaryngology (ORL) (14.3%), skin (8.9%) and cardiac involvement (8.9%). Main immunological findings were ANCA-MPO+ (69.6%), ANCA-PR3+ (23.2%), ANCA-negative (5.4%). Low C3 was found in 19.6% patients. Histologic classification (HC) and need of RRT is described in table 1. Main HC in renal AAV was crescentic, mixed, focal and sclerotic respectively. Eight patients had not biopsy performed. IF was positive for C3 deposits in 20 patients (35.7%). Half of the patients presented <50% normal glomeruli.The treatment of renal involvement in AAV in our cohort was as follows: 83.9% (47) corticosteroids (CS) and cyclophosphamide (of which 40 received intravenous and 7 oral cyclophosphamide; and 12 patients associated plasma exchange (PE) with this treatment), 5.36% CS alone, 2 patients received CS and mycophenolate; 1 CS and rituximab, 1 CS and PE, and 2 patients received no treatment. A total of 13 patients received PE and 18 RRT. The mean time to RRT was 65.44±32.72 months. Relapses were not uncommon, 33.93% of the patients presented ≥1 relapse and 10.71% presented ≥2.Infections were very frequent since they were present in 91.07% of the patients. Other frequent non-immunological complications observed in the follow-up of these patients were thrombosis in 31.14%, cardiovascular events in 28.57% and cancer in 19.64%.Patients with ANCA-PR3+ were younger at diagnosis (p<0.001), were more likely to present cardiac (p=0.045) and ORL involvement (p<0.001). However, neither ANCA-PR3+ nor ANCA-MPO+ were specifically associated with the need of RRT or higher risk of death in our cohort. Use of CS alone for the treatment of renal AAV was associated with higher mortality (p=0.006) but CS and cyclophosphamide with lower mortality (p=0.044). ANCA-negative patients were more likely to receive no treatment. Having <50% normal glomeruli and C3 deposits on IF were associated with an increased need for RRT. Presenting focal disease on HC was protective for the need of RRT. Older age at diagnosis, systemic involvement of AAV and need of RRT was associated with higher mortality.Conclusion:AAVs are complex vasculitides with frequent renal involvement. Increased C3 deposition, non-focal histological forms, and <50% normal glomeruli were related to the need for RRT. In turn, the need for RRT, a later age at diagnosis, and systemic involvement were associated with higher mortality. Holistic and multidisciplinary early management of AAVs in experience centers can help improve renal prognosis and decrease mortality.References:[1]Binda et al. ANCA-associated vasculitis with renal involvement. J Nephrol. 2018 Apr;31(2):197-208.[2]Kronbichler et al. Clinical associations of renal involvement in ANCA-associated vasculitis. Autoimmun Rev. 2020 Apr;19(4):102495.Disclosure of Interests:None declared

scholarly journals POS0830 FACTORS INFLUENCING PATIENT-REPORTED OUTCOMES IN ANCA-ASSOCIATED VASCULITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 668.2-669
Author(s):  
S. Monti ◽  
P. Delvino ◽  
C. Klersy ◽  
G. Coppa ◽  
A. Milanesi ◽  
...  
Keyword(s):  
Disease Activity ◽  
Global Assessment ◽  
Patient Reported Outcomes ◽  
Granulomatosis With Polyangiitis ◽  
Disease Duration ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Anca Associated Vasculitis ◽  
Organ Systems ◽  
Damage Accrual ◽  
Patient Reported

Background:Patient-reported outcomes (PROs) are currently poorly integrated in the clinical evaluation of disease activity in patients with ANCA-associated vasculitis (AAV).Objectives:To assess the distribution of the Patient Global Assessment (PtGA) in patients with AAV in stable remission, and to identify correlates of PtGA; to assess the discordance between PtGA score and PhGA.Methods:Patients with a diagnosis of AAV [eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis, microscopic polyangiitis] in stable, complete remission (defined by a BVAS=0) and with a Physician Global Assessment (PhGA)=0 were included. A questionnaire including several aspects of disease captured by PROs was collected. PtGA on a 0-100 mm visual analogue scale (VAS) was assessed, with higher scores representing higher/worse levels of disease activity. Similarly, VAS for pain, chronic damage according to the patient’s opinion, general health (GH), fatigue, and sleep quality were collected. The worst symptom in the patient’s opinion affecting the overall assessment of disease activity was recorded. The Cragg Hurdle model was used to assess the predictors of PtGA.Results:65 patients were included, female 57%, mean age 61±12 years. Mean disease duration at enrollment was 8±6 years. Mean vasculitis damage index (VDI) was 4.4 ±2.3, with 45% of patients having a VDI ≥ 5. Despite having been classified as being in remission, PtGA was elevated in 37% of patients. We explored several correlates of PtGA. Higher degree of damage accrual (VDI) did not influence the patient’s evaluation of current disease activity. Similarly, we did not identify a correlation between older age, educational level, number of organ-systems involved, number of comorbidities, the number of previous major or minor relapses, higher disease duration, nor the type of AAV diagnosis (figure 1, panel A). Only sex significantly correlated with PtGA scores: 19 (51%) of female patients reported an elevated PtGA compared to only 5 (18%) of male (p=0.009). PtGA resulted to be significantly correlated with other (mostly modifiable) PROs including VAS pain, perception of the level of chronic damage accrual, GH, and fatigue (figure 1, panel B). The agreement between patients’ and physicians’ assessments of disease activity was 63%. Patients reported pain, followed by chronic respiratory symptoms to be the worst-experienced ongoing manifestations affecting their evaluation of disease activity.Conclusion:A significant proportion of patients with AAV considered to be in remission by the physician still declares to have persistent aspects of uncontrolled disease. PtGA is significantly influenced by persistent pain and fatigue, which warrant better assessment in the future.Disclosure of Interests:None declared

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