Background. Pulmonary arterial hypertension (PAH) is an independent predictor of death in patients with human immunodeficiency virus (HIV) infection. HIV is the leading cause of PAH (HIV-PAH) worldwide.Aims. We described the characteristics, treatment patterns, and prognosis of a cohort of HIV-PAH patients and compared them with those of an equivalent cohort of patients with idiopathic/familial PAH (IPAH/FPAH).Methods. We retrospectively analysed and compared the demographic, clinical, and treatment data from patients with HIV-PAH and those with IPAH/FPAH in the Spanish PAH registry (REHAP) from 1998 to 2018. The HIV-PAH overall survival (OS) rate up to 5 years was compared to the age-and sex-matched IPAH/FPAH population. Changes in treatment patterns in patients with HIV-PAH after 2010 and their effects on OS were also analysed.Results. Compared to those with IPAH/FPAH (n = 739), patients with HIV-PAH (n = 132) were younger, mainly men, and had a better functional status. The clinical presentation, haemodynamics, and respiratory function were similar between the groups. Parenteral drug use was the most common mode of HIV transmission. Approximately 11% of patients with HIV-PAH did not receive PAH-targeted therapy. The age-and sex-adjusted 5-year OS rate from diagnosis was 74.0% for patients with HIV-PAH and 68.7% for those with IPAH (p < 0.159). During/after 2010, 23% of patients with IPAH/FPAH received upfront dual oral combination, while oral monotherapy remained the main first-line treatment in patients with HIV-PAH. The overall OS rate remained stable. Conclusions.Patients with HIV-PAH were predominantly young men. The short-term prognosis is similar to that of age-and sex-matched patients with IPAH/FPAH, despite a better functional status. Oral monotherapy remains the preferred firstline treatment in the current cohorts.
Background:Sjögren’s syndrome (SjS) is a systemic autoimmune disease with a broad clinical presentation from dry syndrome to systemic extraglandular manifestations. The diagnosis can be complex since none of the markers, except anti-Ro, is sufficiently sensitive or specific. Although, minor salivary glands biopsy (MSGB), Schirmer’s test and unstimulated whole salivary flows (UWSF) are the hallmark for the diagnosis of this entity, its use is not widespread in some centers.Objectives:The aim of the study was to analyze the usefulness and safety of the diagnostic protocol for the classification of SjS and the immunological and analytical markers in dry syndrome due to SjS.Methods:Prospective observational study of a cohort of patients with sicca syndrome from a reference center. The diagnostic protocol (Schirmer’s test, UWSF and minimally invasive MSGB) was applied in the same consultation. Demographic, clinical, analytical and histological data were reviewed.Results:Over a period of 6 months, 48 patients with dry syndrome were analyzed, of which 39 women (81.2%). The main suspicion was SjS (39), followed by sarcoidosis (3), IgG4-related disease (2) and other diagnoses (4). The mean age was 59.1±4.4 years. Almost half (45.8%) reported xerostomia and 41.6% xerophthalmia. Recurrent parotidomegaly was described in 6 patients (12.5%) and arthralgias in 12 (25%). Immunologically, 23 (47.9%) presented anti-nuclear antibodies, 13 (27.1%) anti-Ro, 4 (8.3%) anti-La, 12 (25%) rheumatoid factor and 15 (31.2%) low C4. Schirmer test was positive in 32 patients (66.7%), UWSF in 22 (45.8%) and 9 (18.8%) had a focus score ≥1, although 16 (33.3%) had focal lymphocytic sialadenitis in the MSGB. A total of 21 (43,8%) patients were classified according to the 2016 ACR/EULAR criteria. 12 (57.1%) were seropositive SjS and 9 (42.9%) seronegative SjS. MSGB sensitivity was 71% and specificity 96%. Patient reported symptoms were unhelpful to differentiate SjS from other causes of dry syndrome. The number of protocols needed to diagnose a SjS was 2.28 (5.33 in seronegative SjS). Complications associated with the procedure were low (1 of 48) and mild (self-limited paraesthesia). Patients with SjS, unlike those with dry syndrome of another etiology, had more anemia (p<0.001), lymphopenia (p=0.022), ESR (p=0.030), beta-2 microglobulin (p=0.011), ANA (p<0.001), anti-ENA (p=0.006), anti-Ro (p<0.001), low C4 (p<0.001) and hypergammaglobulinemia (p=0.002).Conclusion:Immunological and histological manifestations were more predictive than clinical ones to differentiate SjS from other causes of dry syndrome. MSGB is a simple, sensitive, specific and safe procedure. The application of the diagnostic protocol (Schirmer test, UWSF and MSGB) allowed to standardize the classification of SjS and increased the diagnosis of patients with seronegative SjS.References:[1]Ramos-Casals M, Brito-Zerón P, Bombardieri S On behalf of the EULAR-Sjögren Syndrome Task Force Group, et al. EULAR recommendations for the management of Sjögren’s syndrome with topical and systemic therapies.Annals of the Rheumatic Diseases2020;79:3-18.[2]Guellec D, Cornec D, Jousse-Joulin S, et al. Diagnostic value of labial minor salivary gland biopsy for Sjögren’s syndrome: a systematic review.Autoimmun Rev. 2013;12(3):416–420.Disclosure of Interests:None declared
Background:Myositis-specific antibodies (MSA) are highly specific and useful to classify patients as having syndromes with distinct clinical features and prognosis. MSA are almost always mutually exclusive and quite specific, adding value as a useful biomarker for diagnosis. Although individual autoantibodies aren’t sensitive enough to detect the full spectrum of idiopathic inflammatory myopathies (IIM), the sensitivity of a myositis panel is increasing as more autoantibodies are discovered, and as better assays become available.Objectives:We aimed to analyze the usefulness of a myositis-specific immunoblot for the diagnosis of IIM in a hospital cohort from January 2019 to December 2020. We also seek to correlate immunological findings with the risk of associated interstitial lung disease (ILD), cancer, or death.Methods:Retrospective single-center observational study conducted in a Spanish tertiary hospital. In patients with high clinical suspicion of IIM, a myositis immunoblot was performed including Jo1, PL-7, PL-12, EJ, SRP, Mi2, Ku, MDA-5, TIF1-γ, HMGCR, PM-Scl and Ro52 antibodies. The demographic characteristics, the risk of ILD, cancer and death were analyzed.Results:In a cohort of 313 patients with high suspicion of IIM, 87 patients (27.8%) presented a positive MSA (MSA+ve). The mean age at diagnosis was 56.7±16.9 years, with no significant differences between MSA+ve and MSA-ve (p=0.597). Most of the patients were women with significant differences between both groups (80.5% MSA+ve vs 68.1% MSA-ve, p=0.030).IIM were classified as antisynthetase syndrome (ARS) (38%), dermatomyositis (DM) (31%), overlap myopathy (OM) (16.9%) and necrotizing myopathy (NM) (14.1%) according to the manifestations and MSA found (Jo1, PL-12, PL-7, EJ in ARS; Mi-2, MDA-5 and TIF1-γ in DM; Ku and PM-Scl in OM; HMGCR and SRP in NM). The most frequent MSA were anti-Jo1 (16.9%), TIF1-γ (15.5%), Ku (12.7%), Mi-2 (9.9%), PL-7 (9.9%), HMCGR (8.5%), PL-12 (7%), MDA-5 (5.6%), SRP (5.6%) and EJ (4.2%). The presence of Ro52 associated with other MSA was found in 20 patients (22.9%).ILD was the most frequent manifestation (45.2% of the MSA+ve). Non-specific interstitial pneumonia (NSIP) was the most frequent ILD (39.5%), followed by usual interstitial pneumonia (34.2%). The main risk factors associated with IIM-ILD were some subtypes of the MSAs (p<0.001), the association of Ro52 (p<0.001), and older age (p=0.027). Among the IIM, ARS and OM (p<0.001) were more frequently associated with IIM-ILD. The MSAs most associated with IIM-ILD were Jo1, PL-7, PM-Scl, Ku and SRP (p<0.001).Cancer was found in 9.6% of MSA+ve patients. The most frequent tumors were gynecological (37.5%), followed by gastrointestinal (25%) and breast cancer (12.5%). Factors associated with cancer were age (p=0.010), TIF1-γ (p<0.001), SRP (p=0.004), PL-12 (p=0.013), PL-7 (p=0.047) and HMGCR (p=0.027).The mortality of these patients was 3.5%. There were no differences regarding MSA+ve/-ve (p = 0.911). However, MDA-5 (p=0.033) and older age (p=0.001) were associated with higher mortality. There were no significant differences between the IIM classifications, the associated SAD, the presence of cancer or ILD. However, longer follow-up periods and future studies are necessary to confirm these results.Conclusion:The use of a myositis blot allowed classifying, stratifying the risk of ILD, the risk of cancer and the risk of mortality in IIM. IIM-ILD was the most frequent complication, usually manifested as NSIP. The associated risk factors were ARS, OM, some MSAs, Ro52+ and older age. Cancer was a serious and frequent manifestation in these patients, especially in patients with TIF1-γ and other MSAs, so it is essential to know the risk factors and perform an early screening, especially in older patients.A better knowledge of the serological profiles of IIM will provide more individualized approaches and better risk stratification, helping in the management and treatment of these patients.References:[1]Satoh et al. Clin Rev Allergy Immunol. 2017 Feb;52(1):1-19.[2]Betteridge et al. J Intern Med. 2016 Jul;280(1):8-23.Disclosure of Interests:None declared
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
Background:Antiphospholipid syndrome (APS) is a systemic autoimmune disease, associated with a hypercoagulable state and fetal loss and with other clinical manifestations including cardiac involvement. APS occurs as a primary disorder (PAPS) or secondary to another autoimmune disease (SAPS). Due to its vascular nature, various organs and tissues may be affected, including the cardiac system. Cardiac manifestations of APS are valve abnormalities (valve thickening and vegetations), occlusive arterial disease (atherosclerosis and myocardial infarction) and pulmonary hypertension (PH).Objectives:To assess the prevalence of non-myocardial involvement (valvulopathy and pulmonary hypertension) in a cohort of patients with antiphospholipid antibodies (aPLs).Methods:Retrospective observational study in a Spanish reference center for systemic autoimmune diseases. All patients with aPLs and performed transthoracic echocardiogram (TTE) were included in the study. Patients were divided between PAPS, SAPS and aPLs carriers. A cohort of 50 patients with systemic lupus erythematosus (SLE) without aPLs was used as a control. Anti-cardiolipin, anti-B2GP1 and lupus anticoagulant antibodies were determined by standard techniques.Results:A total of 220 patients were reviewed. 145 (65.9%) were female. The mean age was 42 years. Among all patients with aPLs, 102 were PAPS, 73 SAPS, and 45 asymptomatic carriers (silent APS). Patients with aPLs, unlike patients with SLE without aPLs, presented more often pathological TTE (114 patients, 52%) (p = 0.02), with more valvular involvement (87, 39%) (p = 0.005) and pulmonary hypertension (21, 9.5%, p = ns). Valve involvement was identified in 99 patients: 45 in PAPS, 27 in SAPS, 14 in aPLs carriers and 13 in the SLE without aPLs, these differences being statistically significant (p = 0.002). Valvulopathy was asymptomatic in the majority of patients but required valve replacement in two patients. Mitral valve was the most affected, especially in the form of insufficiency (57%), followed by aortic valve, combined mitral and aortic valve, and less frequently the pulmonary valve alone (3 cases).aPLs globalPAPSSAPSaPLs carriersSLE w/o aPLsTotal220 (100%)102 (46.4%)73 (33.2%)45 (20.4%)50 (100%)Men75 (34.1%)41 (40.2%)23 (31.5%)11 (24.4%)6 (12%)Women145 (65.9%)61 (59.8%)50 (68.5%)34 (75.6%)44 (88%)Conclusion:Subclinical valve involvement was very common in patients with APS. There was no correlation with other clinical manifestations of APS nor were other risk factors identified. PH was less frequent than valvular involvement in patients with APS. However, despite not being statistically significant, close to 10% of patients with APS had PH compared to 6% of patients without APS.aPLs globalPAPSSAPSaPLs carriersSLE w/o aPLsTTE performed220 (100%)102 (46.4%)73 (33.2%)45 (20.4%)50 (100%)Pathological TTE114 (52%)16 (32%)p=0.02Valvular involvement87 (39%)45 (20.4%)27 (12.3%)14 (6.3%)13 (26%)p=0.005Pulmonary hypertension21 (9.5%)3 (6%)p=nsEvery patient with APS should have an echocardiogram in the initial study protocol in order to rule out both valvulopathy and pulmonary hypertension. This could modify the patient’s management both in the short and long term, as well as the prognosis.References:[1]Radin M, Ugolini-Lopes MR, Sciascia S, Andrade D. Extra-criteria manifestations of antiphospholipid syndrome: Risk assessment and management.Semin Arthritis Rheum. 2018;48(1):117–120.[2]Tenedios F, Erkan D, Lockshin MD. Cardiac involvement in the antiphospholipid syndrome.Lupus. 2005;14(9):691–696.[3]Kolitz T, Shiber S, Sharabi I, Winder A, Zandman-Goddard G. Cardiac Manifestations of Antiphospholipid Syndrome With Focus on Its Primary Form.Front Immunol. 2019;10:941.Disclosure of Interests:None declared
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