anti-Ro Research Articles

P280 Maternal use of hydroxychloroquine with anti-Ro and or anti-La during pregnancy: a systematic review

Abstract Background/Aims Anti-Ro antibody and, to a lesser extent, anti-La antibody have been associated with fetal heart block during pregnancy. Use of hydroxychloroquine has previously been shown to reduce this risk in pregnancy. According to ACR 2020, all women with positive anti-Ro and or anti-La antibodies are conditionally advised to commence HCQ during pregnancy, but this is based on limited data. We performed a systematic review to evaluate the prevalence of hydroxychloroquine prescription in women with anti-Ro or La antibodies and examine the prevalence of congenital heart block. Methods This was a systematic review of case controlled and observational studies. A PICO model was used to design the research question. Literature searches were initiated based on five keywords: hydroxychloroquine, pregnancy(antenatal), maternal, anti-Ro(SSA), anti-La(SSB). Studies were retrieved from Pubmed, MEDLINE, Mendeley, Cochrane, University College Dublin Library using relevant search strategies from inception to May 2021. The search was restricted to English language articles only. Data were extracted and cross-checked by two reviewers. Results Sixteen studies involving 1,706 anti-Ro and 454 anti-La positive females, resulting in 2,279 pregnancies, were eligible for inclusion in this review. Mean age was 30.7 years at gestation. The prevalence of HCQ prescription in pregnancy in Ro+ women was 32.7%. The most common diagnoses were systemic lupus erythematosus (n = 990) and Sjögren’s syndrome (n = 452), while the remaining women had rheumatoid arthritis, antiphospholipid syndrome, undifferentiated connective tissue disease, scleroderma, autoimmune thyroiditis, dermatomyositis, polychondritis and overlap conditions. Eight studies included asymptomatic antibody carriers (n = 192). The prevalence of congenital heart block in neonates of women on HCQ in pregnancy was 1.8%. Due to issues with data reporting in the included studies, it was not possible to separate out Ro+ women on HCQ from those not on HCQ. For this reason, we could not compare and draw conclusions on the prevalence of CHB in pregnancies of Ro+ women. Conclusion This systematic review highlights the need for additional studies to examine use of HCQ in women positive for Ro or La antibodies. While these antibodies are commonly encountered in SLE and Sjögren’s syndrome, they can occur in other rheumatic diseases and should be screened for to limit risk of CHB. Disclosure S.A. Al Nokhatha: None. S. Maguire: None. R. Harrington: None.

P063 Tracking down autoantibodies

Background/AimsVaccine-associated autoimmunity is not infrequent, pertaining to either the cross-reaction between antigens or the action of adjuvant. This issue is more inexplicable to the COVID-19 vaccine, because of nucleic acid formulation and the hastened development process inflicted by the urgent pandemic condition. Here we are presenting a young patient who developed a significant abnormal autoimmune profile immediately post covid vaccination.MethodsA 31-year-old IT engineer was referred to Rheumatology with post-vaccine arthralgia. He had a history of recent aortic root aneurysm repair after having chest pain on exertion. Echocardiography showed dilated aortic root with significant aortic regurgitation, CT aortogram confirmed spiral type A dissection. He underwent an emergency cardiothoracic surgery in October 2020, followed by an uneventful recovery.He received the first dose of Pfizer COVID-19 vaccine on 2nd February, the very next day he developed painful ankles, knees, left hip, and right shoulder.Blood tests showed elevated CRP of 45, ESR 34, rheumatoid factor positive at 92, anti-CCP >340, ANA 13, ds-DNA 202, U1RNP positive, anti-SM antibody positive, Ro and La antibodies positive, antiJo1 antibody positive, with normal complements.He denied any swelling of the joints. No history of hair loss, photosensitive skin rashes, Raynaud's, sicca symptoms, oro-genital ulceration, or cracking of the skin. There were no constitutional symptoms, chest pain, or bowel issues. He was previously labeled as asthmatic, which is stable after surgery. He doesn’t smoke or drinks alcohol. There was no family history of autoimmune conditions.On examination, he has tenderness across both hands and wrists with palmar erythema but no synovitis. He has painful right shoulder abduction with left hip pain on flexion and extension. Cardiovascular and GI examination was unremarkable apart from sternotomy scar and metallic valvular heart sounds. His dipstick urinalysis was negative for blood and protein. In recent x-rays hands and feet were normal.We agreed on a trial a tapering course of prednisolone started with 20mg daily. Three weeks later in follow-up, he reported partial response to steroids. His inflammatory markers were coming down. We have started azathioprine as a steroid-sparing agent.ResultsThis gentleman with negative autoimmune screening prior to cardiothoracic surgery expressed florid newly detected autoantibodies straightaway after the COVID-19 vaccine. This is suggestive of undifferentiated connective tissue disease with the likelihood of overlap syndrome between rheumatoid arthritis and SLE.ConclusionCOVID-19 vaccination showed a beacon of light to end the pandemic by achieving herd immunity. There is an excusable socioeconomic rush towards mass vaccination without long-term safety analysis, however, it is also crucial that any vaccine licensing process should entail meticulous scrutiny of the human proteome against vaccine peptide sequences. This will minimize the risks of acute autoimmune reactions to inoculation and future chronic autoimmune pathology.Disclosure R. Mazumder: None. K.S. Afridi: None.

P237 New-onset systemic lupus erythematosus following COVID-19 vaccination: a report of two cases

Abstract Background/Aims There is a growing number of reports of new-onset autoimmune disease or complications of underlying autoimmune disorders following COVID-19 infection and vaccination. Methods We describe two cases of systemic lupus erythematosus (SLE) that developed de novo in two female patients shortly after receiving their COVID-19 vaccinations. Results The first case is a 29-year-old female with no prior medical history. One week following her COVID-19 Pfizer/BioNTech vaccination, she developed widespread pruritus, fatigue, myalgia, arthralgia, fever and night sweats. Blood tests showed pancytopenia and she was referred for an urgent haematology opinion due to lymphoma. Positron Emission Tomography/computed tomography (PET/CT) demonstrated widespread lymphadenopathy. Bone and lymph node biopsy showed reactive changes only. Her symptoms progressed with polyarticular inflammatory arthritis, oral ulceration, Raynaud’s, pleuritic chest pain, palmar purpuric rash, and a widespread tender urticarial rash. Further investigations showed low complement C3/C4, anti-double stranded DNA antibody titre (dsDNA) >200 IU/mL, positive Anti-Ro antibody, positive Anti-La antibody, weakly positive anti-RNP antibody and an Anti-C1q antibody >400 units/ml with a urine protein/creatinine ratio (PCR) of 39 mg/mmol. A diagnosis of SLE with urticarial vasculitis was made and she commenced Hydroxychloroquine in addition to weaning prednisolone (60mg). A skin biopsy confirmed lupus vasculitis. Despite high dose prednisolone, urine PCR increased over 2 weeks from 39 to 84 mg/mmol. Renal biopsy demonstrated class 3 lupus nephritis. She was pulsed with 500mg IV methyl prednisolone over 3 days and commenced mycophenolate 1g BD. Within weeks she was in clinical remission. The second case is a 70-year-old female with a past medical history of diverticulosis, uterine fibroids and small hand joint osteoarthritis. She presented with a sudden onset, 6-week history of bilateral symmetrical small and large joint synovitis that developed 8 days following the first dose of the COVID-19 Oxford-AstraZeneca vaccine. Her investigations showed reduced lymphocyte counts (0.9 109/L), raised CRP 26 mg/L and ESR 32 mm/hr. Antinuclear antibodies were weakly positive with a homogenous pattern. DsDNA titre was raised at 175 IU/mL and C4 reduced at 0.14 g/L. There was no proteinuria or any evidence of major internal organ involvement. She was started on a short reducing course of oral prednisolone given the severity of her presenting clinical features. Her symptoms improved, with no recurrence on stopping steroids but she has continued elevation in DsDNA; a conservative management approach is being adopted. Conclusion Both cases met the EULAR/ACR and SLICC classification criteria for SLE. There was a clear temporal association between the onset of SLE symptoms and COVID-19 vaccination. Our cases raise the possible association/causation of SLE following COVID-19 vaccination. Potential mechanisms include immune responses elicited by the COVID-19 vaccination, triggering autoimmunity in genetically predisposed individuals. Further research and data from registries are required. Disclosure J. Beynon: None. M. Alsharkawy: None. L. Sammut: None. J. Ledingham: None.

P232 In the absence of idiopathic inflammatory myopathy, is the presence of anti-transcriptional intermediary factor-1 gamma clinically relevant for malignancy?

Abstract Background/Aims The idiopathic inflammatory myopathies (IIMs) are associated with a number of antibodies which are linked to certain clinical phenotypes. The presence of anti-transcriptional intermediary factor-1 gamma (TIF-1y) is strongly associated with malignancy. However, it is uncertain whether the presence of this antibody, in the absence of IIM, should prompt further investigations to exclude an underlying malignancy. Methods Patients were identified who had undergone myositis immunoblot testing at Liverpool University Foundation Trust laboratories as part of their clinical care between January 2015 and January 2020. Adult patients were included if they tested TIF-1y antibody positive but had no evidence of IIM. Data was collected on patient demographics, whether this was a weak or strong antibody finding and whether there was the presence of other antibodies on the immunoblot panel. The clinical notes were then used to assess whether the patient was known to have a malignancy or went on to develop a malignancy during the follow up period. Results The presence of TIF-1y antibody occurred in 66 patients. Four patients were excluded from analysis, three due to a diagnosis of IIM and one due to a lack of clinical information. The average age was 69 years (range 35 - 88 years) and 59% were female. A strongly positive TIF-1y antibody was found in 19 patients, of which eight were associated with another positive antibody available on the immunoblot panel. There were five malignancies identified, two were historic diagnoses, one patient was found to have a lung malignancy at the time the panel was sent and two patients subsequently developed pancreatic malignancy. A weakly positive TIF-1y antibody was found in 43 patients, of which 16 results were associated with another positive antibody on immunoblot analysis, excluding three patients with a positive anti-Ro antibody. Of 11 patients with malignancy, five had a historic diagnosis and six patients developed malignancy over the follow up period. Conclusion The myositis immunoblot is a useful tool in patients with IIM. However, when used outside of this context can produce a number of positive results, the significance of which are unclear. In the case of TIF-1y and its known association with malignancy, this can lead to unnecessary investigations and increase patient anxiety. This data has obvious limitations as there may have been incomplete information if patients were managed elsewhere and the presence of TIF-1y did not lead to investigation for underlying malignancy in the majority of patients, meaning some cancers may have been present but remained undetected. The results do not suggest the presence of this antibody increases the likelihood of malignancy in patients without IIM but further studies are recommended. Disclosure C.V. Cotton: None. C. Keymer: None. Z. McLaren: None.

Analysis of TNFSF13B polymorphisms and BAFF expression in rheumatoid arthritis and primary Sjögren's syndrome patients.

BackgroundThe increased expression of B cell‐activating factor (BAFF) has been linked to autoantibody production in autoimmune diseases (ADs). The aim of this study was to investigate the association among TNFSF13B gene (OMIM: 603969) single nucleotide polymorphisms (SNPs), TNFSF13B mRNA, and soluble BAFF (sBAFF) expression in patients with rheumatoid arthritis (RA) and primary Sjögren's syndrome (pSS). The diagnostic value of sBAFF also was evaluated by the area under the curve (AUC) of receiver operating characteristic or receptor (ROC) curves.MethodsGenotypes of the TNFSF13B rs9514827 (−2841 T > C), rs1041569 (−2701 A > T) and rs9514828 (−871 C > T) SNPs were determined by PCR‐RFLP assay. TNFSF13B mRNA and sBAFF expression were performed by RT‐qPCR and ELISA, respectively. The study included 320 RA patients, 101 pSS patients, and 309 healthy subjects (HS).ResultsThe rs9514828 T allele and the TAT haplotype were associated with an increased risk to develop RA. In both ADs, the TNFSF13B mRNA levels were increased in comparison with HS. The rs9514828 (−871 C > T) polymorphism was associated with increased gene expression in RA patients. Also, sBAFF levels were higher in both ADs, however pSS patients showed the highest sBAFF levels. sBAFF showed higher diagnostic performance for pSS with an AUC of 0.968, with a similar accuracy of anti‐SSA/Ro antibody diagnosis (AUC = 0.974).ConclusionsOur findings demonstrate that the TNFSF13B rs9514828 (−871 C > T) polymorphism is a risk factor for RA in the western Mexican population. sBAFF levels may be a potential diagnosis biomarker in pSS.

Hypocomplementemia in primary Sjogren’s syndrome: association with serological, clinical features, and outcome

ObjectiveThe aim of the present study was to assess the clinical characteristic of hypocomplementemia (HC) in primary Sjogren’s syndrome (pSS), and to address possible risk factors and the prognosis associated with HC in pSS patients.MethodspSS patients with HC in Hebei General Hospital from September 2016 to March 2019 were retrospectively analyzed and compared to those with normocomplementemia (NC). Logistic regression analysis was used to detect risk factors.ResultsOf the 333 patients with pSS, 84 patients (25.23%) were presented with HC at diagnosis. The presence of hyper-IgG and anti-Ro52 antibodies was significantly more common in patients with HC. In addition to systemic involvement, pSS patients with HC had more hematological, renal, and nervous system involvement, and received more immunosuppressant treatments than NC group (p < 0.05). ESSDAI score was significantly higher in patients with HC (p < 0.05). Multivariate logistic analysis indicated that leukopenia (OR = 2.23) and hyper-IgG (OR = 2.13) were independent risk factors for pSS with HC. In addition, profound CD16/CD56+ NK-cell lymphopenia was found in pSS-HC patients. More pSS patients developed SLE in the HC group than NC group (4.76% vs. 0.80%, p = 0.04) during the follow-up.ConclusionHC was not an uncommon manifestation of pSS and had an independent association with the main clinical and immunological features. Patients with pSS-HC had an increased possibility to develop SLE that required more positive treatment with glucocorticoids and immunosuppressants.Key Points:• Hypocomplementemia had an independent association with the main clinical and immunological features in primary Sjogren’s syndrome patients.• ESSDAI score was significantly higher in patients with hypocomplementemia.• The pSS patients with hypocomplementemia had an increased possibility to develop SLE.

Novel approaches to the surveillance and management of fetuses at risk for anti-Ro/SSA mediated atrioventricular block

Approximately one percent of pregnant women will produce anti-Sjogren's syndrome-related antigen A (anti-Ro/SSA) antibodies. Of these pregnancies, one to three percent will have a fetus that develops atrioventricular (AV) block. Earlier stages of AV block (1° or 2°) may respond to anti-inflammatory treatment, but complete (3°) AV block, which can occur within 24 hours of a normal fetal rhythm, is likely irreversible and carries substantial risk for significant morbidity and for mortality. Emerging data has shown that ambulatory fetal heart rhythm monitoring can detect the transition period from normal rhythm to 2° AV block, the time during which treatment with IVIG and dexamethasone can potentially restore normal sinus rhythm. Weekly or biweekly fetal echocardiograms occur too infrequently to detect this transition period but may still be useful in diagnosing extranodal anti-Ro antibody mediated cardiac disease. In this review, we evaluate the most innovative methods for surveillance and treatment of this disease.

Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies.

To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. We conducted a multicentre, international, retrospective cohort study. 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Investigation and management of stillbirth: a descriptive review of major guidelines.

Stillbirth is a common and devastating pregnancy complication. The aim of this study was to review and compare the recommendations of the most recently published guidelines on the investigation and management of this adverse outcome. A descriptive review of guidelines from the American College of Obstetricians and Gynecologists (ACOG), the Royal College of Obstetricians and Gynecologists (RCOG), the Perinatal Society of Australia and New Zealand (PSANZ), the Society of Obstetricians and Gynecologists of Canada (SOGC) on stillbirth was carried out. Regarding investigation, there is consensus that medical history and postmortem examination are crucial and that determining the etiology may improve care in a subsequent pregnancy. All guidelines recommend histopathological examination of the placenta, genetic analysis and microbiology of fetal and placental tissues, offering less invasive techniques when autopsy is declined and a Kleihauer test to detect large feto-maternal hemorrhage, whereas they discourage routine screening for inherited thrombophilias. RCOG and SOGC also recommend a complete blood count, coagulopathies' testing, anti-Ro and anti-La antibodies' measurement in cases of hydrops and parental karyotyping. Discrepancies exist among the reviewed guidelines on the definition of stillbirth and the usefulness of thyroid function tests and maternal viral screening. Moreover, only ACOG and RCOG discuss the management of stillbirth. They agree that, in the absence of coagulopathies, expectant management should be considered and encourage vaginal birth, but they suggest different labor induction protocols and different management in subsequent pregnancies. It is important to develop consistent international practice protocols, in order to allow effective determination of the underlying causes and optimal management of stillbirths, while identifying the gaps in the current literature may highlight the need for future research.

Recurrent Congenital Heart Block Due to Maternal Anti-Ro Antibodies: Successful Prevention of Poor Pregnancy Outcome with Hydroxychloroquine and Added Dexamethasone

Autoimmune Congenital Heart Block (CHB) is an immune-mediated disease caused by transplacental passage of maternal circulating anti-Ro/SSA and anti-La/SSB antibodies which can bind to fetal cardiac tissue, damaging conduction tissues by inflammation and fibrosis. Approximately 2% of pregnancies with positive anti-Ro antibodies will be complicated by fetal atrioventricular block and the risk of recurrence in subsequent pregnancies is 10 times higher. We report a case of a clinically asymptomatic patient diagnosed with anti-Ro antibodies who had two pregnancies complicated by CHB with different outcomes. Despite preventive treatment with hydroxychloroquine (HCQ) from 6 weeks of pregnancy onward, the fetus developed second to third degree CHB. Dexamethasone was added. The pregnancy evolved to near-term with persistent intermittent CHB. It is not clear how pregnancies with recurrent fetal CHB despite prophylaxis with HCQ should be managed and there is a need for controlled studies to answer the remaining questions in relation to this subject.

Anti-Ro52 antibodies are associated with the prognosis of adult idiopathic inflammatory myopathy-associated interstitial lung disease.

In the present study, we aimed to assess the prevalence and clinical significance of anti-Ro52 antibodies in a cohort of patients with idiopathic inflammatory myopathy-associated interstitial lung disease (IIM-ILD) with different myositis-specific autoantibodies (MSAs). A cohort of 267 IIM-ILD patients, including 62 patients with PM, 126 patients with DM and 79 patients with clinically amyopathic DM (CADM) were retrospectively analysed in this study. Clinical and laboratory findings, pulmonary function tests (PFTs), HRCT patterns and treatment information were compared between patients with and without anti-Ro52 antibodies. The association between prognosis and anti-Ro52 antibodies was also evaluated based on different MSA subgroups. Anti-Ro52 antibodies were more frequent in patients with anti-MDA5 (62.1%, P &lt; 0.01) and anti-Jo1 (64.9%, P &lt; 0.01) antibodies than in those with other MSAs. The proportion of patients with anti-Jo1 antibodies was higher in the anti-Ro52 antibody-positive group than in the anti-Ro52 antibody-negative group. Patients with anti-Ro52 antibodies were more likely to exhibit the Gottron sign than the anti-Ro52 antibody-negative group (P &lt; 0.001). Furthermore, it was a predictive factor for rapid progression interstitial lung disease (RP-ILD) (P = 0.001) and was also associated with a higher mortality rate (log-rank test, P = 0.001). Furthermore, RP-ILD was more frequently exhibited in anti-MDA5- and anti-Ro52-positive patients. Moreover, anti-Ro52 antibody positivity was closely associated with a higher mortality rate in anti-MDA5-ILD patients (log-rank test, P &lt; 0.05). Anti-Ro52 antibodies were highly prevalent in patients with anti-MDA5 and anti-Jo1 antibodies. Within all patients with IIM-ILD, those with anti-Ro52 autoantibodies had a higher frequency of RP-ILD and a poorer prognosis, especially in the anti-MDA5 antibody subgroup.

Clinical characteristics of patients with antisynthetase syndrome and positive anti-Ro52 antibody

To analyze the clinical characteristics of patients with antisynthetase syndrome (ASS) and positive anti-Ro52 antibody. The clinical data of 203 ASS patients admitted to the First Affiliated Hospital of Zhengzhou University from 2017 to 2020 were analyzed retrospectively. Demographics, clinical manifestations, laboratory results, treatment and outcome were collected including data of 18 patients with rapidly progressive interstitial lung disease (RP-ILD). In total, the majority were women (148,72.9%). The average onset age was (51.9±13.3) years. There were 163 (80.3%) patients with positive anti-Ro52 antibody. The positivity in women (77.3% vs. 55.0%, P=0.004) was higher, and the median time from disease onset to diagnosis [4.5 (2.0, 24.0) months vs. 2.0 (1.0, 12.0) months, P=0.024] was longer in patients with positive anti-Ro52 antibody than those negative. Compared with negative patients, patients with positive anti-Ro52 antibody had a higher incidence of interstitial lung disease (ILD) (96.9% vs. 65.0%, P<0.001), arthritis (33.7% vs. 17.5%, P=0.046), and arthralgia (39.3% vs. 20.0%, P=0.022). Higher rate of positve antinuclear antibody (ANA) (85.3% vs. 55.0%, P<0.001), lower rate of positive anti-Jo-1 antibody (32.5% vs. 50.0%, P=0.039), lower albumin level [(34.6±5.2) g/L vs. (37.3±4.7) g/L,P=0.004] and lower lymphocyte counts [(1.4±0.8) ×109/L vs. (1.8±0.8) ×109/L, P=0.014] were more common in patients with positive anti-Ro52 antibody. The presence of anti-Ro52 antibody is associated with a particular phenotype of ASS, leading to common ILD, involvement of joints, high ANA positivity, low albumin and low lymphocyte counts.

POS-534 AUTOIMMUNE PANCREATITIS IN SYSTEMIC LUPUS ERYTHEMATOSUS: CASE REPORT

Systemic lupus erythematosus (SLE) is a chronic, multifaceted autoimmune inflammatory disease with a wide range of clinical presentations resulting from its effect on multiple organ systems. We report a case of SLE associated with autoimmune pancreatitis. In this study, we present a patient diagnosed as having SLE who developed acute auto-immune pancreatitis. This is a 36-year-old woman, with lupus diagnosed since 2009. Initially, the manifestations of her disease were dermatological and articular. Then appeared the renal involvement with a lupus nephropathy class IV at the renal biopsy (PBR). She was previously treated with the NIH protocol then oral prednisolone with improvement in her symptoms. She continued these medications but was lost to follow-up since 2016 and presented after 6 years with pigmented skin lesions on her upper and lower limbs, abdominal pain and distension, vomiting, and an altered general condition. In biology, the patient presented a functional acute kidney failure, an elevated amylasemia (30 times normal), an elevated lipasemia (6 times normal), a normocytic normochromic hemolytic anemia with positive direct coombs test, lymphopenia, a positive immunological assessment (AAN, anti DNA AC, anti Sm, anti SSA, anti RNP), a low C3, a low C4. The patient presented a lupus flare with a SLEDAI score of 6 points: moderate lupus activity. Ultrasound confirmed a large abundance of ascites. Ascites fluid puncture showed an exudate with hyperleukocytosis with predominantly PNN and no germ on direct examination nor on culture.The infectious origin of the pancreatitis was eliminated (CMV, tuberculosis, covid19), as well as the tumoral origin (negative tumor markers, abdominal CT scan showed a swollen pancreas in its caudal portion with loss of physiological lobulations and normal spontaneous density.Necrosis flows difficult to individualize. In addition, no deep neoplastic focus). The autoimmune origin of the pancreatitis due to its lupus attack was retained. She was put on corticosteroids (500mg intravenously for 3 days) then relayed by oral route, albumin infusion, evacuation puncture. The subsequent evolution was marked by the progressive normalization of the pancreatic balance and the slower disappearance of the ascites. Acute pancreatitis is an unusual manifestation of SLE and it should be suspected in any SLE patient with these similar symptoms. In many cases, this complication has been attributed to the drugs administered. In our case, a favorable course of pancreatitis with corticosteroids adds further evidence to the idea that lupus-related pancreatitis is not a side effect of corticosteroid therapy. Moreover, treatment with these medications improves the prognosis.

Treatment of Systemic Lupus Erythematosus with Anti SSA and SSB Positive Pregnancies

Systemic lupus erythematosus (SLE) is autoimmune disease that results in inflammation in various organ systems, frequent recurrences in the clinical course characterized by the production of antibodies against core antigens. Pregnancy will trigger flares. Increased intravascular volume in pregnancy can worsen abnormal heart and kidney function. Increasing the glomerular filtration rate by 50% exacerbated previously stable proteinuria. Pregnancy-induced hypercoagulability increases the risk of thrombosis. Anti-Ro/SSA was found in 35% of SLE patients and anti-La / SSB was found in 15% of SLE patients. The presence of Ro and La antibodies puts the fetus at a risk of developing neonatal lupus erythematosus (NLE) 6-13% and can reach up to 25% if the mother has previously given birth to a child with NLE. Neonatal lupus erythematosus including congenital heart block or cutaneous lupus. Congenital heart block (CHB) is a very serious complication. The American College of Rheumatology (ACR) recommends serial fetal echocardiography from 16 weeks of gestation continuing through to 26 weeks. Pregnancies with grade I and II CHB are treated with oral dexamethasone 4 mg daily because fluorinated glucocorticoids such as dexamethasone and betamethasone can cross the placenta, however, this therapy cannot be given in third-degree heart block. Early diagnosis and therapy will reduce the risk of congenital heart block in neonates after birth.

BULLOUS SUBACUTE CUTANEOUS LUPUS ERYTHEMATOSUS. A CASE REPORT

Lupus erythematosus is an autoimmune collagen disorder with multiple organs involvement. Subacute cutaneous lupus erythematosus represents about 10% of Lupus erythematosus cases and is frequently drug induced. A 83-year-old female patient, otherwise healthy, presented for disseminated annular lesions with mild pruritus lasting for 5 days.The patient denied any new drug usage in the last two months.Laboratory findings showed raised Anti-Ro antibodies and Anti-La (SS-B). The patient received systemic corticotherapy with the remission of the lesions in one month.Subacute cutaneous lupus erythematosus can present with bullous skin lesions and can arise in elderly people without drug usage.

Interstitial Lung Disease Is a Major Characteristic of Patients Who Test Positive for Anti-PM/Scl Antibody.

ObjectiveThis study aimed to analyze the clinical features of anti-PM/Scl antibodies in Chinese patients.MethodWe reviewed the clinical data of anti-PM/Scl antibody-positive patients, including their long-term follow-up.ResultsA total of 30 patients carried anti-PM/Scl antibodies, 21 (70%) were females, and the mean age was 55.4 years, 15 (50%) and 10 (33.3%) patients were positive for anti-PM/Scl-75 and anti-PM/Scl-100, respectively. Fifteen cases (50%) were diagnosed as inflammatory myopathy, namely, 11 dermatomyositis (DM) and 4 polymyositis (PM). Five (16.7%) patients were diagnosed with overlap syndrome, and only one (3.3%) was diagnosed as systemic sclerosis. The other 9 (30%) patients were classified as undifferentiated connective tissue disease. Twenty-six (86.7%) had interstitial lung disease (ILD) and was the sole manifestation in 8 (26.7%) patients, 15 (58.0%) showed non-specific interstitial pneumonia based on high-resolution CT or lung biopsy. The majority of patients (95%) with mild and moderate groups on basis of pulmonary function tests. Compared to the anti-PM/Scl-100 group, the occurrence of clinical characteristics was not significantly different from the anti-PM/Scl-75 group, except the levels of C-reactive protein and erythrocyte sedimentation rate in the anti-PM/Scl-75 antibody-positive group were higher (p < 0.05). All patients with positive Ro-52 antibodies had ILD and were more likely to develop skin rash in the group with Ro-52 (p = 0.024). With a follow-up of the present cohort, 70.8% improved with treatment, but 16.7% of patients are easy to relapse.ConclusionThe anti-PM/Scl antibody occurred frequently in DM/PM patients, ILD was the major clinical feature, especially in patients combined with Ro-52. Some patients may complicate with ILD alone without extrapulmonary manifestations. Anti-PM/Scl antibodies positive patients were responsive to treatment.

Erythema Multiforme Is Not Always Erythema Multiforme

Rowell syndrome (RS) describes the rare presentation of EM like lesions found in association with a diagnosis of lupus erythematosus (LE). Major diagnostic criteria includes LE, EM, and anti-nuclear antibodies. Minor criteria includes of chilblains, anti-Ro/anti-La antibodies, and rheumatoid factor. We present a 26-year-old white male seen in consultation for chronic erythema multiforme (EM) with a duration of 6 months unresponsive to treatment. Prior to our evaluation, he had been seen by another dermatologist and was diagnosed with biopsy-proven EM. His rash did not respond to previous treatment, including corticosteroids and antifungals. Lab results showed a positive ANA and positive anti-Ro antibody. The patient met diagnostic criteria for RS and was started on hydroxychloroquine 200 mg twice daily. Improvement was noticed two weeks after beginning treatment. Our case demonstrates that an atypical presentation of recurrent erythema multiforme, which does not respond to typical EM treatment, should raise a suspicion for RS and prompt screening for autoimmune markers and lupus erythematosus.&#x0D; &#x0D; &#x0D;

Clinical significance of anti-SSA/Ro antibody in Neuromyelitis optica spectrum disorders

Background: Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune inflammatory disease of the central nervous system (CNS), also described as CNS autoimmune astrocytopathy, due to the production of pathogenic antibodies against aquaporin-4 (AQP4) expressed on the foot of astrocytes. NMOSD coexists with autoimmune diseases and related autoantibodies [anti-Sjogren's syndrome A (anti-SSA)/Ro antibody, anti-Sjogren's syndrome B (anti-SSB)/La antibody, anti-nuclear (anti-ANA) antibodies, anti-double-stranded DNA (anti-dsDNA) antibody, anti-thyroglobulin antibody, and anti-thyroid peroxidase antibody].Objectives: No precise conclusion has been drawn on the role of the anti-SSA/Ro antibody in NMOSD. Therefore, the aim of this work was to evaluate whether the anti-SSA/Ro antibody has an impact on the clinical manifestation or prognosis of NMOSD.Methods: Data were retrospectively collected from 102 patients with NMOSD diagnosed by experienced neurologists. The study population was divided into two groups based on the serum anti-SSA/Ro antibody status: NMOSD with or without anti-SSA/Ro antibody. The clinical, neuroimaging and laboratory parameters were compared between the two groups, including the neurological symptoms, MRI results, frequency of systemic autoantibodies, Expanded Disability Status Scale (EDSS), and NMOSD relapse rate. The EDSS and relapse were applied as measures of the NMOSD patient prognostic value. Cox regression analysis was used to evaluate the prognostic impact of anti-SSA/Ro antibody on NMOSD.Results: Among the 102 NMOSD patients, striking differences were observed in the positive rate of AQP4-IgG (89.2% vs. 72.3%, p = 0.046) between those patients with and without the anti-SSA/Ro antibody. In addition, NMOSD patients with anti-SSA/Ro antibody showed the presence of more frequent anti-ANA antibodies (p = 0.002), anti-SSB/La antibody (p < 0.001), anti-dsDNA antibody (p < 0.002), Sjogren's syndrome (SS, p < 0.001) and systemic lupus erythematosus (SLE, p = 0.045). Univariate and multivariate Cox regression analysis were performed to confirm that the anti-SSA/Ro antibody affected the EDSS score and the relapse of NMOSD patients. The analysis of the survival curve revealed that the EDSS score in the NMOSD patients positive for the anti-SSA/Ro antibody reached 4.0 (p = 0.035) and relapsed (p = 0.039) earlier than in the negative group.Conclusion: The anti-SSA/Ro antibody could be associated with disease activity and severe disability in NMOSD.

Proton Pump Inhibitor Induced Subacute Cutaneous Lupus Erythematosus: A Case Series of 7 Patients and Brief Review of Literature.

Background:Subacute cutaneous lupus erythematosus (SCLE) manifests with erythematous, nonscarring, annular, or papulosquamous plaques. Proton pump inhibitors (PPIs) are increasingly being incriminated in its causation, but reports of similar nature from India are lacking.Aims:To describe the characteristics of seven patients with SCLE induced by PPIs and to review the published cases in order to provide a better perspective of the association.Materials and Methods:We describe seven patients of PPI-induced SCLE, seen over a period of 6 years. We also review the literature for additional data on PPI-induced SCLE. The selected publications were reviewed, and relevant clinical and laboratory data were extracted.Results:Of the total seven cases, there were four males and three females with a mean age of 60.2 ± 5.5 years (range 53-70 years). Nine episodes of PPI-induced SCLE were recorded in the seven patients. Of the initial episodes, esomeprazole was implicated in four, pantoprazole in two, and rabeprazole in one patient. Latency period ranged from 2 weeks to 1 year (mean 11.4 ± 16.2 weeks). Morphology was described as annular scaly plaques in six and papulosquamous in one. Antinuclear antibodies and anti-Ro antibodies were positive in all patients. Naranjo probability scale was used in all patients; two were categorized as definite and five as probable. Treatments included drug withdrawal in six patients, topical steroids in one, systemic corticosteroids in all seven, and hydroxychloroquine in one patient, used alone or in combinations. Complete remission was achieved in six cases, while one had partial remission.Limitation:Retrospective nature of this study and limited number of patients.Conclusion:PPIs can trigger SCLE.

Risk Predictors for Lymphoma Development in Sjogren Syndrome - A Systematic Review

Introduction: Sjogren syndrome is a systemic autoimmune disease characterized by lymphocytic infiltrates of salivary and tears glands, leading to oral and ocular dryness and by autoantibody secretion. The association between primary Sjogren syndrome and lymphoma, mostly non-Hodgkin ́s lymphoma or lymphoproliferative disease, has been documented for the past 40 years. The aim of our systematic review was to evaluate the available evidence regarding the identification of serological and hematological predictors for lymphoma in Sjogren syndrome patients. Materials and Methods: Articles were searched and selected through PubMed. Hand search from the relevant journals was also performed. Articles were reviewed and analyzed. The articles were reviewed based on serological and hematological risk predictors such as Anti-Ro antibodies, Anti-La,C3 levels, C4 levels, Cryoglobulins, Lymphopenia, anemia, antinuclear antibodies (ANA), and Rheumatoid Factor positivity. Results: Review of these articles showed significant associations of prognostic factors with development of lymphoma. Within the limitations, Identification of a high-risk phenotype for lymphoma development at the time of Sjogren Syndrome diagnosis has been a major diagnostic challenge over years. Conclusion: All these factors act as a guide to identify patients with high risk at the time of diagnosis leading to an increase in disease free survival rate.