Case Report: Refractory Immune-Mediated Necrotizing Myositis and Limited English Proficiency

Statement of Significance: We report the case of a 76-year-old Spanish-speaking patient with a three-year history of statin induced immune-mediated necrotizing myositis (IMNM) who presented with worsening symptoms and increasing creatinine kinase levels despite escalating treatment strategies. IMNM is a rare and challenging diagnosis. This case report details a myositis flare refractory to first and second-line therapies. Our report also examines limited English proficiency as a structural barrier to care in the United States, particularly in the setting of visits conducted via telehealth modalities. Purpose: To report a rare presentation of statin-induced IMNM and the clinical impacts of language barriers and telehealth. Methods: Case report. Results: A 76-year-old male with a three-year history of statin-induced IMNM presented for follow-up to the rheumatology clinic. He reported worsening weakness after beginning leucovorin to mitigate side effects ascribed to methotrexate therapy. He had previously achieved baseline strength and normal creatinine kinase (CK) levels with a regimen of weekly methotrexate and monthly infusion of intravenous immunoglobulin (IVIG). His decline in condition appeared to result from inappropriate medication scheduling due to a language barrier. The patient was taking weekly leucovorin on the day before his weekly dose of methotrexate, thus mitigating the efficacy of methotrexate. However, his condition continued to decline with three months of the recommended treatment schedule. The patient was then switched to mycophenolate mofetil as an alternative immunosuppressant. This therapy has demonstrated benefit thus far and provided the patient with symptomatic relief.

Fulminant necrotizing streptococcal myositis with dramatic outcome – a rare case report

Necrotizing myositis represents a rare, aggressive form of bacterial-induced soft tissue necrotizing infection. We present a fulminant case of a 44-year-old patient with a necrotizing soft tissue infection and a history of rheumatoid arthritis transferred to our service, Cluj-Napoca Emergency County Hospital, from a local hospital where he had been admitted two days before with chills and light-headedness after an accidental minor blunt trauma in the right thigh region. After admission to our hospital and first assessment, broad spectrum antibiotherapy was started with Meropenem, Vancomycin and Metronidazole along with surgical debridement. The evolution was fulminant with rapid development of multiple organ dysfunction syndrome, therefore he was transferred to the intensive care unit, intubated, and started the volemic resuscitation and vasopressor therapy. The blood culture was positive for group A beta-hemolytic streptococcus (GAS) and high dose Penicillin G was added to the therapeutic scheme. Despite all efforts, the patient developed disseminated intravascular coagulation syndrome and died in the next hours. The clinical picture together with the findings from the autopsy were suggestive for a streptococcal toxic shock syndrome developed as a complication of GAS induced necrotizing myositis.

Autoimmune necrotizing myositis. Presentation of a clinical case

Inflammatory myopathies (IM) or myositis are a heterogeneous group of muscle diseases of rare occurrence. Such diseases are characterized by inflammation of the different components of muscle tissue, which can occur either in isolation or, more commonly, as part of a systemic disorder. Immune-mediated necrotizing myopathies (IMNM) are a type of autoimmune myopathy characterized by proximal muscle weakness, myofiber necrosis with minimal inflammatory cell infiltrate on muscle biopsy and infrequent extramuscular involvement. Even though there are clinical and histopathological similarities. The spectrum of inflammatory myopathies is considerably variable. Therefore, the performance of complementary studies is essential for the proper identification of the IM subtype to contribute accurately on treatment so determine the better prognosis). The present article shows the case of a young 29 years old, with no personal and family history background of autoimmune disease and no relevant pathological back-ground. The patient consulted the medical ward of the Institution with pain, functional impairment of upper and lower extremities, muscle weakness mainly located in the pectoral girdle area and, although to a lesser degree, in the pelvic girdle area. It was also associated with asthenia, tendency to drowsiness and hyporeactivity.

AB0296 PASSIVE TRANSFER OF ANTI-SSA, ANTI-Ro52, AND ANTI-MITOCHONDRIAL M2 FROM INTRAVENOUS IMMUNOGLOBULIN PRODUCTS TO PATIENTS WITH RHEUMATIC DISEASES

Background:Passive transfer of ANA and anti-SSA has been reported in patients with common variable immunodeficiency disorder who received intravenous immunoglobulin (IVIG). IVIG is also recommended to treat some special or life-threatening rheumatic diseases.Objectives:This study was aimed to explore whether any extractable nuclear antibodies (ENAs) were transferred to these rheumatic patients who received IVIG therapy.Methods:IVIG products of three batches were tested for ANA by using indirect immunofluorescent assay, and for ENAs by using line immunoassay (LIA) and chemiluminescence immunoassay (CLIA). These IVIG products were administrated to rheumatic patients at a dose of 20g/d×3 days (day1 to day3). Serum samples of these patients before IVIG (day0) and after IVIG (day4, day8, day10, day12, and more than one month) were tested by using LIA and CLIA. Anti-SSA was also detected using ELISA.Results:In these IVIG products, ANA was positive at a titer of 1:640 (cytoplasmic speckled) and 1:80 (speckled). Among 14 types of ENAs that could be tested using LIA, anti-SSA, anti-Ro52, anti-mitochondrial M2, and anti-centromere B antibodies were clearly detectable in IVIG products (Table 1). Likewise, another assay CLIA also detected the same positive autoantibodies in these products. LIA showed the highest concentration in anti-mitochondrial M2, while CLIA showed the highest concentration in anti-mitochondrial M2 and anti-Ro52. One 31-year-old male patient who was diagnosed as SLE (Figure 1) and one 72-year-old male patients who was diagnosed as necrotizing myositis received these IVIG products. Anti-SSA, anti-Ro52, anti-mitochondrial M2, but not anti-centromere B, were positive in the day4 serum samples, although all of these antibodies were negative at baseline (day0). The concentration of these antibodies decreased gradually as days passed and became undetectable around one month after IVIG.Table 1.The concentration of autoantibodies in intravenous immunoglobulin productsanti-SSAanti-Ro-52anti-mitochondrial M2anti-centromere BCut-offLIA(grey value)20±328±369±1019±4≥11CLIA (U/ml)333±107444±86434±66390±89>20ELISA (U/ml)90±13NANANA>20LIA, line immunoassay; CLIA, chemiluminescence immunoassay; ELISA, enzyme linked immunosorbent assayConclusion:This study preliminarily reported transient positivity of anti-SSA, anti-Ro52, and anti-mitochondrial M2 in rheumatic patients maybe because the passive transfer of these antibodies from IVIG products to the patients, although the potential influence of this transfer on the rheumatic diseases remained unknown.Figure 1.The concentration of autoantibodies in a 31-year-old male SLE patient receiving intravenous immunoglobulin at a dose of 20g/d×3 days (day1 to day3). Serum samples of these patients before IVIG (day0) and after IVIG (day4, day8, day10, day12, and day51) were tested by using line immunoassay (LIA) and chemiluminescence immunoassay (CLIA). Anti-SSA was also detected using ELISA. The horizontal red lines were the corresponding cut-off values of each assay.Disclosure of Interests:None declared

AB0430 CONTRIBUTION OF SCLERODERMA/MYOSITIS-RELATED ANTIBODIES DETECTED BY IMMUNOBLOT TO THE DIAGNOSIS OF SYSTEMIC AUTOIMMUNE RHEUMATIC DISEASES IN 134 PATIENTS FROM A SINGLE REFERRAL CENTER

Background:Immunoblot assays are increasingly used in clinical practice as part of the diagnostic armamentarium of systemic autoimmune rheumatic diseases (SARDs).Objectives:To assess the contribution of an extended scleroderma/myositis-related antibodies (Ab) determination by immunoblot to the diagnosis of patients with SARDs.Methods:We reviewed all medical records of patients with positive scleroderma/myositis-related Ab line blot determinations (Euroimmune AG, Lübeck, Germany) in our center from November 2017 to September 2020. These assays were requested due to high suspicion of SARDs in patients presenting with non-specific symptoms.Results:134 patients (37men/97women; mean age 59.6 ± 14.8 years) were positive for at least 1 Ab, 25 of them were positive for 2 Abs. Main clinical features at the time of immunoblot requests were: arthralgia/arthritis (n=88), Raynaud’s phenomenon (n=59), rash (n=27), sicca syndrome (n=14.9%), myopathy (n=18). During follow-up, 28 patients were diagnosed with undifferentiated connective tissue disease (UCTD), 26 scleroderma, 23 overlap myositis, 18 interstitial pneumonia with autoimmune features (IPAF), 8 other inflammatory diseases, 8 Sjögren’s syndrome, 7 systemic lupus erythematosus, 5 dermatomyositis, 1 necrotizing myositis. In 10 patients the diagnosis of SARD was finally ruled out (Figure 1). Interstitial lung disease (ILD) was present in 50 patients, being particularly frequent in those with anti-PL12, anti-PL7 and anti-MDA5 Abs. Cancer was detected in 9 (6.7%) patients, 6 of them were anti-Ro52 + (Table 1).Conclusion:Immunoblot assays are of great help in the diagnosis of patients with high clinical suspicion of SARDs. While some Abs, such as anti-Ro52, anti-Ku and anti-PMScl75/100, remain to be nonspecific, other Abs including anti-PL12, anti-PL7 or anti-MDA5 are particularly helpful in detecting SARDs patients with associated ILD.References:Table 1.Mi-2 (n=5)PL-7(n=6)PL-12(n=4)Jo-1(n=6)MDA5(n=1)antiRo52(n=57)SRP (n=3)Scl-70(n=12)CENP(n=14)Th(n=2)Ku(n=14)Fibrilarina (n=2)PM-Scl75/100 (n=23)NOR90(n=8)RNA pol(n=2)ILD04 (66.7)4 (100)3 (50)1 (100)19 (33.3)07 (58.3)1 (7.1)07 (50)1 (50)9 (39.1)5 (62.5)0Cancer1 (20)00006 (10.5)1 (33.3)0001 (7.1)0000Disclosure of Interests:None declared

Delayed-onset Necrotizing Myositis following COVID-19 Infection

As the numbers of cases of COVID-19 continue to rise, the heterogeneity of its clinical manifestation continues to increase. Here, we describe a case of delayed-onset, biopsy-proven necrotizing myositis following infection with SARS-CoV-2.

Statin related musculoskeletal complications; Necrotizing Autoimmune Myositis… more than Myalgia.

Statins are widely prescribed and well tolerated with most side effects now considered a nocebo effect. Occasionally, statins can be associated with immune mediated necrotizing myositis that is both difficult to diagnose and treat. Aggressive immunosuppressive therapy is the best recognized method of treatment of this complication.