Hemoptysis and a cardiac murmur: is it primary or secondary antiphospholipid syndrome?

Lupus ◽  
2019 ◽  
Vol 28 (14) ◽  
pp. 1712-1715
Author(s):  
T Kolitz ◽  
O Fruchter ◽  
L Sasson ◽  
Y Geva ◽  
O Moreh-Rahav ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Infectious Agent ◽  
Intravenous Immunoglobulins ◽  
Clinical Findings ◽  
High Dose ◽  
Cardiac Murmur ◽  
Elective Procedure ◽  
Work Up

Endocarditis is most frequently infective in origin, and thus, when a patient presents with a clinical picture suggestive of endocarditis, an extensive work up aimed at finding the infectious agent is warranted. Among systemic lupus erythematosus (SLE) patients, cardiovascular disease is prevalent in more than 50% of patients including valvular disease and non-infective endocarditis, known as Libman–Sacks (LS) endocarditis. The prevalence of LS syndrome among SLE patients with secondary antiphospholipid syndrome (APS) is higher than in SLE without APS. Here, we present a case of a patient diagnosed with primary APS who presented with hemoptysis and a cardiac murmur. The diagnosis of SLE was established following the findings of non-infective verrucous vegetations together with diffuse alveolar hemorrhage (DAH). Treatment with high-dose corticosteroids and intravenous immunoglobulins yielded substantial resolution of the vegetations and regression of the DAH. Hence, aortic valve replacement was successfully performed as an elective procedure and without any postoperative complications. The patient is in remission after a 6-month follow-up. The clinical findings of DAH and double valve non-infectious endocarditis prompted the diagnosis of SLE with secondary APS.

scholarly journals AB0601 RAPID AND SUSTAINED EFFICACY OF AN INDUCTION TREATMENT WITH A TRIPLE THERAPY INCLUDING HIGH-DOSE INTRAVENOUS IMMUNOGLOBULINS, METHOTREXATE AND GLUCOCORTICOIDS IN ANTI-3-HYDROXY-3-METHYLGLUTARYL-COENZYME A REDUCTASE MYOPATHY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1597.1-1597
Author(s):  
E. Treppo ◽  
M. Infantino ◽  
M. Benucci ◽  
V. Ravagnani ◽  
B. Palterer ◽  
...  
Keyword(s):  
Triple Therapy ◽  
Antibody Titer ◽  
Intravenous Immunoglobulins ◽  
High Dose ◽  
Sustained Remission ◽  
Red Rice ◽  
Gradual Improvement ◽  
High Doses ◽  
High Dose Ivig

Background:Anti-3-hydroxy-3-methylglutaryl-coenzime A reductase (HMGCR) myopathy is a new entity, which has been clearly associated to statin use, even if it can be diagnosed in patients without a history of exposure to statin or even in the childhood (1).Objectives:The aim of the study is to describe the efficacy of a triple therapy regimen consisting in high-doses of intravenous immunoglobulins (IVIG), methotrexate (MTX), and glucocorticoids (GC) in 16 patients with Anti-HMGCR myopathy enrolled in 6 specialized centres.Methods:A total of 16 patients with anti-HMGCR myopathy (7 females; 9 males) were collected. Mean (±standard deviation) age at the onset of disease was 72.4±10.3 years old. All patients were diagnosed having anti-HMGCR myopathy [anti-HMGCR antibodies were measured by chemiluminescence assay (BioFlash, Inova, CA)] (2). Median follow-up was 29.5 months (interquartile range: 15.75-60 months). Anti-HMGCR antibodies were available in the follow-up in 8/16 patients.Results:Thirteen out of 16 patients (81.3%) had been exposed to statin (1/13 to red rice), 3/16 (18.7%) were not exposed. As induction therapy, 11/16 patients have been treated with triple therapy (high-dose IVIG, MTX and GC), 2/16 with double therapy (high-dose IVIG and GC), 2/16 have been treated with GC alone, the patient exposed to red rice resolved only with red rice suspension. Clinical remission and normalization of CPK values within month +24 were obtained in all the patients. All the patients were in remission at the last follow-up. Gradual improvement started soon from the first month, and among the 13 patients treated with an aggressive immunosuppresssive therapy including IVIG (13/13), GC (13/13) and methotrexate (11/13), 9/13 normalized the CPK value within 6 months. Clinical and laboratory response was accompanied by significant decrease or normalization of the anti-HMGCR antibody titer. All the patients were either not taking GC (56.3%), or were taking low doses of GC (43.7%) at the last follow-up. Four patients had stopped GC within 6 months. No serious side effects were recorded. After persistent remission, a maintenance immunosuppressive therapy was then administered. Only 3 relapses in 3 different cases were recorded, all of them during drug-free remission in long-term follow-up. Reinduction was again effective in all.Conclusion:Anti-HMGCR myopathy is a rare and serious myopathy which usually affects older people during statin treatment. After statin suspension, a rapid and sustained remission can be achieved by induction with a triple aggressive therapy consisting in medium-to high doses of GC, high-dose IVIG, and MTX (3). GC should be tapered as soon as possible. Relapse appears infrequent during maintenance treatment. Monitoring anti-HMGCR antibody titer may be clinically relevant.References:[1]AL Mammen et al. N Engl J Med. 2016;374:664-9[2]Musset L et al. Autoimmun Rev. 2016;15:983-93.[3]Aggarwal A et al. Scand J Rheumatol. 2019; 1-7.Acknowledgments:We thank MD Francesca Grosso and MD Valentina Mecheri from the University of Florence, MD Angela Zuppa and MD Chiara De Michelis, from San Martino Hospital, Genova, for their valued collaboration in data collectionDisclosure of Interests:Elena Treppo: None declared, Maria Infantino: None declared, Maurizio Benucci: None declared, Viviana Ravagnani: None declared, Boaz Palterer: None declared, Marina Grandis: None declared, Martina Fabris: None declared, Paola Tomietto: None declared, Mariangela Manfredi: None declared, Arianna Sonaglia: None declared, Maria Grazia Giudizi: None declared, Francesca Ligobbi: None declared, Daniele Cammelli: None declared, Paola Parronchi: None declared, Salvatore De Vita Consultant of: Roche, GSK, Speakers bureau: Roche, GSK, Novartis, Luca Quartuccio Consultant of: Abbvie, Bristol, Speakers bureau: Abbvie, Pfizer

scholarly journals Anecdotal Response to Amphotericin in a Patient with Probable Susac Syndrome: Implications in the Pathogenesis

2019 ◽  
Vol 32 (11) ◽  
pp. 727
Author(s):  
Raquel Gil-Gouveia ◽  
Natália Marto ◽  
Pedro Vilela ◽  
Ana Catarino
Keyword(s):  
Hearing Loss ◽  
Fungal Infection ◽  
Infectious Agent ◽  
Brain Biopsy ◽  
High Dose ◽  
Radiological Findings ◽  
Susac Syndrome ◽  
Infection Treatment ◽  
Immune Mediated

Susac syndrome is a rare, probably immune-mediated endotheliopathy presenting with encephalopathy, sensorineural hearing loss and retinal arterial occlusions. A 33-year-old female with Susac syndrome was worsening despite high-dose steroids so a brain biopsy was performed which suggested a possible fungal infection. Treatment with amphotericin B resulted in prompt reversal of symptoms and radiological findings, and no further symptoms occurred during 8 years of follow-up. A diagnosis of fungal infection was not confirmed. The etiology of Susac syndrome is unknown and this anecdotal observation suggests that an infectious agent susceptible to amphotericin might have caused or triggered Susac syndrome in this patient.

scholarly journals Severe refractory thrombocytopenia in a woman positive for coronavirus disease 2019 with lupus and antiphospholipid syndrome

Lupus ◽  
2020 ◽  
Vol 29 (11) ◽  
pp. 1472-1474 ◽  
Author(s):  
Alina Hayden ◽  
Aishwarya Vyas-Lahar ◽  
Vincent Rella ◽  
Alla Rudinskaya
Keyword(s):  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Platelet Transfusion ◽  
Scattered Data ◽  
High Dose ◽  
Systemic Lupus ◽  
Current Case ◽  
Inflammatory State ◽  
History Of ◽  
New Challenges

The coronavirus disease 2019 (COVID-19) pandemic has created new challenges that necessitate prompt responses in unexpected clinical situations. Multiple extrapulmonary manifestations and complications of COVID-19 have already been described, but only scattered data are present on immunologic manifestations. We present a case of severe refractory thrombocytopenia in a 51-year-old woman with a history of long-standing systemic lupus erythematosus and antiphospholipid syndrome who presented with hemoptysis in the setting of COVID-19 infection. The patient failed to respond to initial treatment with intravenous immunoglobulin, high-dose steroids, and platelet transfusion, but responded to eltrombopag, with prompt improvement of a platelet count. The current case report provides clinical data of relevance to the largely unexplored question of the immunologic complications of COVID-19 in patients with a pre-existing inflammatory state.

Long-term follow-up of benign positional vertical opsoclonus in infants: retrospective cohort

2017 ◽  
Vol 102 (6) ◽  
pp. 757-760 ◽  
Author(s):  
Amir Sternfeld ◽  
Daniella Lobel ◽  
Hana Leiba ◽  
Judith Luckman ◽  
Shalom Michowiz ◽  
...  
Keyword(s):  
Retrospective Cohort ◽  
Clinical Investigation ◽  
Clinical Findings ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Ocular Symptoms ◽  
Long Term Follow Up ◽  
Work Up

Background/AimsBenign positional vertical opsoclonus in infants, also described as paroxysmal tonic downgaze, is an unsettling phenomenon that leads to extensive work-up, although benign course has been reported in sporadic cases. We describe long-term follow-up of a series of infants with the phenomenon.MethodsThis retrospective cohort included all infants diagnosed with rapid downgaze eye movement in 2012–2015 and followed until 2016. The databases of two medical centres were retrospectively reviewed. Benign positional vertical opsoclonus was diagnosed based on clinical findings of experienced neuro-ophthalmologists. Data were collected on demographics, symptoms and signs, neuro-ophthalmological and neurological evaluations, and outcome. Imaging studies were reviewed. Main outcome measures were long-term outcome and findings of the thorough investigation.ResultsThe cohort included six infants. All infants were born at term. Age at presentation was several days to 12 weeks. Episodes lasted a few seconds and varied in frequency from <10 to dozens per day. In five infants, symptoms occurred in the supine position. There was a wide variability in the work-up without any pathological findings. Follow-up ranged from 1 to 2.5 years. Ocular symptoms gradually decreased until resolution. Infants reached normal developmental milestones.ConclusionsOur identification of six patients in only 3 years suggests benign positional vertical opsoclonus may be more prevalent than previously described. In our experience, it affects otherwise healthy infants and resolves spontaneously. In view of the good long-term outcome, a comprehensive clinical investigation may not be necessary.

Аcute transverse myelitis and Guillain – Barre overlap syndrome in a patient with СOVID-19

2022 ◽  
Vol 26 (6) ◽  
pp. 43-51
Author(s):  
E. A. Ruina ◽  
V. N. Grigoryeva ◽  
A. A. Lesnikova ◽  
K. M. Beliakov
Keyword(s):  
Spinal Cord ◽  
Transverse Myelitis ◽  
Bowel Dysfunction ◽  
Intravenous Immunoglobulins ◽  
Sensory Loss ◽  
Focal Lesion ◽  
High Dose ◽  
Bladder Bowel Dysfunction ◽  
Guillain Barré

We report a case of transverse myelitis and Guillain–Barre syndrome (GBS) overlap in the 42-year-old patient with moderate course of Coronavirus disease 2019 (СOVID-19). Nasopharyngeal SARS-CoV 2 RT-PCR was positive. Severe neck pain developed in this patient on the 5-th day of СOVID-19. A few hours later weakness in the feet arised and then spread to the thighs and arms. Quadriparesis, arefl exia in all limbs, sensory loss below the level of T4 and bladder/bowel dysfunction were present. Pyramidal signs were negative. There was no increase of COVID-19 severity at the time of neurological signs development. Magnetic resonance imaging of the spinal cord showed the focal lesion in the C2-T1 segments, which was consistent with the features of longitudinally extensive transverse myelitis. Along with the myelitis, acute motor axonal polyneuropathy was diagnosed. This diagnosis of GBS was supported by ascending weakness with arefl exia, albumin-cytological dissociation in cerebrospinal fl uid and the data of neuroelectrophysiological examination. We proposed that both myelitis and GBS had disimmune nature associated with COVID-19. The other possible causes of damage to the spinal cord and peripheral nervous system were excluded.Immunotherapy with high dose of intravenous immunoglobulins was administered. Steroids also were used taking into account the myelitis. At the follow up in 4 months the motor functions were found to be improved nonsignifi cantly, the patient was still severe disabled.

scholarly journals Pregnancy-triggered triple autoimmunity (Hashimoto’s thyroiditis, antiphospholipid syndrome and systemic lupus erythematosus)

2018 ◽  
Author(s):  
Shoaib Z Junejo
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Hashimoto’S Thyroiditis ◽  
Kidney Biopsy ◽  
Anticoagulation Therapy ◽  
Hashimoto's Thyroiditis ◽  
Systemic Lupus ◽  
Work Up ◽  
New Onset

Introduction: We describe a case of 22 year old female with her first pregnancy triggered Hashimoto’s thyroiditis (HT), Antiphospholipid Syndrome (APS) and Systemic Lupus Erythematosus (SLE). Case Report: A 22 year old female was diagnosed with HT on levothyroxine during the early first trimester. During 21 weeks of gestation patient has intrauterine fetal demise and underwent medical abortion. SLE work up including antinuclear antibody and anti-double stranded DNA were positive. She underwent kidney biopsy, which revealed membranous and mesangial proliferative lupus nephritis. Diagnosis of SLE and APS was made. Treatment with anticoagulation therapy was started. SLE therapy was initiated with prednisone, mycophenolate mofetil and hydroxychloroquine with complete resolution of symptoms. We report a 23-year-old gravida in her first pregnancy, suffering from MGN and severe nephrotic syndrome, complicated by APLA syndrome. The patient was treated with enoxaparin, aspirin azathioprine, and Prednisone for a short time, in addition to furosemide and albumin intravenously. She was delivered at 30 weeks due to deteriorating maternal and foetal conditions. Discussion: APS is a prothrombotic disorder with various manifestations, most commonly venous and arterial thromboembolism and recurrent pregnancy loss. Pregnancy may trigger an underlying APS, which may well be the causative for the miscarriage. New onset SLE during pregnancy is rare. However, in our case, the anemia, thrombocytopenia, and proteinuria led us to the correct diagnosis of SLE. HT is associated with higher rates of infertility and early miscarriages, due to the associated hormonal changes and instability. However, the association of APS and HT is not well recognized in pregnant women. Conclusion: We present here a challenging case of new-onset triple autoimmune disorders trigged by pregnancy. Clinicians should be aware of this association and initiate early autoimmune work up for SLE and APS in patients with new onset of HT during pregnancy. A successful neonatal and maternal outcome was achieved in this case. The patient's history revealed thrombocytopenia and APLA syndrome and continues to be treated chronically with enoxaparin. Kidney biopsy performed after delivery showed membranous MGN stage II-III. Herein, we present a case of successful pregnancy and foetal outcome in a young woman with APLA syndrome and MN. Keywords: Pregnancy triggered Hashimoto’s thyroiditis, Antiphospholipid Syndrome and Systemic Lupus Erythematosus

scholarly journals Secondary autoimmune diseases occurring after HSCT for an autoimmune disease: a retrospective study of the EBMT Autoimmune Disease Working Party

Blood ◽  
2011 ◽  
Vol 118 (6) ◽  
pp. 1693-1698 ◽  
Author(s):  
Thomas Daikeler ◽  
Myriam Labopin ◽  
Massimo Di Gioia ◽  
Mario Abinun ◽  
Tobias Alexander ◽  
...  
Keyword(s):  
Risk Factors ◽  
Autoimmune Disease ◽  
Autoimmune Diseases ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Working Party ◽  
Thyroid Stimulating Hormone ◽  
European Multicenter Study ◽  
Autologous Hsct

Abstract To specify the incidence and risk factors for secondary autoimmune diseases (ADs) after HSCT for a primary AD, we retrospectively analyzed AD patients treated by HSCT reported to EBMT from 1995 to 2009 with at least 1 secondary AD (cases) and those without (controls). After autologous HSCT, 29 of 347 patients developed at least 1 secondary AD within 21.9 (0.6-49) months and after allogeneic HSCT, 3 of 16 patients. The observed secondary ADs included: autoimmune hemolytic anemia (n = 3), acquired hemophilia (n = 3), autoimmune thrombocytopenia (n = 3), antiphospholipid syndrome (n = 2), thyroiditis (n = 12), blocking thyroid-stimulating hormone receptor antibody (n = 1), Graves disease (n = 2), myasthenia gravis (n = 1), rheumatoid arthritis (n = 2), sarcoidosis (n = 2), vasculitis (n = 1), psoriasis (n = 1), and psoriatic arthritis (n = 1). After autologous HSCT for primary AD, the cumulative incidence of secondary AD was 9.8% ± 2% at 5 years. Lupus erythematosus as primary AD, and antithymocyte globulin use plus CD34+ graft selection were important risk factors for secondary AD by multivariate analysis. With a median follow-up of 6.2 (0.54-11) years after autologous HSCT, 26 of 29 patients with secondary AD were alive, 2 died during their secondary AD (antiphospholipid syndrome, hemophilia), and 1 death was HSCT-related. This European multicenter study underlines the need for careful management and follow-up for secondary AD after HSCT.

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