scholarly journals A case of thymoma-associated multiorgan autoimmunity including polymyositis and myocarditis

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Chihiro Furuta ◽  
Motoki Yano ◽  
Hiroki Numanami ◽  
Masayuki Yamaji ◽  
Rumiko Taguchi ◽  
...  

Abstract Background Polymyositis and myocarditis associated with thymoma are exceptionally rare conditions and usually accompanied by myasthenia gravis (MG) and have been recognized as critical conditions. Thymoma-associated multiorgan autoimmunity was reported recently with skin, liver, and intestinal manifestations similar to those seen in graft-versus-host disease. Case presentation A 77-year-old female presented to our department with exacerbation of ptosis and local recurrence of thymoma. Chest computed tomography revealed local recurrence of thymoma. Following 6 month observation, erythema on the extremities and body trunk suddenly appeared. Afterwards, the patient developed progressive muscle weakness and fatigue. We diagnosed as myocarditis and polymyositis. She was transferred to the intensive-care unit and received artificial ventilation. Steroid pulse therapy was induced immediately. The blood test findings were markedly improved, but the symptoms of MG and weakness of the muscles persisted. Various treatment including eculizumab was induced, and the symptoms of MG and weakness of the muscles were improved. On the 136th day of hospitalization, she was discharged. Conclusion We were able to cure this patient, as we were able to start treatment immediately after the appearance of severe symptoms. An early diagnosis and treatment are important for curing such patients.

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Suqiong Ji ◽  
Chenchen Liu ◽  
Zhuajin Bi ◽  
Huajie Gao ◽  
Jian Sun ◽  
...  

Abstract Background Central nervous system overlapping autoimmune syndromes are uncommon, especially with the coexistence of MOG-IgG and GFAP-IgG. Case presentation A 23-year-old woman presented with transient convulsions, a loss of consciousness, persistent fever, headache, and vomiting. Cerebrospinal fluid (CSF) analysis revealed elevated cellularity, and magnetic resonance imaging (MRI) showed diffuse leptomeningeal enhancement. She had fever and headache with antiviral and antibiotic treatment for 2 weeks, and she had empirical anti-tuberculosis treatment and oral prednisolone therapy. She was followed for 3 months after presentation with improved symptoms and normal CSF analysis. A 3-month follow-up MRI showed asymmetric lesions in the cerebellum, corona radiata, and white matter with enhancement. The anti-tuberculosis treatment was continued, and steroid therapy was discontinued. After she stopped taking prednisolone, an interrupted headache gradually appeared. MRI at 4 months after presentation revealed a partial reduction in lesions but enlarged areas in the left cerebellum and right parietal white matter and a new lesion in the region of the right ependyma with linear enhancement. Her CSF was positive for anti-myelin oligodendrocyte glycoprotein (MOG) and anti-glial fibrillary acidic protein (GFAP) antibodies using a transfected cell-based assay. She was diagnosed with overlapping syndrome of MOG‑IgG‑associated disease and GFAP astrocytopathy. She received steroid pulse therapy (methylprednisolone, 1 g for 5 days), followed by a gradual tapering of oral prednisolone and the addition of an immunosuppressant (tacrolimus, 3 mg per day). Six months after the initial presentation, she had no symptoms. An MRI showed that the lesions had diminished, and no enhancement was found. Conclusions We report a case that was positive for double antibodies, which was initially misdiagnosed as infectious meningoencephalitis. This case broadens the clinical and phenotypic presentation of the overlapping syndrome spectrum.


2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Ahmed Maseh Haidary ◽  
Sarah Noor ◽  
Esmatullah Hamed ◽  
Tawab Baryali ◽  
Soma Rahmani ◽  
...  

Abstract Introduction The novel coronavirus, since its first identification in China, in December 2019, has shown remarkable heterogeneity in its clinical behavior. It has affected humans on every continent. Clinically, it has affected every organ system. The outcome has also been variable, with most of the older patients showing grave outcomes as compared with the younger individuals. Here we present a rare and severe variant of Guillain–Barre syndrome that complicated the disease in recovery phase. Case presentation A 60-year-old Afghan man, who had been recovering from symptoms related to novel coronavirus associated disease, presented with sudden onset of progressive muscle weakness and oxygen desaturation. Electrophysiological workup confirmed the diagnosis of Guillain–Barre syndrome, and early institution of intravenous immunoglobulin resulted in complete resolution. Conclusion Guillain–Barre syndrome has recently been reported in many patients diagnosed with novel coronavirus associated disease. While clinical suspicion is mandatory to guide towards an effective diagnostic workup, early diagnosis of this complication and timely institution of therapeutic interventions are indispensable and lifesaving.


2021 ◽  
pp. 329-333
Author(s):  
Kanako Kurihara ◽  
Jun Tsugawa ◽  
Shinji Ouma ◽  
Toshiyasu Ogata ◽  
Mikiko Aoki ◽  
...  

A 66-year-old woman with a history of bronchial asthma had shortness of breath and fatigue upon mild exercise. She was diagnosed as congestive heart failure. A blood test showed eosinophilia without the presence of anti-neutrophil cytoplasmic antibody (ANCA), and a myocardial biopsy specimen revealed eosinophilic infiltration in the myocardium. Eosinophilia was improved when she was administered short-term methylprednisolone. After that, she had numbness and pain in her lower limbs with re-elevation of eosinophils. She had dysesthesia and hypalgesia in the distal part of the limbs. Sural nerve biopsy revealed axonal degeneration and thickness of the arterial wall, indicating a diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA). Two courses of steroid pulse therapy were performed, resulting in marked improvement of her sensory symptoms. ANCA-negative EGPA might be associated with myocarditis and peripheral neuropathy. A sufficient immunotherapy should have been considered to prevent rapid progression.


2021 ◽  
Vol 69 (1) ◽  
Author(s):  
Muhammad Adel ◽  
Ahmed Magdy

Abstract Background Coronavirus disease (COVID-19) presents in children usually with less severe manifestations than in adults. Although fever and cough were reported as the most common symptoms, children can have non-specific symptoms. We describe an infant with aplastic anemia as the main manifestation. Case presentation We describe a case of SARS-CoV-2 infection in an infant without any respiratory symptoms or signs while manifesting principally with pallor and purpura. Pancytopenia with reticulocytopenia was the predominant feature in the initial laboratory investigations, pointing to aplastic anemia. Chest computed tomography surprisingly showed typical findings suggestive of SARS-CoV-2 infection. Infection was later confirmed by positive real-time reverse transcription polymerase chain reaction assay (RT-PCR) for SARS-CoV-2. Conclusions Infants with COVID-19 can have non-specific manifestations and a high index of suspicion should be kept in mind especially in regions with a high incidence of the disease. Chest computed tomography (CT) and testing for SARS-CoV-2 infection by RT-PCR may be considered even in the absence of respiratory manifestations.


Nephron ◽  
2020 ◽  
pp. 1-5
Author(s):  
Mika Fujimoto ◽  
Kan Katayama ◽  
Kouhei Nishikawa ◽  
Shoko Mizoguchi ◽  
Keiko Oda ◽  
...  

There is no specific treatment for recurrent Henoch-Schönlein purpura nephritis (HSPN) in a transplanted kidney. We herein report a case of a kidney transplant recipient with recurrent HSPN that was successfully treated with steroid pulse therapy and epipharyngeal abrasive therapy (EAT). A 39-year-old Japanese man developed HSPN 4 years ago and had to start hemodialysis after 2 months despite receiving steroid pulse therapy followed by oral prednisolone, plasma exchange therapy, and cyclophosphamide pulse therapy. He had undergone tonsillectomy 3 years earlier in the hopes of achieving a better outcome of a planned kidney transplantation and received a living-donor kidney transplantation from his mother 1 year earlier. Although there were no abnormalities in the renal function or urinalysis 2 months after transplantation, a routine kidney allograft biopsy revealed evidence of mesangial proliferation and cellular crescent formation. Mesangial deposition for IgA and C3 was noted, and he was diagnosed with recurrent HSPN histologically. Since the renal function and urinalysis findings deteriorated 5 months after transplantation, 2 courses of steroid pulse therapy were performed but were ineffective. EAT using 0.5% zinc chloride solution once per day was combined with the third course of steroid pulse therapy, as there were signs of chronic epipharyngitis. His renal function recovered 3 months after daily EAT and has been stable for 1.5 years since transplantation. Daily EAT continued for >3 months might be a suitable strategy for treating recurrent HSPN in cases of kidney transplantation.


2016 ◽  
Vol 53 (11) ◽  
pp. 1015-1016 ◽  
Author(s):  
Tomomi Sato ◽  
Junpei Somura ◽  
Yoshihiro Maruo

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