scholarly journals Amnesia in a Patient with Rheumatoid Arthritis: A Case of Granulomatosis with Polyangiitis

2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Pablo Weilg ◽  
Giancarlo Diaz-Zamora ◽  
Larry Young
Keyword(s):  
Rheumatoid Arthritis ◽  
Crescentic Glomerulonephritis ◽  
Granulomatosis With Polyangiitis ◽  
Diffuse Alveolar Damage ◽  
Oral Prednisone ◽  
Small Region ◽  
Pulse Therapy ◽  
Steroid Pulse ◽  
Acute Infarction ◽  
History Of

A 56-year-old male with a history of seropositive rheumatoid arthritis complained of polyarthritis and forgetfulness. The initial CT scan of the head without contrast was negative for acute pathologies. However, as he continued to decline despite being on oral prednisone, an MRI of the head was ordered and revealed a subtle small region of acute infarction in the left mammillary body. He was diagnosed with granulomatosis with polyangiitis in light of his positive C-ANCA antibodies, PR3 antibody, and a kidney biopsy, which showed focal necrotizing pauci-immune crescentic glomerulonephritis. Despite undergoing steroid pulse therapy, the patient developed diffuse alveolar damage which finally responded to rituximab infusion.

scholarly journals Optic neuropathy secondary to granulomatosis with polyangiitis in a patient with Graves’ disease: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Miki Sato-Akushichi ◽  
Reiko Kinouchi ◽  
Naoko Kawai ◽  
Kenichiro Nomura
Keyword(s):  
Optic Neuropathy ◽  
Visual Loss ◽  
Granulomatosis With Polyangiitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Pulse Therapy ◽  
Hypertrophic Pachymeningitis ◽  
Steroid Pulse ◽  
Corrected Visual Acuity ◽  
History Of ◽  
Myeloperoxidase Antineutrophil Cytoplasmic Antibody

Abstract Background Dysthyroid optic neuropathy is the most commonly suspected diagnosis of optic neuropathy in Graves’ patients; however, other causes need to be ruled out. We present a unique case of optic neuropathy secondary to hypertrophic pachymeningitis with antineutrophil cytoplasmic antibody-associated vasculitis, which was suspected to be antithyroid drug related. Case presentation A 79-year-old Japanese male presented with acute visual loss in the left eye. He had a 24-year history of Graves’ disease and was taking methimazole. Best-corrected visual acuity was 0.8 in the right eye and light perception in the left eye, and relative afferent pupillary defect in the left eye was seen. Ocular movement was normal, and there were no findings explaining visual loss in intermediate optic media and fundus in the left eye. Contrast-enhanced magnetic resonance imaging demonstrated thickened dura mater. Tests for myeloperoxidase-antineutrophil cytoplasmic antibody, proteinuria, and hematuria were positive; pulmonary nodule lesions and a blood clot in the left lower leg were also found. After excluding the presence of diseases that could lead to hypertrophic pachymeningitis, we diagnosed optic neuropathy due to hypertrophic pachymeningitis with granulomatosis with polyangiitis—a subtype of antineutrophil cytoplasmic antibody-associated vasculitis. Since he had history of using methimazole, antineutrophil cytoplasmic antibody-associated vasculitis was considered as drug related. We started high-dosage steroid pulse therapy followed by 1 mg/kg body weight daily of oral prednisolone, and subsequently tapered. Methimazole was stopped. Best-corrected visual acuity recovered to 0.9, 2 weeks after starting treatment. Though myeloperoxidase-antineutrophil cytoplasmic antibody remained negative, the symptom relapsed 6 months after treatment initiation. We gave a second high-dose steroid pulse therapy followed by prednisolone tapered together with methotrexate. Remission remained, and using 4 mg/week methotrexate without prednisolone, myeloperoxidase-antineutrophil cytoplasmic antibody was kept within the normal limit until now, 4 years after onset. Conclusion We present a case of optic neuropathy with hypertrophic pachymeningitis related to antineutrophil cytoplasmic antibody-associated vasculitis, which was suspected to be drug related. The patient had good visual recovery after quitting the drug and receiving immunosuppressive therapy with systemic steroids. Hypertrophic pachymeningitis with antineutrophil cytoplasmic antibody-associated vasculitis related to antithyroid drugs should be considered as a differential diagnosis for optic neuropathy in Graves’ patients in whom optic nerve compression is not obvious.

scholarly journals Eosinophilic Granulomatosis with Polyangiitis Presenting with Myocarditis as an Initial Symptom: A Case Report and Review of the Literature

2021 ◽  
pp. 329-333
Author(s):  
Kanako Kurihara ◽  
Jun Tsugawa ◽  
Shinji Ouma ◽  
Toshiyasu Ogata ◽  
Mikiko Aoki ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Nerve Biopsy ◽  
Pulse Therapy ◽  
Sural Nerve Biopsy ◽  
Lower Limbs ◽  
Rapid Progression ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Initial Symptom ◽  
Steroid Pulse ◽  
Eosinophilic Granulomatosis

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.

scholarly journals Predictors for clinical effectiveness of baricitinib in rheumatoid arthritis patients in routine clinical practice: data from a Japanese multicenter registry

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Nobunori Takahashi ◽  
Shuji Asai ◽  
Tomonori Kobayakawa ◽  
Atsushi Kaneko ◽  
Tatsuo Watanabe ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Clinical Practice ◽  
Disease Activity ◽  
Retention Rate ◽  
Oral Prednisone ◽  
Multivariate Logistic Regression Analysis ◽  
Jak Inhibitors ◽  
Short Term ◽  
History Of ◽  
Multicenter Registry

AbstractThis study aimed to evaluate the short-term effectiveness and safety profiles of baricitinib and explore factors associated with improved short-term effectiveness in patients with rheumatoid arthritis (RA) in clinical settings. A total of 113 consecutive RA patients who had been treated with baricitinib were registered in a Japanese multicenter registry and followed for at least 24 weeks. Mean age was 66.1 years, mean RA disease duration was 14.0 years, 71.1% had a history of use of biologics or JAK inhibitors (targeted DMARDs), and 48.3% and 40.0% were receiving concomitant methotrexate and oral prednisone, respectively. Mean DAS28-CRP significantly decreased from 3.55 at baseline to 2.32 at 24 weeks. At 24 weeks, 68.2% and 64.1% of patients achieved low disease activity (LDA) and moderate or good response, respectively. Multivariate logistic regression analysis revealed that no previous targeted DMARD use and lower DAS28-CRP score at baseline were independently associated with achievement of LDA at 24 weeks. While the effectiveness of baricitinib was similar regardless of whether patients had a history of only one or multiple targeted DMARDs use, patients with previous use of non-TNF inhibitors or JAK inhibitors showed lower rates of improvement in DAS28-CRP. The overall retention rate for baricitinib was 86.5% at 24 weeks, as estimated by Kaplan–Meier analysis. The discontinuation rate due to adverse events was 6.5% at 24 weeks. Baricitinib significantly improved RA disease activity in clinical practice. Baricitinib was significantly more effective when used as a first-line targeted DMARDs.

Conjunctivitis, episcleritis and anterior uveitis as the first presenting features of granulomatosis with polyangiitis

2021 ◽  
Vol 14 (10) ◽  
pp. e243558
Author(s):  
Lucas Donato Foster ◽  
Michael Nyugen ◽  
Edward Margolin
Keyword(s):  
Anterior Uveitis ◽  
Crescentic Glomerulonephritis ◽  
Granulomatosis With Polyangiitis ◽  
Oral Prednisone ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Final Diagnosis ◽  
Ocular Inflammation ◽  
Reactive Protein ◽  
Ocular Manifestations ◽  
Organ Systems

Granulomatosis with polyangiitis (GPA) is a rare disorder characterised by inflammation of small-sized and medium-sized blood vessels that result in damage to various organ systems, but it most commonly affects the respiratory tract and kidneys. It is one of the few entities that can present with ocular inflammation as well as renal impairment at the same time. We describe a case of a 38-year-old man with conjunctivitis, episcleritis, anterior uveitis as a first manifestation of GPA. His presentation with red eye and anterior uveitis prompted further workup, which revealed acute renal failure (creatinine 442 mmol/L), elevated inflammatory markers (erythrocyte sedimentation rate of 85 mmol/hour and C reactive protein of 72 mg/L), and a c-antineutrophil cytoplasmic antibody titre >8. An urgent renal biopsy was performed demonstrating necrotising crescentic glomerulonephritis, which led to the final diagnosis of GPA. Treatment induction with intravenous methylprednisolone and plasmapheresis followed by an oral prednisone taper and intravenous rituximab infusions leading to resolution of all symptoms and normalisation of kidney function. This report highlights conditions that can present with both ocular inflammation and renal dysfunction with a focus on GPA and its ocular manifestations.

P0430EFFECT OF CORTICOSTEROIDS THERAPY FOR PATIENTS OF IGA NEPHROPATHY WITH CRESCENTS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Mengjun Liang ◽  
Aihua Li ◽  
Zongpei Jiang
Keyword(s):  
Renal Function ◽  
Side Effects ◽  
Iga Nephropathy ◽  
Steroid Therapy ◽  
Oral Prednisone ◽  
Pulse Therapy ◽  
The Other ◽  
Intravenous Methylprednisolone ◽  
Steroid Pulse ◽  
Renal Function Deterioration

Abstract Background and Aims Patients with IgA nephropathy (IgAN) presented proteinuria≥1g/d and eGFR≥50ml/min/1.73m2 after supportive treatment had been advised 6-month course of corticosteroids therapy. Update of Oxford classification of IgAN had recommended crescents be added to the MEST score for they were predictive of outcome. Whether we should take some more positive therapy for crescents? Method We conducted a single-center, retrospective cohort study enrolling 46 patients from 2017.01 to 2018.06, diagnosed with IgAN by renal biopsy. Eligible patients had proteinuria of 0.5∼3.5g/d, eGFR≥30ml/min/1.73m2 and crescent proportion <50%. Patients were divided into two groups, one for classical steroid treatment (intravenous methylprednisolone 0.25g/d for 3 days at the beginning of months 1, 3 and 5, plus oral prednisone 0.5 mg/kg/d for 6 months, called 1-3-5 Group) and the other assigned an optimized steroid therapy (intravenous methylprednisolone 0.25g/d for 3 days at the beginning of months 1, 2 and 3, plus oral prednisone 0.5 mg/kg/d for 6 months, called 1-2-3 Group). The primary endpoint was remission of proteinuria, secondary endpoint was deterioration in renal function. Results There were 23 patients in each group and no significant differences in age, gentle, baseline proteinuria and eGFR between the two groups, except for the proportion of crescents (for Oxford C1 and C2: 52.5% and 13% in 1-3-5 Group vs. 95.7% and 4.3% in 1-2-3 Group respectively, p=0.001). After 6 months therapy, proteinuria in 1-3-5 Group was 0.5(0.2,0.8)g/d (vs. 1.2(0.8,2.6)g/d at baseline, p <0.001) and that in 1-2-3 Group was 0.3(0.2,0.6)g/d (vs. 1.5(0.7,2.6)g/d at baseline, p <0.001). 78.3% of patients in 1-3-5 Group had got remission of proteinuria, while 95.7% in 1-2-3 Group (p=0.187). The 6th month eGFR in 1-3-5 Group was 80.7(59.8,116.2)ml/min/1.73m2 (vs. 77.5(54.8,104.6)ml/min/1.73m2 at baseline, p=0.212), while that in 1-2-3 Group was 97.8(68.6,130.9)ml/min/1.73m2 (vs. 79.5(52.9,108.7)ml/min/1.73m2 at baseline, p=0.002). The slope of eGFR in 1-3-5 Group was 0.7(-1.7,3.3)ml/min/1.73m2/month, while that in 1-2-3 Group was 3(1.2,5.4)ml/min/1.73m2/month, p=0.027. For side effects, two patients in 1-2-3 Group had met bronchitis during the 2nd and 3rh therapy-month respectively; in 1-3-5 Group, one patient had got glaucoma during the 2nd therapy-month and the other had happened steroid-induced diabetes mellitus during the 3rd therapy-month. Conclusion Our preliminary results had indicated that optimized steroid therapy had equal effect on reducing proteinuria but more significant advantage to protect against renal function deterioration in IgAN with crescents. 1-2-3 month-steroid pulse therapy had not increase the morbidity of irreversible or severe side effects.

scholarly journals Rheumatoid Meningitis Occurring during Etanercept Treatment

2017 ◽  
Vol 2017 ◽  
pp. 1-5 ◽  
Author(s):  
Koji Tsuzaki ◽  
Takashi Nakamura ◽  
Hiroyuki Okumura ◽  
Naoko Tachibana ◽  
Toshiaki Hamano
Keyword(s):  
Rheumatoid Arthritis ◽  
Mononuclear Cells ◽  
Brain Magnetic Resonance Imaging ◽  
Brain Biopsy ◽  
Pulse Therapy ◽  
Clinical Findings ◽  
Etanercept Treatment ◽  
Steroid Pulse ◽  
Parietal Lobes ◽  
Rheumatoid Meningitis

We report a 65-year-old man who had repetitive seizures 6 months after receiving etanercept, methotrexate, and prednisolone for rheumatoid arthritis. Mononuclear cells were mildly increased in the cerebrospinal fluid (CSF). Brain magnetic resonance imaging (MRI) showed high intensity along sulci of the frontal and parietal lobes. Brain biopsy revealed lymphocyte and plasma cell infiltration in the meninges, confirming the diagnosis of rheumatoid meningitis. After steroid pulse therapy, seizures resolved and clinical findings improved. When etanercept was replaced by tocilizumab, rheumatoid meningitis did not recur. Although TNF-α inhibitors can control joint symptoms of rheumatoid arthritis, they may induce rheumatoid meningitis.

Steroid Therapy as Treatment for Idiopathic Fibrosis of the Retroperitoneum and Mediastinum

PEDIATRICS ◽  
1986 ◽  
Vol 78 (5) ◽  
pp. 936-938
Author(s):  
CHARLES FEILD ◽  
WATSON ARNOLD ◽  
ELIZABETH S. GLOSTER ◽  
GREG SHARP ◽  
JOHN REDMAN
Keyword(s):  
Steroid Therapy ◽  
Retroperitoneal Fibrosis ◽  
Oral Prednisone ◽  
Pulse Therapy ◽  
Chief Complaint ◽  
Oral Steroid ◽  
Shortness Of Breath ◽  
Steroid Pulse ◽  
Alternative Modality ◽  
Oral Steroid Therapy

Multifocal idiopathic fibrosis of both mediastinum and retroperitoneum is rare in childhood.1,2 We describe a patient who presented with biopsyproven mediastinal and retroperitoneal fibrosis associated with progressive pulmonary fibrosis, cardiomyopathy, and bilateral ureteropyelocaliectasis; the patient responded to intravenous pulse and oral steroid therapy with relief of his uretenal obstruction and an improvement in pulmonary and cardiac function. Although surgical ureterolysis is generally the management of choice for ureteral obstruction caused by retroperitioneal fibrosis,3,4 steroid pulse therapy and a regimen of oral prednisone may provide an alternative modality of themapy.5-7 CASE REPORT This 12-year-old black boy was seen in the emergency room with a chief complaint of shortness of breath.

scholarly journals Clinical Management of Acute Interstitial Pneumonia: A Case Report

2012 ◽  
Vol 2012 ◽  
pp. 1-4
Author(s):  
Yang Xia ◽  
Zhenyu Liang ◽  
Zhenzhen Fu ◽  
Laiyu Liu ◽  
Omkar Paudel ◽  
...  
Keyword(s):  
Interstitial Pneumonia ◽  
Diffuse Alveolar Damage ◽  
Pulse Therapy ◽  
Respiratory Dysfunction ◽  
Steroid Pulse ◽  
Acute Interstitial Pneumonia ◽  
Progressive Dyspnea ◽  
Ground Glass Attenuation ◽  
Traction Bronchiectasis ◽  
Fluid Test

We describe a 51-year-old woman who was admitted to hospital because of cough and expectoration accompanied with general fatigue and progressive dyspnea. Chest HRCT scan showed areas of ground glass attenuation, consolidation, and traction bronchiectasis in bilateral bases of lungs. BAL fluid test and transbronchial lung biopsy failed to offer insightful evidence for diagnosis. She was clinically diagnosed with acute interstitial pneumonia (AIP). Treatment with mechanical ventilation and intravenous application of methylprednisolone (80 mg/day) showed poor clinical response and thus was followed by steroid pulse therapy (500 mg/day, 3 days). However, she died of respiratory dysfunction eventually. Autopsy showed diffuse alveolar damage associated with hyaline membrane formation, pulmonary interstitial, immature collagen edema, and focal type II pneumocyte hyperplasia.

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