scholarly journals A case report of a severe neonatal systemic vasculitis on the first day of life

2021 ◽  
Author(s):  
Stephanie Wong ◽  
Erkan Demirkaya ◽  
Roberta Berard
Keyword(s):  
Reactive Arthritis ◽  
Index Finger ◽  
Systemic Vasculitis ◽  
Post Partum ◽  
Normal Growth ◽  
Fresh Frozen Plasma ◽  
Left Index Finger ◽  
High Dose ◽  
Preterm Baby ◽  
New Onset

Abstract BACKGROUND: Transmission of autoantibodies across the placenta has been associated with neonatal cutaneous vasculitis and lupus syndromes. Here we report a novel association of a case of new-onset maternal seronegative inflammatory arthritis associated with a transient systemic vasculitis in a neonate. CASE PRESENTATION: In the first 24 hours of life, a preterm baby boy was noted to have blue discoloration to all four extremities. A workup for sepsis and thrombosis were negative. Despite antibiotics, fresh frozen plasma and anticoagulation, the discoloration remained, particularly in the left index finger. This was associated with fever and a maximum C-reactive protein (CRP) of 148 mg/L. Two doses of intravenous immunoglobulin (IVIG) were given with short-term improvement. Echocardiogram was normal. The fever, elevated CRP and finger discoloration responded to high dose steroid administration. MRI angiography (MRA) of body and heart showed tortuosity of arteries in the upper and lower extremities with gadolinium uptake, suggestive of vasculitis. Autoantibody profile negative. Genetic panel for hereditary autoinflammatory diseases was negative as was as whole exome sequencing performed on the trio. The baby was weaned off steroids by 5 months of age. A small distal autoamputation of the left index finger occurred. He was born to a 28-year-old woman who developed new onset severe symmetrical polyarthritis at 8 weeks gestation. It was presumed this was reactive arthritis secondary to a dental infection. Infectious work up and autoantibodies were negative. She was treated with high dose prednisone for the remainder of her pregnancy. The mother was weaned off prednisone and treated with hydroxychloroquine for 8 months post-partum and remains in remission. A repeat MRA done at 1 year old showed mild residual tortuosities of the arteries in the forearms. The remainder of the medium and large vessels were within normal limits with no gadolinium enhancement to suggest active disease. The child is now 4 years old with normal growth and development. CONCLUSION: This is a unique case of new-onset seronegative presumed reactive arthritis in a mother with the rare development of a successfully treated medium vessel vasculitis in an infant.

scholarly journals A case report of a severe neonatal systemic vasculitis on the first day of life

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Stephanie Wong ◽  
Erkan Demirkaya ◽  
Roberta Berard
Keyword(s):  
Lupus Erythematosus ◽  
Reactive Arthritis ◽  
Index Finger ◽  
Systemic Vasculitis ◽  
Clinical Signs ◽  
The Body ◽  
Oral Steroid ◽  
Left Index Finger ◽  
High Dose ◽  
New Onset

Abstract Background Neonatal systemic vasculitis syndromes have been reported in infants born to mothers with systemic lupus erythematosus, Sjögren’s syndrome, Behҫet’s disease, cutaneous polyarteritis nodosa and anti-neutrophil cytoplasmic antibody-associated vasculitides. Here we report a novel association of a case of new-onset maternal seronegative inflammatory arthritis associated with a transient systemic vasculitis in a neonate. Case presentation In the first 24 h of life, a preterm Caucasian baby boy was noted to have blue discoloration to all four extremities. Despite antibiotics, fresh frozen plasma and anticoagulation, the discoloration remained, particularly in the left index finger. This was associated with fever and a maximum C-reactive protein (CRP) of 148 mg/L. Intravenous immunoglobulin (IVIG) was given with short-term improvement. Initial echocardiogram showed enlarged coronary arteries with normalization on repeat 1 week later. Clinical signs and symptoms responded to high dose oral steroid administration. MRI angiography (MRA) of the body and heart showed tortuosity of arteries in the upper and lower extremities with gadolinium uptake, suggestive of vasculitis. Autoantibody profile negative. Genetic panel for hereditary autoinflammatory diseases was negative as was whole exome sequencing performed on the trio. The baby was weaned off steroids by 5 months of age. A small distal autoamputation of the left index finger occurred. He was born to a 28-year-old woman who developed new onset severe symmetrical polyarthritis at 8 weeks gestation. This was presumed a reactive arthritis secondary to a dental infection. Infectious work up and autoantibodies were negative. She was treated with high dose prednisone for the remainder of her pregnancy. The mother was weaned off prednisone, treated with hydroxychloroquine for 8 months post-partum and remains in remission. A repeat MRA done at 1 year old showed mild residual tortuosities of the arteries in the forearms. The remainder of the medium and large vessels were within normal limits with no gadolinium enhancement to suggest active disease. The child is now 4 years old with normal growth and development. Conclusion This is a unique case of new-onset seronegative presumed reactive arthritis in a mother with the rare development of a successfully treated medium vessel vasculitis in an infant.

scholarly journals Levamisole/Cocaine Induced Systemic Vasculitis and Immune Complex Glomerulonephritis

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Lohit Garg ◽  
Sagar Gupta ◽  
Abhishek Swami ◽  
Ping Zhang
Keyword(s):  
Immune Complex ◽  
Systemic Vasculitis ◽  
Renal Involvement ◽  
Morphological Properties ◽  
High Dose ◽  
Cocaine Use ◽  
Immune Mediated ◽  
History Of ◽  
Psychotropic Effects ◽  
Complex Deposition

Levamisole is an antihelminthic and immunomodulator medication that was banned by the USFDA in 1998. It has been increasingly used to adulterate cocaine due to its psychotropic effects and morphological properties. Adverse reactions including cutaneous vasculitis, thrombocytopenia, and agranulocytosis have been well described. Despite systemic vasculitis in this setting, renal involvement is uncommon. We report here a case of ANCA positive systemic vasculitis with biopsy proven immune complex mediated glomerulonephritis likely secondary to levamisole/cocaine. A 40-year-old Caucasian male with no past medical history presented with 3-week history of fatigue, skin rash, joint pains, painful oral lesions, oliguria, hematuria, worsening dyspnea on exertion, and progressive lower extremity edema. He had a history of regular tobacco and cocaine use. Lab testing revealed severe anemia, marked azotemia, deranged electrolytes, and 4.7 gm proteinuria. Rheumatologic testing revealed hypocomplementemia, borderline ANA, myeloperoxidase antibody, and positive atypical p-ANCA. Infectious and other autoimmune workup was negative. Kidney biopsy was consistent with immune mediated glomerulonephritis and showed mesangial proliferation and immune complex deposition consisting of IgG, IgM, and complement. High dose corticosteroids and discontinuing cocaine use resulted in marked improvement in rash, mucocutaneous lesions, and arthritis. There was no renal recovery and he remained hemodialysis dependent.

scholarly journals A woman with visual loss, amenorrhoea and polyuria: the first reported case of nodular lymphocyte-predominant Hodgkin lymphoma presenting with hypopituitarism

2021 ◽  
Vol 2021 ◽  
Author(s):  
Matthew Seymour ◽  
Thomas Robertson ◽  
Jason Papacostas ◽  
Kirk Morris ◽  
Jennifer Gillespie ◽  
...  
Keyword(s):  
Hodgkin Lymphoma ◽  
Visual Loss ◽  
Post Partum ◽  
Bone Lesion ◽  
Optic Chiasm ◽  
High Dose ◽  
List Type ◽  
Sellar Mass ◽  
Blurred Vision ◽  
Surgical Biopsy

Summary A 34-year-old woman presented 18 months post-partum with blurred vision, polyuria, amenorrhoea, headache and general malaise. Comprehensive clinical examination showed left superior temporal visual loss only. Initial investigations revealed panhypopituitarism and MRI demonstrated a sellar mass involving the infundibulum and hypothalamus. Lymphocytic hypophysitis was suspected and high dose glucocorticoids were commenced along with desmopressin and thyroxine. However, her vision rapidly deteriorated. At surgical biopsy, an irresectable grey amorphous mass involving the optic chiasm was identified. Histopathology was initially reported as granulomatous hypophysitis. Despite the ongoing treatment with glucocorticoids, her vision worsened to light detection only. Histopathological review revised the diagnosis to partially treated lymphoma. A PET scan demonstrated avid uptake in the pituitary gland in addition to splenic involvement, lymphadenopathy above and below the diaphragm, and a bone lesion. Excisional node biopsy of an impalpable infraclavicular lymph node confirmed nodular lymphocyte-predominant Hodgkin lymphoma. Hyper-CVAD chemotherapy was commenced, along with rituximab; fluid-balance management during chemotherapy (with its requisite large fluid volumes) was extremely complex given her diabetes insipidus. The patient is now in clinical remission. Panhypopituitarism persists; however, her vision has recovered sufficiently for reading large print and driving. To the best of our knowledge, this is the first reported case of Hodgkin lymphoma presenting initially as hypopituitarism. Learning points Lymphoma involving the pituitary is exceedingly rare and, to the best of our knowledge, this is the first reported case of nodular lymphocyte-predominant Hodgkin lymphoma presenting as hypopituitarism. There are myriad causes of a sellar mass and this case highlights the importance of reconsidering the diagnosis when patients fail to respond as expected to appropriate therapeutic intervention. This case highlights the difficulties associated with managing panhypopituitary patients receiving chemotherapy, particularly when this involves large volumes of i.v. hydration fluid.

scholarly journals Case Report on Management of Combined Factor V and factor VIII Deficiency during Pregnancy

2021 ◽  
pp. 1-2
Author(s):  
Dr. Kirti Solanki ◽  
Dr. Swati Kochar ◽  
Dr. Shweta Choudhary ◽  
Dr. Priyanka Gaur ◽  
Dr. Krishna Krishna
Keyword(s):  
Factor Viii ◽  
Post Partum ◽  
Autosomal Recessive Disorder ◽  
Fresh Frozen Plasma ◽  
Factor V ◽  
Bleeding Tendency ◽  
Factor Viii Deficiency ◽  
Risk Of Bleeding ◽  
Increased Risk ◽  
Fresh Frozen

Combined factor V and factor VIII deficiency (CF5F8D) is a rare autosomal recessive disorder associated with mild to moderate risk of bleeding tendency. These patients have an increased risk of bleeding after surgical procedures. Pregnant women are at increased risk of having a miscarriage, placental abruption, or post partum hemorrhage. Management of these patients requires the replacement of deficient factors. We are reporting a case of management of a 31-year old second gravida female with combined factor V and factor VIII deficiency, who was transfused with fresh frozen plasma before and during labor to prevent bleeding episodes.

scholarly journals Response preparation involves a release of intracortical inhibition in task-irrelevant muscles

2020 ◽  
Author(s):  
Isaac N. Gomez ◽  
Kara Ormiston ◽  
Ian Greenhouse
Keyword(s):  
Transcranial Magnetic Stimulation ◽  
Magnetic Stimulation ◽  
Index Finger ◽  
Intracortical Inhibition ◽  
Left Index Finger ◽  
Task Condition ◽  
Response Preparation ◽  
Action Preparation ◽  
The Right ◽  
Task Irrelevant

AbstractAction preparation involves widespread modulation of motor system excitability, but the precise mechanisms are unknown. In this study, we investigated whether intracortical inhibition changes in task-irrelevant muscle representations during action preparation. We used transcranial magnetic stimulation (TMS) combined with electromyography in healthy human adults to measure motor evoked potentials (MEPs) and cortical silent periods (CSPs) in task-irrelevant muscles during the preparatory period of simple delayed response tasks. In Experiment 1, participants responded with the left-index finger in one task condition and the right-index finger in another task condition, while MEPs and CSPs were measured from the contralateral non-responding and tonically contracted index finger. During Experiment 2, participants responded with the right pinky finger while MEPs and CSPs were measured from the tonically contracted left-index finger. In both experiments, MEPs and CSPs were compared between the task preparatory period and a resting intertrial baseline. The CSP duration during response preparation decreased from baseline in every case. A laterality difference was also observed in Experiment 1, with a greater CSP reduction during the preparation of left finger responses compared to right finger responses. MEP amplitudes showed no modulation during movement preparation in any of the three response conditions. These findings indicate cortical inhibition associated with task-irrelevant muscles is transiently released during action preparation and implicate a novel mechanism for the controlled and coordinated release of motor cortex inhibition.New & NoteworthyIn this study we observed the first evidence of a release of intracortical inhibition in task-irrelevant muscle representations during response preparation. We applied transcranial magnetic stimulation to elicit cortical silent periods in task-irrelevant muscles during response preparation and observed a consistent decrease in the silent period duration relative to a resting baseline. These findings address the question of whether cortical mechanisms underlie widespread modulation in motor excitability during response preparation.

Isolated intestinal polyarteritis nodosa in an elderly patient

2021 ◽  
Vol 14 (3) ◽  
pp. e241431
Author(s):  
Ariana González-Meléndez ◽  
Eduardo J Medina-Parrilla ◽  
Román Vélez ◽  
Luis M Vilá
Keyword(s):  
Abdominal Pain ◽  
Polyarteritis Nodosa ◽  
Elderly Population ◽  
Systemic Vasculitis ◽  
Exploratory Laparotomy ◽  
The Elderly ◽  
High Dose ◽  
Systemic Involvement ◽  
Intestinal Pseudo Obstruction ◽  
Single Organ

Polyarteritis nodosa (PAN) is a necrotising systemic vasculitis involving medium-sized and small-sized vessels. PAN limited to a single organ is rare, particularly in the elderly population. Herein, we present a 73-year-old-woman who developed severe abdominal pain. Mesenteric angiography showed multifocal areas of segmental dilation and narrowing of the superior mesenteric, ileocolic and right colonic arteries. Exploratory laparotomy revealed multiple areas of necrosis of the jejunum for which resection was performed. Histopathological exam disclosed mesenteric vasculitis with fibrinoid necrosis of the arterial wall with leucocytic infiltrates and haemorrhages consistent with PAN. She was started on high-dose corticosteroids with an initial good response. However, 6 months later, she developed intestinal pseudo-obstruction for which oral cyclophosphamide was started. After 5 months of cyclophosphamide therapy, she remained stable without further relapses. Our case suggests that PAN should be considered in elderly patients presenting with abdominal pain even in the absence of systemic involvement.

scholarly journals P179 Glucocorticoid exposure and link to serious adverse events in patients with ANCA-associated vasculitis

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Philip Spearpoint ◽  
Cormac Sammon ◽  
Antonio Ramirez de ◽  
Arellano Serna ◽  
Peter Rutherford
Keyword(s):  
Cardiovascular Disease ◽  
Adverse Events ◽  
Renal Disease ◽  
Real World ◽  
Low Dose ◽  
Remission Induction ◽  
High Dose ◽  
Anca Associated Vasculitis ◽  
Increased Risk ◽  
New Onset

Abstract Background Remission induction in ANCA-associated vasculitis (AAV) is with high dose glucocorticoids (GC) and immunosuppressants. Patients are exposed to high GC dose and/or prolonged low dose. EULAR/EDTA guidelines target 7.5-10mg at 3 months but acknowledge this is often achieved later. This study used UK real world practice data to examine the scale of GC exposure and associated clinical risks in AAV. Methods The study utilised the Clinical Practice Research Datalink (CPRD) - Hospital Episode Statistics (HES) linked database. AAV patients were identified using specific READ and ICD codes and followed between 01/01/1997 and 01/01/2018. GP prescriptions were used to describe periods of continuous GC use, stop and restart and when high dose (> 30mg/day) and low dose (<30mg/day) was prescribed. Diagnostic codes indicative of infections and adverse events linked to GCs were used to estimate rates in the AAV population using a generalized linear model with a Poisson distribution. Results 450 AAV patients with at least one GC prescription were analysed. The median dose decreased to 9.3 mg (IQR 5.0 - 17.0) at 6 months and 5.1 mg (0.00 - 10.0) at 12 months,50% patients were taking > 10mg at 5 months and 25% were still > 10mg at 12 months. However, within 6 months of achieving 10mg/day, 50% relapse to needing dose >10mg, 75% within 2 years and 90% within 6 years. In adjusted Poisson model (age, gender, year of diagnosis before/after 2013) the rate of infection in AAV patients taking high dose was 2.59 times (CI95 1.95, 3.45) that of those on low dose and lower in those not taking GCs (IRR 0.27 (0.22-0.34)). Increased risk of new onset cardiovascular disease (IRR 2.55 (0.92, 7.04)) and new onset renal disease (IRR 3.4 (1.29-8.96)) were higher in patients receiving high dose. Conclusion AAV patients have significant exposure to high dose GCs and in real world practice, GC dose remains higher than recommended in current clinical guidelines. High dose GCs are associated with high risk of infection and new cardiovascular disease and renal disease. This creates a significant patient burden and has implications for healthcare resource use. Disclosures P. Spearpoint: Corporate appointments; Employee of Vifor Pharma. C. Sammon: Corporate appointments; Employee of PHMR. A. Ramirez de Arellano Serna: Corporate appointments; Employee of Vifor Pharma. P. Rutherford: Corporate appointments; Employee of Vifor Pharma. Shareholder/stock ownership; Vifor Pharma.

scholarly journals Photo Quiz: A 53-Year-Old Thai Man with Progressive Swelling of the Left Index Finger

2020 ◽  
Vol 58 (4) ◽  
Author(s):  
Piyaporn Chokevittaya ◽  
Anucha Apisarnthanarak ◽  
Siriththin Chansirikarnjana ◽  
Chinnakart Boonyasirikool ◽  
Jutatip Kintarak ◽  
...  
Keyword(s):  
Index Finger ◽  
Left Index Finger

scholarly journals Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease Presenting as Recurrent and Migrating Focal Cortical Encephalitis

2020 ◽  
Vol 7 ◽  
pp. 2329048X2096617
Author(s):  
Xinran Maria Xiang ◽  
Rachel Evans ◽  
Jesus Lovera ◽  
Rashmi Rao
Keyword(s):  
Brain Mri ◽  
Occipital Cortex ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
New Right ◽  
High Dose ◽  
Antibody Testing ◽  
Leptomeningeal Enhancement ◽  
The Right ◽  
Work Up ◽  
New Onset

Although pediatric myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease is increasingly well-recognized, its full clinical spectrum is still being defined. Cortical encephalitis is emerging as a distinct clinico-radiologic syndrome of adult MOG antibody-associated disease. We describe a 12-year-old girl who presented with new onset seizures and left-sided hemiparesis. Brain MRI showed edema of the right temporal-parietal-occipital cortex with associated focal leptomeningeal enhancement. Patient received high-dose corticosteroids and 21 days of acyclovir despite negative infectious work-up due to the focal nature of encephalitis. Patient remained seizure-free for 20 months before presenting with new right hemiclonic seizures with right-sided hemiparesis and edema of the left temporal-parietal cortex with associated leptomeningeal enhancement. Patient’s MOG antibody titer was 1:40. She completed high-dose corticosteroids and intravenous immunoglobulin. Our patient highlights the importance of MOG antibody testing in pediatric focal cortical encephalitis to avoid unnecessary anti-viral agents and provide more appropriate immunotherapy and a more informed prognosis.

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