scholarly journals The Vogt-Koyanagi-Harada and SAPHO Syndrome: An Unusual Association

2019 ◽  
Vol 70 (9) ◽  
pp. 3441-3443
Author(s):  
Dragos Palade ◽  
Laura Otilia Damian ◽  
Doinel Radeanu ◽  
Anne Claudia Stefanut ◽  
Gheorghe Cobzac ◽  
...  
Keyword(s):  
Late Phase ◽  
Sapho Syndrome ◽  
High Dose ◽  
Autoimmune Reaction ◽  
Unusual Association ◽  
Neurologic Involvement ◽  
Undetectable Viremia ◽  
History Of ◽  
Inflammatory Bowel ◽  
Common Pathogenesis

Vogt-Koyanagi-Harada syndrome(VKH) is a multisystemic disorder characterized by granulomatous panuveitis and cutaneous, otorhynolaryngologic and neurologic involvement, due to an autoimmune reaction against melanocytic antigens. Autoinflammatory associations of VKH, including inflammatory bowel disease and psoriasis, were rarely described. SAPHO syndrome (an acronym for synovitis, acne, hyperostosis, pustulosis and osteitis) is a rare autoinflammatorymusculo-scheletal disease. This paper reports on a male patient presenting for long-lasting bone and joint pain, dysphagia,having a ten-year history of bilateral optic neuritiswhich required high-dose corticosteroids. Fundoscopy revealed diffuse chorioretinalnonpigmented atrophic lesions. Vitiligo, acne scars, clavicular deformity, recurrent chest pain and sacroiliitis pointed to a long-standing SAPHO syndrome and late-phase VKH. Blood tests found inflammation and anti-hepatitis C antibodies with repeatedly undetectable viremia. Bone scintigraphy with the characteristic bull�s head upper chest uptake and the radiographs confirmed SAPHO syndrome. The patient was treated with sulphasalazine and cyclosporin A, with alleviation of the articular and bony disease. In conclusion, SAPHO syndrome may add to the list of the diseases associated with VKH. Although VKH and SAPHO syndrome may have appeared coincidentally, a common pathogenesis involving an infectious trigger and IL-17 could be considered.

scholarly journals A218 LISTERIA MONOCYTOGENES MENINGITIS IN A NEWLY DIAGNOSED PATIENT WITH INFLAMMATORY BOWEL DISEASE: A CASE REPORT

2020 ◽  
Vol 3 (Supplement_1) ◽  
pp. 92-93
Author(s):  
M Ayoub ◽  
T Walters
Keyword(s):  
Inflammatory Bowel Disease ◽  
Case Report ◽  
Listeria Monocytogenes ◽  
Bowel Disease ◽  
Inflammatory Markers ◽  
Opportunistic Infections ◽  
Brain Mri ◽  
High Dose ◽  
History Of ◽  
Inflammatory Bowel

Abstract Background Anti-Tumor Necrosis Factor (TNF) is a frequently utilized therapy in the treatment of Inflammatory Bowel Disease (IBD). Opportunistic infections are a known but an infrequently encountered complication. Listeria monocytogenes (LM) is an aerobic gram-positive intracellular bacillus. Clinical presentation of infection is host dependent, ranging from self-limited illnesses in immunocompetent individuals to life-threatening sepsis and meningitis in the immunocompromised. TNF plays a crucial role in host’s defense against LM. Although published in adults, few case reports have documented invasive LM in children receiving infliximab (IFX), an anti-TNF agent used in IBD therapy. Aims Describe an adolescent with IBD-unclassified (IBD-U) in whom LM sepsis and meningitis was diagnosed after induction therapy with intravenous (IV) IFX. Methods Case report and literature review. Results A 15-year-old girl presented with 2-week history of progressive abdominal pain, bloody diarrhea, urgency, nocturnal stooling, tenesmus, and weight loss. She had been afebrile, with no history of exposures. Examination revealed pallor and tenderness in the right and left lower quadrants. Investigations showed elevated white cell count (WBC), platelets, inflammatory markers, and low albumin. Abdominal ultrasound showed thickening of the descending and sigmoid colon. Stool multiplex PCR was negative. Colonoscopy showed Mayo 3 pancolitis. The terminal ileum was not intubated and endoscopy was normal. She was diagnosed with IBD-U. Due to poor response to high dose IV steroids, IV IFX (after documenting normal vaccination titers) was given with good clinical response and no adverse effects; she was discharged on tapering prednisone. She re-presented 3 days later with fever, severe headaches, photophobia, and neck stiffness. Her IBD remained quiescent. She had consumed a cold meat sandwich 10 days prior. Antibiotics were started as investigations showed leukocytosis and very high inflammatory markers. Brain MRI showed pus in the lateral ventricles. Cerebrospinal fluid (CSF) analysis showed WBC 1832 x106/L, low glucose, and high protein. Blood and CSF cultures detected LM. She defervesced within 24 hours and completed a 21-day course of Ampicillin monotherapy. 2-month follow up showed IBD in continuous remission on IFX with no neurological sequalae, and a normal brain MRI. Conclusions This is the youngest patient with IBD reported with invasive listeriosis secondary to IFX and adds to 3 cases in children. It highlights the importance of vigilance when evaluating IBD patients with fever during IFX-based therapy. Physicians should be reminded of such patients’ immunocompromised state and their high risk of acquiring opportunistic infections. It is unclear if listeriosis avoidance precautions, currently recommended in pregnant women, should be adopted in patients receiving anti-TNF therapy. Funding Agencies None

scholarly journals The Role of Vitamin D Deficiency Prevention in Improving Obstetric, Perinatal and Long-Term Outcomes

2020 ◽  
pp. 35-40
Author(s):  
Iu.V. Davydova ◽  
◽  
A.Iu. Lymanska ◽  
Yu.P. Neroznak ◽  
◽  
...  
Keyword(s):  
Vitamin D ◽  
Vitamin D Deficiency ◽  
Vitamin D3 ◽  
First Trimester ◽  
Soluble Form ◽  
High Dose ◽  
Fetoplacental Complex ◽  
History Of ◽  
Inflammatory Bowel

Much attention is paid to deficiencies prevention during pregnancy, since they negatively affect not only metabolic processes, including fetoplacental complex, but also the condition of the fetus, the newborn, and physical and mental development of the child in the future. Summarizing the recommendations for the use of vitamin D during pregnancy, it should be noted that the typical prophylactic dose is 400 IU/day starting from the first trimester. However, we should consider the use of corrective doses in the second and third trimesters when the fetal skeleton is growing and developing. A high dose of vitamin D (2000 IU/day) is recommended for women at high risk of developing hypertensive complications of pregnancy (a history of preeclampsia, in particular, arising before 28 weeks, arterial hypertension, chronic kidney disease), as well as pregnant women with comorbidity (systemic red lupus, rheumatoid arthritis, multiple sclerosis, cardiovascular disease, inflammatory bowel disease, oncological pathology). For women at risk of vitamin D deficiency, the recommended dose is less than 1000 IU/day. In case of confirmed vitamin D deficiency (<25-30 nmol/L), a correction dose of 2000-4000 IU/day is prescribed for 11 weeks to provide a cumulative dose of about 150,000 or 300,000 units in the second or third trimesters. Today, a sufficient number of vitamin D preparations are presented on the pharmaceutical market of Ukraine, among which one should pay attention to "Olidetrim" (Polpharma, Poland) in the form of oil solution in capsules ensuring its absorption and assimilation. Depending on the dosage, each capsule of the preparation contains 2000 IU or 4000 IU of vitamin D3 (cholecalciferol). In addition to the fat-soluble form, vitamin D preparation Aquadetrim® (Polpharma, Poland) is registered and successfully used in Ukraine. This is a unique form of vitamin D3 based on nanomycellae, which provides better absorption of vitamin D in the intestine. Key words: vitamin D, deficiency, pregnancy, fetus, prevention.

ITI with high-dose FIX and combined immunosuppressive therapy in a patient with severe haemophilia B and inhibitor

2009 ◽  
Vol 29 (02) ◽  
pp. 155-157 ◽  
Author(s):  
H. Hauch ◽  
J. Rischewski ◽  
U. Kordes ◽  
J. Schneppenheim ◽  
R. Schneppenheim ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Immunosuppressive Agents ◽  
Tolerance Induction ◽  
Intravenous Immunoglobulins ◽  
Factor Ix ◽  
High Dose ◽  
Allergic Reactions ◽  
Success Rates ◽  
Serious Infections ◽  
History Of

SummaryInhibitor development is a rare but serious event in hemophilia B patients. Management is hampered by the frequent occurrence of allergic reactions to factor IX, low success rates of current inhibitor elimination protocols and the risk of development of nephrotic syndrome. Single cases of immune tolerance induction (ITI) including immunosuppressive agents like mycophenolat mofetil (MMF) or rituximab have been reported. We present a case of successful inhibitor elimination with a combined immune-modulating therapy and high-dose factor IX (FIX). This boy had developed a FIX inhibitor at the age of 5 years and had a history of allergic reactions to FIX and to FEIBA→. Under on-demand treatment with recombinant activated FVII the inhibitor became undetectable but the boy suffered from multiple joint and muscle bleeds. At the age of 11.5 years ITI was attempted with a combination of rituximab, MMF, dexamethasone, intravenous immunoglobulins and high-dose FIX. The inhibitor did not reappear and FIX half-life normalized. No allergic reaction, no signs of nephrotic syndrome and no serious infections were observed.

Corticosteroids in Inflammatory Bowel Disease patients: a practical guide for physicians.

2020 ◽  
Vol 15 ◽  
Author(s):  
Maria Carla Di Paolo ◽  
Cristiano Pagnini ◽  
Maria Giovanna Graziani
Keyword(s):  
Severe Disease ◽  
Unknown Etiology ◽  
History Of Disease ◽  
Bowel Diseases ◽  
Literature Evidence ◽  
History Of ◽  
Inflammatory Bowel ◽  
Favorable Safety ◽  
Pass Metabolism

: Inflammatory bowel diseases (IBDs) are chronic conditions characterized by unknown etiology and pathogenesis with deregulation of mucosal immunity. Among possible treatments, corticosteroids, already available from the 50’, are still the mainstay of treatment for moderate-severe disease. Nonetheless, the use of steroids is still largely empirical and solid evidence about therapeutic schemes are lacking. Moreover, due to the important side-effects and for the unsatisfactory impact on long-term natural history of disease, the steroid sparing has become an important therapeutic goal in IBD management. Besides conventional steroids, the so called “low bioavailability” steroids, which are steroids with high affinity for peripheral receptors and elevated hepatic first-pass metabolism, have demonstrated efficacy and more favorable safety profile. In the present review of the literature evidence of efficacy and safety of conventional and low bioavailability steroids in IBD patients are evaluated, and practical suggestions for a correct use in clinical practice are presented according to the current clinical guidelines.

Pseudo-Parkinson Disease Secondary to Ritonavir–Buspirone Interaction

2003 ◽  
Vol 37 (2) ◽  
pp. 202-205 ◽  
Author(s):  
Patrick G Clay ◽  
Molly M Adams
Keyword(s):  
Drug Interaction ◽  
Inhibitory Effect ◽  
Hiv Positive ◽  
High Dose ◽  
Resting Tremor ◽  
Case Summary ◽  
History Of ◽  
Drug Drug Interaction ◽  
To Receive

OBJECTIVE: To report a case of Parkinson-like symptoms appearing in a patient after introduction of ritonavir to buspirone therapy. CASE SUMMARY: A 54-year-old HIV-positive white man presented to the clinic with a 2-week history of ataxia, shuffling gait, cogwheel rigidity, resting tremor, and sad affect with masked features. This patient had been receiving high-dose buspirone (40 mg every morning and 30 mg every evening) for 2 years prior to the introduction of ritonavir/indinavir combination therapy (400 mg/400 mg twice daily) 6 weeks prior to initiation of the above symptoms. Buspirone was decreased to 15 mg 3 times daily, ritonavir/indinavir was discontinued, and amprenavir 1200 mg twice daily was added. The patient's symptoms began to subside after 1 week, with complete resolution after about 2 weeks. The patient continued to receive buspirone for an additional 12 months without recurrence of symptoms. DISCUSSION: This is the first reported interaction of buspirone and antiretrovirals. Buspirone, extensively metabolized by CYP3A4, was likely at supratherapeutic levels due to the inhibitory effect of ritonavir and, secondarily, indinavir. The Parkinson-like symptoms developed rapidly and severely, impacted this patient's quality of life, and necessitated significant clinic expenditures to identify this drug–drug interaction. CONCLUSIONS: This case demonstrates a severe drug–drug interaction between buspirone and ritonavir and further demonstrates the need for awareness of the metabolic profile for all agents an HIV-infected patient is receiving.

scholarly journals SAT0462 ASSESSMENT OF BONE MINERAL DENSITY IN INFLAMMATORY BOWEL DISEASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1188.1-1188
Author(s):  
C. Daldoul ◽  
N. El Amri ◽  
K. Baccouche ◽  
H. Zeglaoui ◽  
E. Bouajina
Keyword(s):  
Bone Mineral Density ◽  
Inflammatory Bowel Disease ◽  
Bone Mineral ◽  
Bowel Disease ◽  
Mineral Density ◽  
Femoral Site ◽  
Significant Difference ◽  
History Of ◽  
Inflammatory Bowel ◽  
The Mean

Background:Inflammatory bowel disease (IBD), including ulcerative colitis (UC) and Crohn’s disease (CD), is considered as a risk factor of low bone mineral density (BMD). In fact, the prevalence of osteoporosis ranges from 17% to 41% in IBD patients. The possible contributing factors may include malabsorption, glucocorticoid treatment and coexisting comorbiditiesObjectives:The purpose of our work was to determine the frequency and the determinants of osteoporosis in patients with IBD and to assess whether there is a difference in BMD status between UC and CD.Methods:This is a retrospective study, over a period of 5 years (from January 2014 to December 2018) and including patients followed for IBD who had a measurement of BMD by DEXA. Clinical, anthropometric and densitometric data (BMD at the femoral and vertebral site) were recorded. The WHO criteria for the definition of osteoporosis and osteopenia were applied.Results:One hundred and five patients were collected; among them 45 were men and 60 were women. The average age was 45.89 years old. The average body mass index (BMI) was 25.81 kg/m2 [16.44-44.15]. CD and UC were diagnosed in respectively 57.1% and 42.9%. A personal history of fragility fracture was noted in 4.8%. Hypothyroidism was associated in one case. Early menopause was recorded in 7.6%. 46.8% patients were treated with corticosteroids. The mean BMD at the vertebral site was 1.023 g/cm3 [0.569-1.489 g/cm3]. Mean BMD at the femoral site was 0.920g/cm3 [0.553-1.286g / cm3]. The mean T-score at the femoral site and the vertebral site were -1.04 SD and -1.27 SD, respectively. Osteoporosis was found in 25.7% and osteopenia in 37.1%. Osteoporosis among CD and UC patients was found in respectively 63% and 37%. The age of the osteoporotic patients was significantly higher compared to those who were not osteoporotic (52.23 vs 43.67 years, p = 0.01). We found a significantly higher percentage of osteoporosis among men compared to women (35.6% vs 18.3%, p=0.046). The BMI was significantly lower in the osteoporotic patients (23.87 vs 26.48 kg/m2, p=0.035) and we found a significant correlation between BMI and BMD at the femoral site (p=0.01). No increase in the frequency of osteoporosis was noted in patients treated with corticosteroids (27.9% vs 21.6%, p=0.479). Comparing the UC and CD patients, no difference was found in baseline characteristics, use of steroids or history of fracture. No statistically significant difference was found between UC and CD patients for osteoporosis(p=0.478), BMD at the femoral site (p=0.529) and at the vertebral site (p=0.568).Conclusion:Osteoporosis was found in 25.7% of IBD patients without any difference between CD and UC. This decline does not seem to be related to the treatment with corticosteroids but rather to the disease itself. Hence the interest of an early screening of this silent disease.Disclosure of Interests:None declared

scholarly journals A102 ADVERSE PREGNANCY-RELATED OUTCOMES IN WOMEN WITH INFLAMMATORY BOWEL DISEASE

2021 ◽  
Vol 4 (Supplement_1) ◽  
pp. 77-79
Author(s):  
Y Hanna ◽  
P Tandon ◽  
V W Huang
Keyword(s):  
Inflammatory Bowel Disease ◽  
Disease Activity ◽  
Bowel Disease ◽  
Pregnancy Outcomes ◽  
Adverse Pregnancy Outcomes ◽  
Categorical Variables ◽  
History Of ◽  
Inflammatory Bowel ◽  
Adverse Pregnancy ◽  
Aspirin Prophylaxis

Abstract Background Women with active inflammatory bowel disease (IBD) are at increased risk of adverse pregnancy outcomes such as preeclampsia. Though aspirin prophylaxis is prescribed in the general population (prior to 16 weeks’ gestation) for those at high-risk of preeclampsia, its use in patients with IBD has not been established. Aims To determine the frequency of and risk factors for adverse pregnancy outcomes in women with IBD, and to evaluate the risk for preeclampsia and the use of aspirin for primary prevention. Methods All pregnant women with IBD (Crohns disease (CD), ulcerative colitis (UC) and IBD-unclassified (IBDU)) seen at Mount Sinai Hospital from 2016–2020 were retrospectively identified. Demographics, reproductive history, and IBD characteristics including therapy and activity during pregnancy were recorded. Adverse pregnancy outcomes were also identified. Active disease during pregnancy was defined as a fecal calprotectin &gt; 250 ug/g and/or using clinical disease activity scores. Categorical variables were compared using the Chi-square (x2) test and continuous variables using the Mann-Whitney test. A two-sided p-value less than 0.05 was considered statistically significant. Results 127 patients (66 with CD, 60 with UC, 1 with IBDU) were included with a median age of 32 years at conception. The majority were Caucasian (70.9%), married (82.7%), completed post-secondary education (69.3%), had no prior or current smoking (78.7%) or alcohol use history (67.7%), and had no other comorbidities (81.9%). 50.4% of women had a prior pregnancy. 3 had a history of preeclampsia and 15/127 were prescribed aspirin prophylaxis. 73.2% of women were in clinical remission at conception. Compared to women with CD, women with UC were more likely to have infants with low birth weight (LBW) (p=0.031), small for gestational age (SGA) (p=0.002) and had higher rates of active IBD during pregnancy (p=0.005). 13 women with IBD developed preeclampsia (6 with UC and 7 with CD). IBD type (p=0.844) and disease activity (p=0.308) were not associated with preeclampsia. Married women (p=0.001) while those who had a preconception consultation (50/127) (p=0.009) had lower rates of preeclampsia while those with a prior history of preeclampsia had higher rates (p=0.002). Among women who developed preeclampsia, pregnancy outcomes were comparable to those who did not. Women on aspirin prophylaxis (5/13) had a higher rate of preeclampsia (p=0.012), although they were also more likely to have a history of preeclampsia (p=0.002). Aspirin use was not associated with subsequent disease activity in pregnancy (p=0.830). Conclusions Women receiving aspirin prophylaxis had higher rates of preeclampsia, likely owing to a higher baseline risk. Preeclampsia prevention with aspirin prophylaxis does not appear to result in disease flares but larger studies are needed to confirm this finding. Funding Agencies None

929 Not Another Abscess - A Case of Streptococcal Myositis Misdiagnosed as a Right Axillary Abscess

2021 ◽  
Vol 108 (Supplement_2) ◽  
Author(s):  
O Oyende ◽  
J Jackman
Keyword(s):  
White Cell Count ◽  
General Surgeon ◽  
High Dose ◽  
General Anaesthetic ◽  
Microbiological Analysis ◽  
Reactive Protein ◽  
Affected Tissue ◽  
Life Threatening ◽  
History Of ◽  
High Degree

Abstract Introduction Streptococcal myositis is a rare form of infectious myositis caused by Lansfield A beta-haemolytic streptococci. It is characterised by rapidly spreading inflammation that can result in severe systemic toxicity and necrosis of the affected tissue if not diagnosed and aggressively treated. Presentation We report a case of a 42-year-old male who presented with a one-week history of worsening right axillary swelling that progressed to painful swelling of his arm. Inflammatory markers were significantly elevated with a white cell count of 17 ×109/L and C-reactive protein of 212 mg/L. On examination, a fluctuant axillary swelling was appreciated, and a decision was made for incision and drainage under general anaesthetic. Intraoperative aspiration of his arm revealed copious purulent fluid prompting intraoperative orthopaedic consult and exploration of the anterior compartment in which there was extensive involvement of the biceps muscle. The microbiological analysis revealed gram-positive cocci in chains, and microbiology advice sought for tailoring of antibiotic regimen. He has recovered well. Discussion Though uncommon, the emergency general surgeon should have a high degree of suspicion when evaluating soft tissue infections to avert potentially disastrous outcomes. Conclusion Early diagnosis, aggressive management with high-dose intravenous antibiotics, and surgical debridement are principles to treat this rare, life-threatening infection.

A Rare Case of Acute Interstitial Pneumonia Diagnosed at Autopsy

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S153-S154
Author(s):  
E Conner ◽  
D Troxclair ◽  
H Khokhar ◽  
W Beversdorf
Keyword(s):  
Respiratory Failure ◽  
Interstitial Pneumonia ◽  
Diffuse Alveolar Damage ◽  
Ventilatory Support ◽  
Respiratory Illness ◽  
High Dose ◽  
Atypical Pneumonia ◽  
Acute Interstitial Pneumonia ◽  
Diagnostic Role ◽  
History Of

Abstract Introduction/Objective Acute interstitial pneumonia (AIP) is a rare disease clinically characterized by rapidly progressing respiratory failure in individuals with no history of respiratory illness or other inciting factors. While most often diagnosed in middle-aged adults, it may present in any age group. Initial presentation is described as influenza- like, and respiratory failure requiring ventilatory support often progresses within weeks to months. Prognosis is poor, with an estimated mortality rate approaching 80% without treatment. Methods We present the case of a 44-year-old male nonsmoker with no significant medical history, who presented in 2018 with 1.5 months of dyspnea and headache initially diagnosed as atypical pneumonia. Chest imaging revealed bilateral opacities; however, microbial workup revealed no evidence of infectious etiology. Autoimmune serology studies were likewise unrevealing. Despite aggressive supportive and medical management, he deteriorated to respiratory failure and succumbed. Results At autopsy, the lungs were symmetrically congested and edematous (combined weight 2,340 g) but free of evident consolidation or discrete lesions. Microscopic examination revealed diffuse alveolar damage with extensive hyaline membrane formation, interstitial edema, and fibroblastic proliferation. The vasculature was severely congested, and the alveoli contained hemorrhage and scattered macrophages. No fungal or mycobacterial elements were identified by staining. Based on the histologic features and clinical context, the diagnosis of AIP was made. Conclusion AIP is a rare, aggressive, and diagnostically challenging disease that includes a broad range of both clinical and histologic differentials. Timely recognition and intervention with aggressive respiratory support and high- dose glucocorticoids are the mainstays of clinical management. The diagnostic role of histology is significant, but hinges on early clinical consideration of AIP as disease progression may later preclude the biopsy procedure. We share this case to raise awareness of this rapidly progressive and diagnostically troubling interstitial lung disease while emphasizing the importance of clinicopathologic correlation.

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