Characteristics of Behcet's Disease in the American Southwest

Muruganandam, Maheswari; Rolle, Noelle A.; Sibbitt, Wilmer L.; Cook, Gladys B.; Emil, N. Suzanne; Fangtham, Monthida; Reiter, Kimberly J.; Bankhurst, Arthur D.

doi:10.1016/j.semarthrit.2019.03.003

Cited by 21 publications

(24 citation statements)

References 43 publications

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“…Patients with onset of scleroderma at greater than 37 and 42 years old respectively accounted for 95% and 85% of all cancer cases. It should be noted that the local Hispanic population typically also has Native American ancestry ranging from 15-80% [36]. This malignancy prevalence is similar to other reports cancer in scleroderma ranging from 7.1-20.0% [1][2][3][4][5][6][7][8][15][16][17][18][19][20][21][22][23][24].…”

Section: Resultssupporting

confidence: 82%

Scleroderma and Malignancy

Vondenberg

Cisneros

Kiani

et al. 2021

Preprint

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Background/ObjectivesDescribe clinical characteristics of malignancy-associated scleroderma in a regional population.MethodsScleroderma patients with current or past malignancy were prospectively identified from a cohort of 125 scleroderma subjects of a largely Hispanic population. Demographic information included scleroderma subtype, serologies, cancer diagnosis, age at diagnosis, co-morbid conditions, therapies, and survival outcomes.Results16.0% (20/125) of scleroderma patients were identified as having a malignancy. The mean age of malignancy onset and scleroderma onset were 55.6±10.3 and 52.7±12.9 years respectively (95% CI of difference: -4.6 <2.9< 10.4, p=0.44). Antinuclear antibody was positive in 70%, anticentromere antibody in 35.0%, and anti-topoisomerase antibodies in 25%. Tumor proportions were adenocarcinomas 55.0% (11/20) (1 gastric, 2 pancreatic, 1 lung, 2 thyroid, 5 breast), 15% (3/20) squamous cell carcinoma (1 skin, 1 uterine cervical, and 1 rectal), and 20% (4/20) benign tumors (2 meningiomas, 1 renal adenoma, 1 plasma cell). Age of onset of scleroderma greater than 42 years respectively accounted for 85% of all cancer cases. The standardized incidence ratio (SIR) was 2.0 (CI at 95%: 1.2<2<3.1), indicating 100% increase in cancer risk. Treatment of the malignancy did not resolve the scleroderma in any of the cases. Five-year survival after cancer diagnosis was 70.0%, although 2 of the survivors had tumor recurrence.ConclusionsMalignancy, especially adenocarcinoma, occurs frequently in scleroderma in minority populations with up to 16% of patients affected. High-risk patients are those with scleroderma-onset greater than 42 years in whom routine screening for common cancers should be undertaken.

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Section: Resultssupporting

confidence: 82%

Scleroderma and Malignancy

Vondenberg

Cisneros

Kiani

et al. 2021

Preprint

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“…Hydroxychloroquine is proven to have a relatively benign toxicity profile and a low incidence of infections as an immunosuppressive agent (48, 49). A large cohort study has reported the successful empiric use of hydroxychloroquine to treat BD (50).…”

Section: Discussionmentioning

confidence: 99%

Behcet's Disease With Cerebral Artery Infarction Caused by Cerebral Arteritis as an Early Symptom Only With Elevated Interleukin-8

et al. 2019

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Background: Behcet's disease (BD) is multi-systemic vasculitis, which generally is repeated oral and genital ulcerations as well as ocular and skin lesions. Today, the pathogenesis of BD remains mostly unknown. It is also suggested that the disease is probably related to autoinflammatory and autoimmune disorders, and innate immunity damages were perceived as key in its pathologic process. Only 5% of BD patients have neurological involvement, and it usually occurs in 4–6 years after the initial symptoms. Early onset of neurological impairment makes it difficult to diagnose and treat definitely.Case Presentation: A 38-year-old man was admitted to our hospital with numbness and weakness of the left extremities. Diffusion magnetic resonance imaging (MRI) revealed focal infarction in the posterior limb of the internal capsule. Skin pathology suggested small vessel vasculitis, and high-resolution MRI revealed intracranial arteritis. The patient had a negative skin pathery test and then developed a scar at the venous puncture site at the early stage of disease. Laboratory examination showed that interleukin 8 (IL-8) increased. The patient was treated with an immunosuppressive agent including mycophenolate mofetil, hydroxychloroquine, and colchicine. All symptoms were alleviated after half a year's treatment. There was neither stroke nor recurrence of oral ulcer thereafter.Conclusion: This case demonstrates that neurological involvement might be an early symptom of BD. IL-8 could act as a novel target for the treatment of BD theoretically and probably play a key role in disease recovery.

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“…Behçet's disease (BD) is an autoimmune, systemic disease that affects many organs and systems. 1,2 The International Study Group for BD has set criteria for the diagnosis of BD. In order to diagnose BD, in addition to oral ulcers, there should be at least two findings.…”

Section: Introductionmentioning

confidence: 99%

“…6 BD can be seen anywhere in the world, especially in countries on the historical Silk Road. 2 Besides genetic predisposition, immunological and environmental factors are held responsible. Although environmental factors are not known exactly, environmental pollution and infection agents are emphasized.…”

Section: Introductionmentioning

confidence: 99%

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Investigation of pregnancy associated plasma protein‐A and neopterin levels in Behçet's patients

Akyürek

2020

Dermatologic Therapy

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Behçet's disease (BD) is an autoimmune disease that affects many organs. We aimed to investigate the relationship between BD and these pregnancy‐associated plasma protein A (PAPP‐A), neopterin, and high sensitive C‐reactive protein (hsCRP) parameters. The study included 57 BD patients and 54 healthy controls. After evaluating the active and inactive disease status of the patients, analyzes were performed. When comparing the patient and control groups, neopterin (111.27 ± 37.49; 76.77 ± 38.27 [nmol/L]; P < .001) and hsCRP (11.81 ± 16.8; 3.62 ± 5.06 [mg/L]; P = .001) parameters were significantly higher in patients. Neopterin (117.68 ± 41.67; 94.85 ± 14.75 [nmol/L]; P = .038) and hsCRP (14.68 ± 18.7; 4.47 ± 7.27 [mg/L]; P = .002) found different in active and inactive patients. The sensitivities of neopterin and hsCRP were also found to be high in BD (respectively 93%, 67%). PAPP‐A was especially elevated in skin pathologies (P = .02) and neopterin in joint involvement (P = .03). We think that the use of neopterin and hsCRP can help in diagnosis and follow‐up of BD.

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Characteristics of Behcet's Disease in the American Southwest

Cited by 21 publications

References 43 publications

Scleroderma and Malignancy

Scleroderma and Malignancy

Behcet's Disease With Cerebral Artery Infarction Caused by Cerebral Arteritis as an Early Symptom Only With Elevated Interleukin-8

Investigation of pregnancy associated plasma protein‐A and neopterin levels in Behçet's patients

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