Clinical characteristics of patients with polyarteritis nodosa based on a nationwide database in Japan

ABSTRACT Objectives This study aimed to clarify the epidemiological and clinical features and treatment of patients with polyarteritis nodosa (PAN) in Japan. Methods We used the database of the Ministry of Health, Labour and Welfare (MHLW) of Japan in 2013 and 2014. We analysed 121 patients who were antineutrophil cytoplasmic antibodies negative among the patients certified as PAN according to the MHLW diagnostic criteria. Results The analysis included 60 males and 61 females, with a mean age of 52.9 ± 21.0 years. As a general manifestation, fever was observed in 53.7%. Regarding organ involvement, skin manifestations (82.6%), joint and muscle manifestations (75.2%), and neuropsychiatric manifestations (50.4%) were common. Male patients had a higher proportion of mononeuritis multiplex involving motor neuropathy than female patients. Elderly patients had a higher proportion of general and respiratory manifestations. Glucocorticoids were used for treatment in all patients, and 19.0% underwent methylprednisolone pulse. Concomitant immunosuppressants were used in 25.6%, one-third of whom received cyclophosphamide. Methylprednisolone pulse and cyclophosphamide were mostly used in patients with life-threatening organ involvement. Conclusions PAN developed in middle-aged people and led to numerous clinical manifestations. The common manifestations varied with age, and treatment was determined based on the type of organ involvement and disease severity.

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Clinical presentations and long-term prognosis of childhood-onset polyarteritis nodosa in a single centre: a retrospective study

10.21203/rs.3.rs-62870/v1 ◽

2020 ◽

Author(s):

Jeong-Seon Lee ◽

JoongGon Kim ◽

Soyoung Lee

Keyword(s):

Polyarteritis Nodosa ◽

Treatment Guidelines ◽

Clinical Manifestations ◽

Diagnosis And Treatment ◽

Antineutrophil Cytoplasmic Antibodies ◽

Childhood Onset ◽

Single Centre ◽

Laboratory Findings ◽

Paediatric Patients

Abstract Background: Polyarteritis nodosa (PAN) is a systematic necrotizing vasculitis involving medium-sized arteries. Childhood-onset PAN ranges from mild to severe systematic disease causing damage and early mortality. As it is a rare disease in children, there is less study on clinical features, diagnosis and treatment than adults. And diagnosis occurs relatively late in most paediatric patients with polyarteritis nodosa. Therefore, this study aimed to investigate clinical manifestations, laboratory findings, treatment strategies, and long-term outcomes among patients with childhood-onset polyarteritis nodosa treated at a single-centre in Korea. We further aimed to evaluate the usefulness of the Birmingham Vasculitis Activity Score (BVAS) and Five-Factor Score (FFS) in paediatric patients.Methods: We retrospectively analysed data collected from patients with childhood-onset PAN treated at our institution from March 2003 to February 2020. Results: Nine patients (6 male and 3 female) were included in the study. The median ages at symptom onset and diagnosis were 7.6 (3–17.5) and 7.7 (3.5–17.6) years, respectively. The median follow-up duration was 7.0 (1.6–16.3) years. Eight patients were diagnosed with systemic PAN, while one was diagnosed with cutaneous PAN. All patients exhibited skin manifestations, while five exhibited Raynaud’s phenomenon. Organ involvement was observed in one patient. Constitutional symptoms such as fever, arthralgia, myalgia, and weight loss were also observed. Antineutrophil cytoplasmic antibodies were absent in all patients. The median BVAS at diagnosis was 8 (range: 2–29), and one patient had an FFS of 1. Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived despite some complications such as intracranial haemorrhage or digital necrosis requiring amputation.Conclusions: Early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. Further multicentre studies are required to clarify the unique characteristics of childhood-onset PAN and establish treatment guidelines, early detection strategies, and biomarkers. Trial registration: Retrospectively registered.

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Stage-Specific Gender Differences in Cognitive and Neuropsychiatric Manifestations of Vascular Dementia

American Journal of Alzheimer s Disease & Other Dementias® ◽

10.1177/1533317512454712 ◽

2012 ◽

Vol 27 (6) ◽

pp. 433-438 ◽

Cited By ~ 12

Author(s):

Yi Xing ◽

Cuibai Wei ◽

Changbiao Chu ◽

Aihong Zhou ◽

Fang Li ◽

...

Keyword(s):

Gender Differences ◽

Vascular Dementia ◽

Neuropsychiatric Symptoms ◽

Clinical Manifestations ◽

Male Predominance ◽

Gender Comparisons ◽

Verbal Recall ◽

Severe Stage ◽

Neuropsychiatric Manifestations ◽

Male Patients

Studies on gender differences in the clinical manifestations of vascular dementia (VaD) are still lacking. In the present study, gender comparisons of cognitive and neuropsychiatric profiles were conducted separately for mild and moderate-to-severe VaD in a total of 467 patients with VaD. There were no significant gender differences in cognitive manifestations, except that females performed better on immediate verbal recall than males in mild stage. Women were more likely to exhibit delusions (15.5% vs 7.4%), hallucinations (9.5% vs 3.4%), and depression (43.1% vs 27.3%) in mild stage. The predominance of male patients was observed in apathy at moderate-to-severe stage (50.5% vs 34.8%). To conclude, gender differences existed in neuropsychiatric symptoms of VaD and were especially pronounced in mild stage. Delusions, hallucinations, and depression were more prevalent in females in mild VaD, with the male predominance only in apathy in the later stage.

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Systemic vasculitis in a patient with rhupus syndrome

Reumatismo ◽

10.4081/reumatismo.2015.858 ◽

2016 ◽

Vol 67 (4) ◽

pp. 161 ◽

Cited By ~ 2

Author(s):

O. Zengin ◽

M. E. Onder ◽

M. A. Sarica ◽

I. H. Turkbeyler ◽

G. Kimyon ◽

...

Keyword(s):

Rheumatoid Arthritis ◽

Systemic Lupus Erythematosus ◽

Young Women ◽

Lupus Erythematosus ◽

Systemic Vasculitis ◽

Clinical Manifestations ◽

Organ Involvement ◽

Systemic Lupus ◽

Rare Syndrome ◽

Life Threatening

Rhupus is a rare syndrome characterized by overlap of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). Previous reports mentioned that rhupus patients have prominent RA associated clinical manifestations and only mild organic damage related to SLE. Progressive or life-threatening manifestations are rare in rhupus patients. Our patient diagnosed as rhupus was a young women, presented with multi-organ involvement of systemic vasculitis. Rheumatologists should be aware of possibility that rhupus may be accompanied by progressive or life-threatening conditions such as vasculitis.

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Eosinophilia: a pragmatic approach to diagnosis and treatment

Hematology ◽

10.1182/asheducation-2015.1.92 ◽

2015 ◽

Vol 2015 (1) ◽

pp. 92-97 ◽

Cited By ~ 18

Author(s):

Amy D. Klion

Keyword(s):

Clinical Manifestations ◽

Overwhelming Majority ◽

Practical Approach ◽

Diagnosis And Treatment ◽

Organ Involvement ◽

Endomyocardial Fibrosis ◽

Pragmatic Approach ◽

Recent Advances ◽

Choice Of Treatment ◽

Life Threatening

Abstract Eosinophilia is associated with a wide variety of allergic, rheumatologic, infectious, neoplastic, and rare idiopathic disorders. Clinical manifestations range from benign asymptomatic presentations to life-threatening complications, including endomyocardial fibrosis and thromboembolism. The prognosis and choice of treatment depend not only on the degree of eosinophilia and severity of organ involvement, but also on the etiology of the eosinophilia. Unfortunately, despite recent advances in molecular and immunologic techniques, the etiology remains unproven in the overwhelming majority of cases. This review presents a practical approach to the diagnosis and treatment of patients presenting with unexplained marked eosinophilia. A brief overview of the mechanisms of eosinophilia and eosinophil pathogenesis is also provided.

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Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea

Scientific Reports ◽

10.1038/s41598-021-87718-6 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Jeong-Seon Lee ◽

Joong-Gon Kim ◽

Soyoung Lee

Keyword(s):

Polyarteritis Nodosa ◽

Clinical Manifestations ◽

Long Term Outcomes ◽

Childhood Onset ◽

Laboratory Findings ◽

Clinical Presentations ◽

Long Term Prognosis ◽

Digital Amputation

AbstractChildhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been only a few reports because of its rarity. Thus, we aimed to investigate the clinical manifestations, laboratory findings, treatment, and long-term outcomes in patients with childhood-onset PAN and to evaluate the usefulness of the paediatric vasculitis activity score (PVAS). We retrospectively analysed the data of nine patients with childhood-onset PAN from March 2003 to February 2020. The median ages at symptom onset, diagnosis, and follow-up duration were 7.6 (3–17.5), 7.7 (3.5–17.6), and 7.0 (1.6–16.3) years, respectively. All patients had constitutional symptoms and skin manifestations, while five exhibited Raynaud’s phenomenon. Organ involvement was observed in one patient. The median PVAS at diagnosis was 7 (range: 2–32). Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived, but three patients with high PVAS at diagnosis experienced irreversible sequelae, including intracranial haemorrhage and digital amputation. In conclusion, early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. This study suggests that high PVAS score at diagnosis may be associated with poor prognosis.

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Diagnostic difficulties and features of endovideosurgical treatment of a patient with mucocele of the appendix

HERALD of North-Western State Medical University named after I I Mechnikov ◽

10.17816/mechnikov202012285-90 ◽

2020 ◽

Vol 12 (2) ◽

pp. 85-90

Author(s):

Badri V. Sigua ◽

Vyacheslav P. Zemlyanoy ◽

Elguja L. Lataria ◽

Alexey A. Kurkov ◽

Vyacheslav A. Melnikov ◽

...

Keyword(s):

Benign Tumor ◽

Abdominal Cavity ◽

Clinical Manifestations ◽

Vermiform Appendix ◽

Diagnostic Methods ◽

Number Of Patients ◽

Mucocele Of The Appendix ◽

Diagnostic Difficulties ◽

Life Threatening ◽

The Right

The mucocele of the appendix is the expansion of the appendix with the accumulation of a large amount of mucus. The mechanism and causes of mucocele are not fully understood. According to some authors, such changes in the appendix can occur due to cicatricial narrowing of the lumen of the appendix, compression or blockage of its base. Other authors believe that the mucocele of the appendix is a benign tumor that develops from the remnants of primitive mesenchyme and is sometimes prone to malignancy. Clinical manifestations of mucocele of the appendix are nonspecific. In a number of patients, this disease causes pain in the right abdomen, more often pulling, intermittent. However, the disease is often asymptomatic. In this regard, diagnosis is established only during performing an operation, most often, regarding acute appendicitis. Nevertheless, instrumental diagnostic methods such as ultrasound and computed tomography of the abdominal and pelvic organs make it possible to suspect mucocele. Despite the frequent asymptomatic, non-aggressive course, a number of life-threatening complications can become the outcome of the mucocele of the vermiform appendix. The most formidable complication is the rupture of the appendix with mucus entering free abdominal cavity, followed by the development of peritoneal pseudomyxoma due to implantation of mucus-forming cells. The only option for radical treatment of the mucocele of the appendix is a surgical intervention. A presented clinical case demonstrates the difficulties of diagnosis, as well as the features of surgical treatment of a patient with a mucocele of the appendix.

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Intracranial Haemorrhage as a Fatal Complication of Evans Syndrome: A Case Report

Nepal Medical Journal ◽

10.37080/nmj.76 ◽

2019 ◽

Vol 2 (2) ◽

pp. 66-69

Author(s):

Olita Shilpakar ◽

Bibek Rajbhandari ◽

Bipin Karki ◽

Umesh Bogati

Keyword(s):

Intracranial Haemorrhage ◽

Early Recognition ◽

Clinical Manifestations ◽

Health Care Facilities ◽

Evans Syndrome ◽

Fatal Complication ◽

Hematologic Disorder ◽

Immune Mediated ◽

Life Threatening ◽

Immune Neutropenia

Evans syndrome is a rare hematologic disorder characterized by the presence of simultaneous or sequential direct Coombs-positive autoimmune hemolytic anemia (AIHA), immune-mediated thrombocytopenia and/or immune neutropenia without any known underlying etiology. Spontaneous intracranial hemorrhage is a rare and life-threatening complication in patients with Evans syndrome and very few cases have been reported to date. We report a case of a thirty-two- year-old female with intracranial haemorrhage with underlying Evans syndrome who presented with the clinical manifestations of headache, vomiting and altered sensorium and succumbed to the fatal complication despite resuscitative measures. This also emphasizes the importance of early recognition of symptoms and immediate presentation to health care facilities for aggressive management of the patient.

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Sexual Dysfunction in Chronically Medicated Male Inpatients With Schizophrenia: Prevalence, Risk Factors, Clinical Manifestations, and Response to Sexual Arousal

Frontiers in Psychiatry ◽

10.3389/fpsyt.2021.761598 ◽

2022 ◽

Vol 12 ◽

Author(s):

Dianying Liu ◽

Shaohua Liu ◽

Meihong Xiu ◽

Hongdong Deng ◽

Huiyun Guo ◽

...

Keyword(s):

Risk Factors ◽

Sexual Dysfunction ◽

Sexual Arousal ◽

Negative Symptoms ◽

Sexual Experience ◽

Clinical Manifestations ◽

Common Symptom ◽

Erotic Stimuli ◽

Negative Factor ◽

Male Patients

BackgroundSexual dysfunction is a common symptom in patients with schizophrenia, especially in chronically medicated patients. However, the relationship between sexual dysfunction and emotional response to sexual arousal in male patients with schizophrenia remains unclear. This study aimed to assess the incidence, risk factors of sexual dysfunction in males, and their clinical correlations to sexual arousal in male patients with schizophrenia in China.MethodsA total of 162 male patients, aged 18–50 years, with schizophrenia were recruited from a psychiatric hospital in Ganzhou. The clinical symptoms were assessed by the Positive and Negative Syndrome Scale (PANSS). The Arizona Sexual Experience Scale was utilized to evaluate sexual dysfunction. Erotic images were selected from International Affective Picture System (IAPS). Sixty-eight out of the 162 subjects completed the erotic pictures reactivity task.ResultsOverall, 48 (29.6%) patients were measured as having global sexual dysfunction, 72 (44.4%) patients as having strength of sex drive dysfunction, 51 (31.5%) patients as having sexual arousal dysfunction, 55 (34.0%) patients as having penile erection dysfunction, 60 (37.0%) patients as having reached orgasm dysfunction, and 60 (37.0%) patients as having satisfaction with orgasm dysfunction. The sexual dysfunction patients had significantly higher scores on the negative symptoms of the PANSS. The only important predictor of sexual dysfunction was the severity of PANSS negative factor. The sense of pleasure and arousal post viewing erotic images in the sexual dysfunction group were lower compared to the non-sexual dysfunction group. The sense of pleasure and approach motivation were significantly negatively correlated with the severity of sexual dysfunction.ConclusionsThis study shows that nearly one-third of young and middle-aged chronically medicated male inpatients with schizophrenia suffer from sexual dysfunction. The negative factor of the PANSS can be regarded as the risk factor of sexual dysfunction. Schizophrenia patients with sexual dysfunction experienced lower pleasure and higher avoidance motivation than non-sexual dysfunction patients when exposed to erotic stimuli.

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Ovarian Torsion Presentation, Treatment and Iatrogenic Surgical Management

Journal of Pharmaceutical Research International ◽

10.9734/jpri/2021/v33i54a33720 ◽

2021 ◽

pp. 70-76

Author(s):

Sheema Sabahath ◽

Hussain Salah AL Sinan ◽

Asalah Tariq Alsaigh ◽

Rawan AlSalamah AlFadhli ◽

Tahani Salman Al Mansour ◽

...

Keyword(s):

Blood Flow ◽

Abdominal Pain ◽

Side Effects ◽

Adverse Events ◽

Surgical Intervention ◽

Clinical Manifestations ◽

Ovarian Torsion ◽

Adult Females ◽

Uterine Ligaments ◽

Life Threatening

Ovarian torsion is among the gynecological life-threatening conditions that may require urgent surgical intervention among the appearance of clinical manifestations. The most common clinical manifestations include severe abdominal pain, nausea extending to vomiting. The ovarian torsion is not limited to children only. However, it can also occur in adult females, either pregnant or non-pregnant. The etiology of the disease tends to be related to the weakness of the uterine ligaments or malpositioning of it due to known and unknown causes. Despite that, the surgical intervention is needed to release the torsion. Sometimes, it can lead to adverse events or side effects such as decreased blood flow to the surrounding structures. Which by role may lead to unpleasant complications and clinical manifestations of hemorrhage and shock. In this article, we reviewed the topic of ovarian torsion from different aspects, including the definition, causes, clinical evaluation, and clinical management and its common complications.

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Palmoplantar psoriasis: a comparative therapeutic study

International Journal of Research in Dermatology ◽

10.18203/issn.2455-4529.intjresdermatol20194668 ◽

2019 ◽

Vol 5 (4) ◽

pp. 779

Author(s):

Sathya Narayanan Rajendran ◽

Sukanya Mathupal Gurusamy

Keyword(s):

Treatment Options ◽

Clinical Manifestations ◽

Simple Random Sampling ◽

Inflammatory Dermatosis ◽

Duration Of Illness ◽

Erythrodermic Psoriasis ◽

Life Threatening ◽

Group A ◽

Group B ◽

Therapeutic Study

Background: Psoriasis is an immunologically mediated inflammatory dermatosis presenting with extremely variable clinical manifestations ranging from indolent lesions to life threatening forms of pustular and erythrodermic psoriasis. Palmoplantar psoriasis present as hyperkeratotic scaly plaques with fissures and can be managed with different treatment forms. This study was done to compare the efficacy between topical and systemic treatment options.Methods: The study was conducted in a tertiary level teaching hospital after ethical committee clearance. Fifty patients with palmoplantar psoriasis were allocated into the two groups using simple random sampling. PPPASI scoring was calculated to assess the extent of involvement. Patients in Group A were prescribed calcipotriol with clobetasol propionate ointment. Group B patients were given tablet methotrexate.Results: Twenty eight patients were males M: F of 1.27: 1. Mean age was 36. Mean duration of illness was eleven months. Most of these patients were manual laborers. 32 patients had lesions over both palms and soles, 9 over palms alone and 9 involving soles alone. Mean PASI reduction at 16 weeks was seen maximum with methotrexate. Compliance was comparatively good with methotrexate than topical. Though mean PASI reduction and compliance was good, relapse rates were higher with methotrexate in our study.Conclusions: There was no significant change in clinico-epidemiology and presentation of palmoplantar psoriasis. Methotrexate was observed to be the more efficacious modality in treating palmoplantar psoriasis.

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