Moroccan parents caring for children with juvenile idiopathic arthritis: positive and negative aspects of their experiences.

BACKGROUND: Juvenile idiopathic arthritis (JIA) can lead to serious disability in children and adolescents, requiring intensive home care usually provided by parents .These parents must also cope with physical, familial, social and financial constraints.The aim of this study is to evaluate the positive and negative impacts of caregiving on parents to children with JIA, and identify diseases-related variables that affect these outcomes. METHODS: Cross-sectional study including 47 patients diagnosed with JIA defined by the International League of association for Rheumatology (ILAR) 2001 classification. Socio-demographic, clinical and biological data related to patient and disease were collected. Positive and negative effects of caregiving on parents of children with JIA were assessed via a validated instrument; the Caregiver Reaction Assessment (CRA).The CRA assesses parent's self-esteem, financial problems, health problems, disrupted schedule and lack of family support. All parents completed the CRA questionnaire. A statistical analysis was conducted to determine the influence of disease-related variables on caregivers. RESULTS: Forty-seven patients were included with 40.4% female. The average patient age was 11 years, and a mean patient body mass index (BMI) was 18. Forty patients were in school. Median disease duration of JIA was 4 years. The most frequent arthritis subtype was persistent oligoarthritis in 12-patients. Nearly 15% had extra-articular manifestations most frequently ocular involvement (6.4%). Median of global Visual analogic scale (VAS) was 20 and median Child health assessment questionnaire (CHAQ) was 0. The primary caregiver was the mother for all patients. Mean maternal age was 38 years, 42% of mothers were illiterate, and nearly all (95%) were without employment. The mean values of different dimensions of the CRA were respectively: self-esteem 3.5, financial problems 3.7, health problem 2.4, disrupted schedule 3.6 and familial support 2.9. Disrupted schedule of parents was correlated with disease severity assessed by physician VAS (p = 0.02). Financial problems of parents were significantly associated with disease duration (p = 0.04). There was no significant association between the type of JIA, activity or severity of the disease and other dimensions of the CRA. CONCLUSION: This study suggests that the management of children with JIA has a high negative impact among caregiving parents, represented mainly by the disruption of their activities, the lack of family support, financial problems and health problems. However, caregiving often also improves caregiver's self-esteem (feeling of gratification to be helping).

Stiff limb syndrome: a case report.

INTRODUCTION: Stiff limb syndrome is a clinical feature of the stiff person syndrome, which is a rare and disabling neurologic disorder characterized by muscle rigidity and episodic spasms that involve axial and limb musculature. It is an autoimmune disorder resulting in a malfunction of aminobutyric acid mediated inhibitory networks in the central nervous system. We describe a patient diagnosed by neurological symptoms of stiff limb syndrome with a good outcome after treatment, and a review of the related literature. CASE PRESENTATION: A 49-year-old male patient presented with a progressive stiffness and painful spasms of his both legs resulting in a difficulty of standing up and walking. The diagnosis of stiff limb syndrome was supported by the dramatically positive response to treatment using diazepam 25 mg/day and baclofen 30 mg/day. CONCLUSION: This clinical case highlights the importance of a therapeutic test to confirm the diagnosis of stiff limb syndrome especially when there is a high clinical suspicion with unremarkable electromyography.

Breast cancer in a male with ankylosing spondylitis treated with TNFalpha antagonists.

A 72-year-old male with a 4-year history of TNFalpha antagonist therapy (infliximab and etanercept) for ankylosing spondylitis was diagnosed with breast cancer. He had a family history of breast cancer. The low incidence and considerable severity of breast cancer in males, genetic risk factors, and potential role for TNFalpha antagonist therapy are discussed.

Amyopathic dermatomyositis.

UNLABELLED: Amyopathic dermatomyositis (ADM) is a rare condition characterized by skin lesions suggestive of dermatomyositis (DM) without detectable muscle abnormalities after at least 2 years of follow-up. Pulmonary fibrosis is uncommon in patients with ADM. CASE REPORT: A 64-year-old woman presented with a 2 years and 6 months history of nondestructive polyarthritis. She had skin changes suggestive of DM, including a pink rash over the face, neck, and forearms; Gottron's papules over the metacarpophalangeal joints; and heliotrope edema of the eyelids. She reported no muscle symptoms. Findings were normal from muscle enzyme assays, electromyography, and muscle biopsies. A diagnosis of ADM was given. Early lung fibrosis was found. Investigations for a tumor were negative. DISCUSSION: ADM is a rare condition that may be an abortive form of DM with a favorable outcome and a lower risk of malignancy compared to classic DM. However, the development of pulmonary fibrosis may cloud the prognosis.

Vascular involvement in Behçet's disease. Two case reports.

UNLABELLED: Arterial involvement is rare in Behçet's disease but can be at the forefront of the clinical picture and cause life-threatening complications. CASE REPORTS: A 36-year-old man had Behçet's disease with an aortographically documented aneurysm of the abdominal aorta as the inaugural manifestation. He had oral and genital ulcers. Funduscopy showed periphlebitis. In a 38-year-old man with an 8-year history of Behçet's disease, pulmonary and coronary artery aneurysms developed, as well as intracardiac and venous thromboses. DISCUSSION: Arterial involvement occurs in 3-5% of patients with Behçet's disease and usually manifests as multiple spindle-shaped aneurysms. Intracardiac thrombosis and cardiac aneurysm are exceedingly rare. Our patient had an extremely unusual presentation given the low rate of occurrence of arterial lesions in Behçet's disease. CONCLUSION: Arterial involvement in Behçet's disease raises treatment challenges because the lesions tend to recur and can cause life-threatening complications.