A rare clinical case of systemic AA amyloidosis with cardiac involvement complicating ankylosing spondylitis: a case report.

BACKGROUND: Ankylosing spondylitis (AS) is a type of chronic inflammation that is most prevalent in young adults and is characterized by an inflammatory enthesiopathy that gradually develops toward ossification and ankylosis. If inflammation is left unchecked, it can potentially lead to complications such as secondary amyloidosis, also known as AA amyloidosis, involving the deposition of amyloid serum A protein. Our case presents with a thyroid localization of AA amyloidosis which is secondary to this AS. Such a case has been described in only four cases in the literature. Cardiac localization of AA amyloidosis has been exceptionally described in the literature. CASE PRESENTATION: We report the case of a young patient with severe AS complicated by secondary amyloidosis with thyroid, cardiac, and probably renal localization. He was treated with anti-TNF therapy, and his condition improved significantly. CONCLUSIONS: Our case presents several localizations of AA amyloidosis secondary to this AS. Although cardiac involvement is rare in secondary AA amyloidosis, it should always be screened for, even in a cardiacly asymptomatic patient.

Classification of common variable immunodeficiency through immunological and clinical phenotyping in Moroccan patients.

Objective: Common variable immunodeficiency (CVID) is a complex inborn error of humoral immunity with complications of both infectious and non-infectious origins. Classifications of CVID patients provide a clearer understanding of the pathogenesis, prediction, and management of non-infectious complications. This study aims to classify Moroccan CVID patients based on the European classification (EUROclass). Materials and Methods: We recruited 20 CVID patients meeting standard diagnostic criteria (5-6). After collecting clinical and demographic data, we used flow cytometry to analyze B-cell subsets and group patients and assess the relation of each group with clinical manifestations. Results: 90% of the patients in our cohort study had a history of respiratory infections. The noninfectious manifestations included splenomegaly, autoimmunity, lymphadenopathy, and granulomatous diseases diagnosed in 50%, 45%, 40%, and 25% of patients, respectively. We observed significant co-occurrence of splenomegaly with autoimmunity and granulomatous diseases to a lesser extent. Patients had a significant reduction in total, switched memory, marginal zone-like, plasma blasts, and a substantial increase in the percentage of activated B cells, suggesting a defect in the late phases of B-cell differentiation. This condition was linked with an increased occurrence of splenomegaly and granulomatous affections. Besides, patients also had an expansion of CD21low B-cells, which was strongly associated with splenomegaly. Conclusion: The classification of the first Moroccan cohort of CVID patients showed agreement with previous results. It suggests the possibility of adopting this approach on a global scale for better diagnosis and follow-up of CVID patients.

[Causes of stroke among young people: role of the internist]. / Etiologies des accidents vasculaires cérébraux ischémiques chez les jeunes: apport de l'interniste.

In young people brain ischemias vary according to their cause and prognosis. In internal medicine they have a specific cause, considering the tertiary source of recruitment. Our study aimed to provide informations about some specific causes of this disease in young subject. We conducted a retrospective study by reviewing the medical records of young patients hospitalized at the Department of Internal Medicine in Casablanca over the period 2000-2014. All patients underwent CT scan angiography and/or magnetic resonance angiography that determined the nature and the topography of the ischemic stroke. Clinical examination was followed by appropriate investigations to determine the cause of brain ischemia. Data were collected from twenty-five patients, with a sex ratio of 0.73 and an average age of 36±7. Smoking was reported in 32% of cases, diabetes and high blood pressure were found in 8% of cases. Amnesia and migraine episodes were found in 24% of cases. Combination estrogen-progestin and a history of miscarriage were reportedin 12% of cases. Ischemic strokes were mainly caused by acute systemic lupus (32%) associated with antiphospholipid syndrome (80%), Behcet's disease (16%), Takayasu's disease (12%). In addition to anti-aggregation treatment, 76% of patients underwent corticosteroid and immunosuppressive therapies. Internists are involved in the treatment of patients with ischemic stroke, in particular to determine its cause. The causes of brain ischemias in young people are multiple. Research has to be rigorous in order to identify specific causes, to evaluate the risk of recidivism and to establish a therapeutic approach.

A Misleading Diagnosis of Sarcoidosis in an Older Woman.

Common variable immunodeficiency (CVID) is the most frequent primary immunodeficiency and has a reported prevalence of approximately 1:25,000 to 1:50,000. The fact that it is rarely considered as a diagnosis in adults can lead to diagnostic delay, especially in older patients, and to complications such as bronchiectasis and excess mortality. However, practitioners should first exclude common causes of hypogammaglobulinaemia before considering CVID. Here we present a case of CVID revealed by prolonged fever and complicated with granulomatous manifestations and bronchiectasis in an older woman without a history of recurrent infections. LEARNING POINTS: Common variable immunodeficiency (CVID) should be considered in atypical cases with unexplained chronic signs such as fever of unknown origin (even in older patients) after tuberculosis, HIV, neoplasia and connective tissue disease have been ruled out.Common causes of hypogammaglobulinaemia should be excluded before CVID is considered.CVID can mimic sarcoidosis.