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.

Clinical and serological correlation of systemic sclerosis in Moroccan patients.

Objective: SSc is a CTD characterized by excessive fibrosis of the skin and internal organs, along with microvascular damage, and is often associated with typical autoantibodies. The aim of this study was to analyse the correlation between specific autoantibody profiles, clinical and paraclinical features in Moroccan patients with SSc. Methods: We analysed the presence of specific autoantibodies in 46 SSc patients using IIF on HEp-2 cells and immunodot. We then correlated the types of autoantibodies with clinical and laboratory manifestations. Results: Among our patients, 86.9% were females. The mean age of patients at diagnosis was 50.21 years, with an average delay to diagnosis of 5 years. The main clinical manifestations found were RP (89.2%), sclerodactyly (84.8%), proximal scleroderma (67.4%), gastrointestinal involvement (50%) and interstitial lung disease (30.4%). According to the specific autoantibody profile, 14 patients were anti-topo I positive (30.4%), 8 anti-RNP (68 kDa/A/C) positive (17.4%) and 6 anti-RNA polymerase III positive (13%). We found a significant association of anti-RNA polymerase III with sclerodactyly and pulmonary arterial hypertension (P < 0.05). We also found an association between anti-topo I and interstitial lung disease in 30.4% of patients. There was no significant association between the positivity for the autoantibodies and other diagnosed clinical manifestations. Conclusion: Some clinical manifestations of SSc might be positively correlated with the presence of specific autoantibodies. Environmental factors, ethnicity and gene interaction might also influence this correlation.

Budd-Chiari syndrome associated to Behcet disease: An observational retrospective multicenter study in Morocco.

Budd-Chiari syndrome (BCS) is considered a rare but serious complication of Behçet's disease (BD). This study was performed to define the prevalence, clinical and biological features, treatment, and clinical course of BSC associated with BD in a Moroccan population. We retrospectively analyzed the medical records of 1578 patients fulfilling the international diagnostic criteria for BD, including those with BSC. Eighteen male and 3 female patients, with a mean age of 36 ± 8.6 years. The inferior vena cava was involved in 81% (n = 17) of cases. All forms of BCS were noted: the chronic form in 52.4% (n = 11), the subacute form in 38% (n = 8), and the fulminant form (2 cases). Ascites was the main clinical sign and was present in 62% of patients (n = 13). Other venous thromboses (superior vena cava and lower limbs) were associated with BSC in 52.4% of patients (n = 11). Arterial involvement was noted in 28.6% (n = 6). Cardiac manifestations were present in 19% (n = 4) of the patients. All the patients received anticoagulants associated with corticosteroids. Immunosuppressants were used in 95% (n = 20). One patient received infliximab. Severe complications were noted in 38% (n = 8) of patients (digestive bleeding, confusion, infections and liver failure). Four patients have died during the study period. BCS in patients with BD is not uncommon and can be life threatening. It is frequently associated with other vascular manifestations that can be difficult to treat, particularly in the presence of pulmonary artery aneurysms. Prognosis improved with the use of immunosuppressants. Biologics can be promising in the early stages.

[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.