Pulmonary vasculitis in Hughes-Stovin syndrome (HSS): a reference atlas and computed tomography pulmonary angiography guide-a report by the HSS International Study Group.

Introduction: Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by widespread venous/arterial thrombosis and pulmonary artery aneurysms (PAAs), which is associated with serious morbidity and mortality. All fatalities reported in HSS resulted from unpredictable fatal suffocating hemoptysis. Therefore, it is necessary to recognize pulmonary complications at an early stage of the disease.

A critical analysis of 57 cases of Hughes-Stovin syndrome (HSS). A report by the HSS International Study Group (HSSISG).

Background: Hughes-Stovin syndrome (HSS) is a systemic disease characterized by widespread vascular thrombosis and pulmonary vasculitis with serious morbidity and mortality. The HSS International Study Group is a multidisciplinary taskforce aiming to study HSS, in order to generate consensus recommendations regarding diagnosis and treatment.

Methods: We included 57 published cases of HSS (43 males) and collected data regarding: clinical presentation, associated complications, hemoptysis severity, laboratory and computed tomography pulmonary angiography (CTPA) findings, treatment modalities and cause of death.

[Primary aneurysm of the right common iliac artery].

We here report the case of a 55-year old patient, with a history of recurrent buccal aphthosis, hospitalized for pelvic pain of sudden onset. Clinical examination showed sensitive pulsatile pelvic mass with blowing sound suggesting vascular origin. Examination of external genitalia showed scrotal scar after healing of aphthous ulcers.

Antioxidants status in type 2 diabetic patients in Morocco.

Background/aim: Type 2 diabetes is a heterogeneous and multifactorial metabolic disorder with some relationship to oxidative stress (OS). Since no studies were conducted in the Moroccan population, this clinical investigation aimed at evaluating the antioxidants status in Moroccan patients with type 2 diabetes.

Materials And Methods: Blood samples of 60 type 2 diabetic patients and 40 healthy controls subjects were analyzed for determination of glycemia, hemoglobin, CRP, glycated hemoglobin, lipid parameters, malondialdehyde (MDA), vitamins E and C, copper (Cu), zinc (Zn), and selenium (Se).

[Ruptured renal artery aneurysms in microscopic polyangiitis].

Introduction: Renal artery aneurysms are unusual manifestations of microscopic polyangiitis.

Observation: We report the case of a 55 years old male patient, with a microscopic polyangiitis, which was revealed by a demyelinating polyradiculoneuropathy, the diagnosis was made with association of alveolar hemorrhage, peripheral neuropathy and glomerulonephritis with positive antineutrophil cytoplasmic antibodies (antimyeloperoxidase antibodies). The evolution was marked by a state of shock by rupture of aneurysms of intrarenal arteries.

[Ocular involvement in polyarteritis nodosa: two cases].

Introduction: Polyarteritis nodosa (PAN) is a systemic vasculitis with polymorphic manifestations. Ocular involvement is rare; we report two such cases.

Cases: The first case was a 56-year-old man with PAN treated with corticosteroids.

[Haemophagocytic syndrome as a complication of acute pancreatitis during systemic lupus erythematosus].

Background: Acute pancreatitis and haemophagocytic syndrome (HS) are rarely seen in systemic lupus erythematosus (SLE).

Patients And Methods: We report the case of a young female patient without any noteworthy prior history, who was hospitalised for abdominal pain associated with acute pancreatitis possibly related to SLE with associated haemophagocytic syndrome.

Discussion: Screening for cutaneous symptoms of lupus in patients with pancreatitis can help avoid diagnostic errors.

[Durable efficacity and remission after treatment with imatinib mesylate for FIP1L1-PDGFRA transcript negative associated eosinophilic cardiomyopathy].

Introduction: The cardiac involvement in hypereosinophilia remains a major cause of morbidity and mortality. Recent advances have identified new molecular mechanisms responsible for the expansion of the eosinophilic lineage, allowing a better classification of the different forms of Hypereosinophilic syndrome (HES) and especially targeted therapy. Since the discovery of the involvement of deregulated tyrosine kinases in the pathophysiology of these diseases, and particularly the identification of the fusion gene FIP1L1-PDGFRA, new molecules inhibiting specifically this signaling pathway (imatinib) were individualized, leading to dramatic therapeutic benefits in proliferative forms of HES considered before that of very poor prognosis.

[Ulcerative colitis: exceptional consequence after rituximab therapy].

Introduction: Possible adverse complications related to rituximab (RTX) are low, some of which are extremely rare. The authors describe one situation visibly waning exceptional treatment with RTX for SLE refractory to conventional therapies.

Comment: The authors report a patient of 34 years followed for months for an illness in its bullous lupus, with cutaneous, articular, hematologic and immunologic.

[Behcet syndrome: thirty comments with lung and vascular injury of peripheral vessels].

Introduction: Behcet's disease is a systematic vasculitis of unknown cause, characterized essentially by eye, cutaneous, articular, neurological and vascular manifestations.

Methods: We retrospectively analysed the Behcet's disease cases that were followed up in our ward from January 2000 to January 2009. The inclusion criteria were those of International Study Group on Behçet's disease (aphthosis mouth was required).

[Efficacity of rituximab in hemolytic anemia with cold autoantibodies case].

Introduction: Autoimmune hemolytic anemia with cold autoantibodies or cold agglutinin disease is a rare chronic disorder in which no treatment has, until now, evidence of its effectiveness.

Clinical Case: We report a patient who successfully responded to rituximab for a cold agglutinin disease refractory to conventional therapy with very good tolerance and a complete remission.

Conclusion: There are only few observations that have been reported in the literature regarding the efficacity of rituximab in the treatment of cold agglutinin disease.