Bilateral dacryoadenitis as initial presentation of a locally aggressive and unresponsive limited form of orbital granulomatosis with polyangiitis.

Limited orbital granulomatosis with polyangiitis (GPA) is uncommon and its diagnosis may be delayed, especially when isolated lacrimal involvement is the initial presentation, because clinical manifestations are non-specific and systemic diagnostic criteria are not applicable. Making an early diagnosis despite the absence of systemic progression is extremely important because in some cases the disease is locally destructive, with irreversible visual and functional loss, and it can be refractory to corticosteroids and conventional immunosuppressive drugs to induce remission. The authors report an unusual limited form of orbital GPA in a 35-year-old woman presenting with bilateral dacryoadenitis, evolving later to locally aggressive bilateral orbital pseudotumour leading to proptosis, extraocular myositis, diplopia and medial deviation of the nasal septum.

Giant high-pressure pulmonary artery aneurysm in an elderly patient with chronic obstructive pulmonary disease.

The authors report the case of a 74-year-old man, with a history of chronic obstructive pulmonary disease (COPD), GOLD grade 3, stable for the past two decades, who was admitted to our center with severe right heart failure. The chest radiograph showed moderate heart enlargement mainly of the right atrium and pulmonary artery, similar to previous chest radiographs in the previous 20 years. The transthoracic echocardiogram showed a pulmonary artery aneurysm (PAA), dilatation of the right chambers with pulmonary artery systolic pressure of 52 mmHg, and preserved right ventricular systolic function.

Belimumab in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is one of the most challenging autoimmune disorders with a complex pathophysiology and diverse clinical presentation. Many drugs have been used to treat SLE with suboptimal results, especially in patients with moderate-to-severe disease. Belimumab is the first biological drug to be approved for the treatment of SLE in more than 50 years.

Musculoskeletal complications of haematological disease.

Rheumatological manifestations complicate many benign and malignant blood disorders. Significant advances in haematology, with improved diagnostic techniques and newer musculoskeletal imaging, have occurred in the past two decades. This review focuses on the interrelationship between the major haematological diseases (haemochromatosis, haemophilia, sickle cell disease, thalassaemia, leukaemia, lymphoma, myelodysplastic syndromes, multiple myeloma and cryoglobulinaemia) and rheumatic manifestations.

B-cell survival factors in autoimmune rheumatic disorders.

Autoimmune rheumatic disorders have complex etiopathogenetic mechanisms in which B cells play a central role. The importance of factors stimulating B cells, notably the B-cell activating factor (BAFF) and A proliferation inducing ligand (APRIL) axis is now recognized. BAFF and APRIL are cytokines essential for B-cell proliferation and survival from the immature stages to the development of plasma cells.