Damage assessment in ANCA-associated vasculitis.

Antineutrophil cytoplasm antibody associated vasculitis has been transformed from life-threatening conditions to chronic relapsing long-term diseases as a result of significant advances in immunosuppressive therapy. Although mortality still occurs, it is much less frequent, with an average 5-year survival of over 70 %. In the setting of chronic conditions, it becomes increasingly important to monitor the burden of disease in terms of both active inflammation requiring immunosuppression and chronic damage (scarring) from vasculitis and its treatment and associated comorbidity. The damage that accumulates in patients with vasculitis does not respond to immunosuppressive treatment. It is important to distinguish disease activity from disease damage to prevent unnecessary immunosuppression, but it is equally important to recognize damage for what it is, so that it can be addressed appropriately. Damage is an inevitable consequence of long-term vasculitis for over 80 % of patients, which should not surprise us given the severity of the original illness. There is potential value in measuring damage as a means of providing prognostic information. Using a quantified score such as the Vasculitis Damage Index (VDI) allows us to predict mortality. Patients with at least five items of damage on the VDI score have substantially worse mortality (7- to 11-fold worse risk), as compared with those with lesser amounts of damage. These findings should be taken into context when planning the management of patients with vasculitis, as well as in clinical trials of vasculitis. Disease damage is an important surrogate for long-term outcome in vasculitis, and studies should be designed to limit the amount of damage accumulating as a result of therapeutic intervention, rather than simply controlling disease activity, as is currently the aim in recent randomized controlled trials in vasculitis. Furthermore, careful cataloguing of damage, as well as disease activity items, provides much greater detail in describing and observing the long-term natural history of primary systemic vasculitis in patients treated with immunosuppressive agents who survive their initial disease process.

The Role of Wrist Fusion and Wrist Arthroplasty in Rheumatoid Arthritis.

Rheumatoid arthritis is the most common form of inflammatory arthritis with a predilection for the hand and wrist. The aggressive nature of the disease can lead to severe joint destruction causing significant disability. Surgical options for pan-carpal arthritis include total wrist arthroplasty and total wrist fusion both with varying outcomes, yet both have a role in the carefully selected patients. Fusion remains a popular procedure with consistent reliable results with few complications. We present a review of current evidence, indications and guidance for both fusion and arthroplasty in rheumatoid arthritis.

Pulmonary Sarcoidosis following Etanercept Treatment.

Tumour necrosis factor (TNF) is an important cytokine involved in the pathology of a number of inflammatory conditions, and thus blockade with anti-TNF therapies is becoming the cornerstone in managing such diseases. With increasing use, evidence is collected for the association of sarcoid-like granulomatous disease developing after the initiation of anti-TNF-α therapy, with disease reversal after discontinuation.

Epididymo-orchitis as a presenting feature of relapsing polychondritis: a case report.

Relapsing polychondritis (RP) is a rare multisystem disease. It is characterized by recurrent inflammation of cartilaginous structures including the ears, nose, tracheo-bronchial tree and peripheral joints. Proteoglycan-rich structures such as the heart, eyes and blood vessels can also be affected. Systemic symptoms including fever, weight loss and lethargy are common. RP is difficult to diagnose as it presents in a wide variety of ways and there is no diagnostic test. Corticosteroids are the mainstay of treatment but other immunosuppressive drugs can be used in combination with steroids. We present an unusual presentation of RP.

An unusual cause of hearing loss in an 81-year-old man.

An 81-year-old man presented to accident and emergency with a recent history of rapidly progressive bilateral hearing loss. Further questioning revealed recent sinusitis, fevers and general malaise. Initial bloods showed raised inflammatory markers, raised creatinine, low albumin and urine dip was positive for blood and protein. Chest x-ray showed faint bilateral mid-zone infiltrates. He was treated with intravenous methylprednisolone (500 mg×3) for presumed Wegener's granulomatosis which was later confirmed by c-anti-neutrophil cytoplasmic antibodies testing (proteinase-3 positive) and supportive findings on CT chest and sinuses. He was discharged after 10 days in hospital on oral prednisolone and cyclophosphamide. He is continuing treatment and seen regularly in outpatient follow-up. Unfortunately his hearing has not fully recovered and he is awaiting a hearing aid.