Systemic sclerosis (SSc) is a connective tissue disease which is characterized by endothelium dysfunction, inflammation and fibrosis. Although scleroderma is often presented as an overlap syndrome with other autoimmune rheumatic diseases, the development of large vessel vasculitis in patients with SSc is considered extremely rare, since only three case reports have thus far been reported in English literature. Herein, we report a 65-year-old woman with a long-standing history of systemic sclerosis who developed giant cell arteritis, eight years after initial diagnosis.

We report a case of a 25-year-old lady who presented to our department with complaints of easy fatigability and shortness of breath since one week. She had a history of Raynaud’s phenomenon. Examination revealed scleroderma like skin changes and pericardial friction rub. Investigations revealed high titer of anti-U1 RNP antibodies along with co-existing pancytopenia. Chest x-ray and echocardiography confirmed pericardial tamponade. Patient was diagnosed as having mixed connective tissue disorder (MCTD) and she was started on high dose prednisolone, which led to complete reversal of pancytopenia and pericardial tamponade after 1 month of treatment. There are only 6 reported cases of pericardial tamponade in a patient with MCTD, and none of them had pancytopenia. Present case highlights the need to investigate the patient of pericardial tamponade for MCTD, especially in the presence of pancytopenia and relevant clinical history, as prompt treatment with corticosteroids can avoid invasive procedures like pericardiocentesis.

To evaluate the benefits and harms of combination of tumor necrosis factor-alpha (TNF-α) inhibitor and methotrexate (MTX) compared with TNF-α inhibitor monotherapy in the treatment of ankylosing spondylitis (AS). Randomized controlled trials were identified from Medline, Embase, Cinahl, Central and Clinical Trials Registry Platform, as well as from the reference sections of retrieved articles. The risk of bias was evaluated in all included trials. Data were extracted by two reviewers independently using a specially designed extraction form. The Cochrane Collaboration’s Review Manager 5.2 software was used for data analysis. The search retrieved 852 titles, of which 3 original trials were included, involving 187 participants. The overall risk of bias is low in all three trials. Only one study was placebo controlled, and all of them examined small samples. The analysis showed no significant advantage of the MTX combination versus monotherapy. Two trials assessed Assessment of Ankylosing Spondylitis (ASAS) 40 and the pooled risk ratio (RR) was 1.37 and 95% confidence interval 0.84 to 2.23. The RR for ASAS20 was 1.16 (0.88 to 1.52). Likewise, there were no significant difference between two groups in partial remission, Bath Ankylosing Spondylitis Disease Activity Index, Bath Ankylosing Spondylitis Functional Index, Magnetic resonance imaging activity score and other secondary outcomes. Withdrawals for side effects and for any reason were similar in two groups, RR were 1.89 (0.71 to 5.02) and 1.11 (0.67 to 1.84), respectively. The evidence available did not support any benefit of adding MTX to TNF-α inhibitor for the treatment of AS.

We present an interesting case of a 13 year old girl with Wegener’s granulomatosis/granulomatosis with polyangiitis, who was misdiagnosed with Henoch-Schönlein purpura. The case highlights several important learning points for paediatric practice.

Behçet’s disease is a systemic vasculitis of unknown aetiology that may involve veins and arteries of all sizes. It occurs most commonly in the second or third decades of life and it is characterised by recurrent oral and genital ulceration and chronic relapsing uveitis. We present here a case of Behçet’s disease complicated by an inflammatory aortitis successfully treated with infliximab and endovascular stenting.

Ochronosis involves primarily the large cartilaginous joint surfaces, ribs, intervertebral discs, ear cartilage etc. We report on a 52-year-old woman with typical alkaptonuric ochronosis with dark urine, blue-black pigmentation of the auriculae and hands, spondylarthropathy and severe hip and shoulder joints involvement. The differential diagnosis of this rare condition is discussed with a review of the literature.

Antimyeloperoxidase (MPO), perinuclear antineutrophil cytoplasmic antibodies (pANCA), and/or clinically evident vasculitis in patients with scleroderma have been reported only rarely. The clinical significance and prognosis of ANCA-associated vasculitis in systemic sclerosis is uncertain. To report a case and identify the clinical characteristics of scleroderma patients with ANCA-associated vasculitis. Patients with both vasculitis and scleroderma occurring between 1976 to 2006 were identified using an electronic diagnostic index. These diagnoses were confirmed by retrospective review of complete medical records. Clinical features and outcomes recorded included age at vasculitis diagnosis, connective tissue disease (CTD) features, type of scleroderma (limited or diffuse); ANCA serology, vasculitic organ system manifestations; and death. Fourteen cases of scleroderma patients with ANCA-associated and/or small vessel vasculitis were identified. The majority (71%) were female, with mean age at vasculitis diagnosis 53 years. Seven patients (50%) had overlap CTD features, and the majority (79%) had limited variant of scleroderma. All of the 10 patients tested were MPO and pANCA positive. Seven patients (50%) had glomerulonephritis, 11 (79%) pulmonary involvement including 3 with pulmonary-renal syndrome, 6 skin purpura, and 5 mononeuritis multiplex and/or peripheral neuropathy. Six patients (43%) died during followup to 2008. The presence of pANCA-associated small vessel vasculitis is a rarely reported complication of scleroderma. It occurs most commonly in women with limited scleroderma and most commonly includes pulmonary and/or renal involvement, including severe organ-threatening manifestations and death. Further studies are needed to clarify the role and clinical impact of ANCA in scleroderma patients with and without vasculitis.