Rheumatologists and orthopaedic surgeons should be aware of potential complications such as destructive arthritis when treating patients with CPN.Systemic lupus erythematosus (SLE)-associated haemophagocytic lymphohistiocytosis (HLH) is called acute lupus haemophagocytic syndrome (ALHS), which is relatively rare but life-threatening. We present the case of a 43-year-old woman diagnosed with SLE with panniculitis, pleuritis, and autoimmune hepatitis. She was treated with high-dose glucocorticoids. Although disease activity temporarily improved, she developed fever, elevation of liver enzymes, hyperferritinemia, severe inflammatory response, and thrombocytopenia a month after starting glucocorticoids. Bone marrow biopsy was performed and haemophagocytosis was observed. She was diagnosed with ALHS on day 49. Since she developed ALHS during administration of glucocorticoids, her ALHS was determined to be refractory to glucocorticoid monotherapy; therefore, additional immunosuppressive agents were needed. She was treated with methylprednisolone pulse, plasma exchange and cyclosporine A (CyA). However, CyA was discontinued on day 54 because CyA-induced hypertensive encephalopathy was suspected. Subsequently, rituximab (RTX) was introduced to treat refractory ALHS on day 56; the disease activity subsequently reduced. After four courses of RTX, her ferritin levels and platelet counts were within the normal range and the glucocorticoid dose could be tapered to betamethasone 2.0 mg/day on day 132. No subsequent recurrence of SLE and ALHS was observed until day 132. RTX might therefore be an effective therapeutic option for refractory ALHS.Tuberculosis (TB) and its association with rheumatic diseases have been widely recognised. Occurrence of multifocal skeletal involvement constitutes less then 5% of all skeletal TB cases. We present a Malay patient with multifocal osteoarticular TB (OATB). A 35 year-old SLE woman with background usage of corticosteroid therapy and Azathioprine presented with lupus nephritis flare. Renal biopsy revealed diffuse proliferative lupus nephritis and intravenous (IV) Cyclophosphamide 0.5 g/m2 (850 mg) was initiated. One week later, patient complained dorsum of left hand and right knee swelling. On physical examination, patient was afebrile and the left hand swelling was cystic in consistency while right knee was warm and tender. Erythrocyte Sedimentation Rate (ESR) was 50 mm/hr and C-Reactive Protein (CRP) was 9.4 mg/L. Her Mantoux test was positive with 20 mm induration. Wrist radiograph and chest radiograph was normal. Musculoskeletal ultrasound showed 4th extensor compartment tenosynovitis with Doppler signal and right knee effusion with synovial proliferation. Extensor tenosynovectomy and right knee aspiration was performed. Left hand excised tissue and right knee synovial fluid for acid-fast bacilli (AFB) stain, TB PCR, bacterial and fungal cultures were negative. Urgent histopathological examination of the excised tissue showed necrotising granulomatous inflammation. Patient was empirically started on TB treatment and subsequent mycobacterial culture confirmed the diagnosis of TB. The joints swelling resolved after one month of TB treatment. Multifocal OATB is an infrequent form of extrapulmonary TB and diagnosing OATB requires high index of suspicion particularly in SLE patient on immunosuppression. Prompt investigations are essential to the diagnosis of this rare condition for early initiation of anti-tuberculous therapy.Psoriasis is a chronic disease of the skin that often affects the joints (psoriatic arthritis, PsA). Biologic agents such as TNF-α, IL-23 and IL-17 blockers have been proven to be quite effective against psoriasis and PsA, indicating the importance of those cytokines in the pathogenesis of the diseases. The importance of the IL-23/IL-17 axis has also been reported in systemic lupus erythematosus (SLE), but the safety and effectiveness of IL-17 blockers in SLE remain largely unknown. We encountered a patient with PsA and SLE. We treated him with an IL-17 blocker, secukinumab, and quantified the serum levels of various cytokines before and after the initiation of secukinumab therapy.  https://www.selleckchem.com/products/slf1081851-hydrochloride.html  As expected, the treatment was effective against the symptoms of PsA. No serious adverse events were observed in terms of SLE. Interestingly, serum IL-6 was substantially decreased after the initiation of therapy, whereas serum IL-17 was under the detection limit. These data indicate that IL-17 is produced locally, upstream of IL-6 production.A 52-year-old woman was diagnosed as having anti-centromere antibody (ACA)-positive primary Sjögren syndrome (pSS). Eight years later, she visited our hospital because she had developed dyspnoea. She was diagnosed as having pulmonary arterial hypertension (PAH) with pulmonary veno-occlusive disease on the basis of the results of right heart catheterisation, a severe decrease in diffusing capacity of the lung for carbon monoxide (DLCO, 17%) and desaturation (69%) after a 6-minute walk test. She was also diagnosed as having limited cutaneous systemic sclerosis (lcSSc) because she had developed finger sclerosis. The six-minute walk distance had improved by 54 m 3 months after commencing treatment with tadalafil. Clinicians should be alert to the possibility of patients with ACA-positive SS developing lcSSc and PAH during their clinical course.Psoriatic arthritis (PsA) manifests not only skin lesion but also peripheral and axial joint involvements, thus the disease is considered to be part of spondyloarthritis. In patients with active spondyloarthritis, tumour necrosis factor inhibitors biologics are often considered, but there is not sufficient evidence about other type of biologics. Ixekizumab (IXE) is a humanised monoclonal antibody, which acts against interleukin-17A, a cytokine involved in psoriasis pathogenesis. IXE was administered to two patients with refractory PsA, manifested via extended skin lesions and complicated by axial joint involvement. The effectiveness and imaging results during 12 weeks of treatment were assessed. IXE improved disease activity in both a tumour necrosis factor inhibitors biologics-inadequate responder and a bio-naïve patient and, consequently, IXE probably plays a key role in treatment of axial disease of PsA.