Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are related inflammatory rheumatic conditions affecting adults over the age of 50 years. Both conditions respond to initial glucocorticoid (GC) therapy. However, most patients require 12-36 months of a tapering steroid regime. Adverse events at 2 years are seen in up to 65% of patients with PMR and 86% of patients with GCA with over 50% developing serious events. There is also a high incidence of relapse in both diseases -40% within 2 years for GCA and 50% of patients with PMR at some point having a relapse. Effective steroid-sparing adjuvant therapies are urgently required especially in incomplete, poorly sustained or non-responders to glucocorticoids. In this case series, we found that Leflunomide is efficacious, with 22 out of our 23 patients exhibiting a complete or partial response. It was also steroid sparing and well tolerated. It may be a useful adjunctive agent in difficult-to-treat GCA and PMR. Prospective randomised controlled trials of Leflunomide in both GCA and PMR are now required.
Sarcoidosis is a systemic disease of unknown aetiology that is characterized by granulomatous inflammation that can develop in almost any organ system. Musculoskeletal manifestations are seen in up to one-third of patients, ranging from arthralgia through to widespread destructive bone lesions. Inflammatory tendon lesions and periarticular swelling are more common than true joint synovitis. Despite advances in our understanding of the pathophysiology of the disease, diagnosis remains challenging. Definitive diagnosis, irrespective of organ site involvement, hinges on histological confirmation of non-caseating granuloma combined with an appropriate clinical syndrome. Musculoskeletal involvement usually develops early in the disease course. Imaging modalities, particularly fluorodeoxyglucose PET, are helpful in delineating the extent of involvement and measuring disease activity. Bone involvement may only become apparent following isotope imaging. Corticosteroids remain the cornerstone of treatment. MTX is the steroid-sparing agent of choice unless there is renal involvement. Biologic therapies are sometimes used in severe disease, although the evidence base for efficacy is inconsistent.
Diuretics are the most common cause of community-developed hyponatremia. The serum uric acid level effectively discriminates between two biochemical profiles of diuretic-induced hyponatremia, one consistent with extracellular volume depletion and another that simulates SIADH.
A 63-year-old female presented with a 12-week history of worsening proximal pain and stiffness. She was diagnosed with polymyalgia rheumatica and started on corticosteroids. The authors were unable to wean-off her steroid treatment, despite trying various steroid sparing agents on different occasions with no benefit. In August 2010, she was diagnosed with giant cell arteritis with a temporal artery biopsy and ultrasound of the temporal and axillary arteries. An fluorine-18-deoxyglucose positron emission tomography CT showed increased uptake in the aorta and major vessels, in keeping with widespread large vessel involvement. Due to the disease severity, the failure of previous disease-modifying agents and the development of steroid related sideeffects, the authors decided to treat her with intravenous tocilizumab (TCZ;an interleukin 6 blocker). After her first infusion, the patient reported excellent response with normalisation of her inflammatory markers. Prednisolone reduced from 20 mg to 3.5 mg /day after five infusions of TCZ (8 mg/kg).
Adult-onset Still's disease is a rare inflammatory disorder characterised by fever, arthritis and rash. It can present in a number of ways and is associated in 5% of cases with parenchymal lung involvement. We present the case of a 37year-old man who initially presented with fever, weight loss and pancytopaenia. He gradually deteriorated requiring non-invasive ventilation with a Computerised tomography of his chest showing bilateral nodular infiltrates. An open lung biopsy showed acute fibrinous organising pneumonia, which responded well to corticosteroid treatment. He then re-presented over three years later with a similar systemic illness although with less severe lung involvement. Following extensive further investigations, he was diagnosed with Adult-onset Still's disease fulfilling the Yamaguchi criteria. We feel this case is important due to the rare association of Adult-onset Still's disease and interstitial lung disease. More specifically, we are not aware of any published cases of Adult-onset Still's disease with acute fibrinous organising pneumonia.Keywords clinical, interstitial lung disease, other rheumatology, respiratory medicine, rheumatology
Case presentationOur patient was a Caucasian man who initially presented aged 33 with a seven-week history of gradually worsening fevers, night sweats, lethargy and 6 kg weight loss. He had no significant medical history, was on no regular medications and worked in Information Technology. Admission blood tests showed a total white cell count (WCC) of 2.38 (10 9 / L), neutrophils of 1.5 (10 9 /L), lymphocytes of 0.6 (10 9 /L), haemoglobin of 10.4 (g/dl) and platelets of 209 (10 9 /L) (which subsequently dropped to 135). Liver enzymes showed an alanine aminotransferase (ALT) of 59 (IU/L), bilirubin of 32 (mmol/L) and alkaline phosphatase (ALP) of 57 (IU/L). His c-reactive protein (CRP) was 44 (mg/L) and ferritin elevated at 728 (mg/L). Computerised tomography (CT) of the chest showed multiple bilateral lung nodules and splenomegaly. Due to his fever and imaging appearances (Figure 1), he was treated with broadspectrum antibiotics for possible atypical pneumonia. Multiple investigations including several blood cultures were performed to look for an infective cause of the fever all of which were negative. Autoimmune screen was unremarkable. He continued to deteriorate despite antibiotics developing type-1 respiratory failure requiring non-invasive ventilation. His blood tests also worsened becoming pancytopaenic with worsening liver enzymes (ALT increased to 262 IU/ L) and CRP increasing to 205 (mg/L). Bronchoscopy with transbronchial biopsies and washings were nondiagnostic. Due to a lack of a clear diagnosis, he was intubated and transferred to a tertiary care centre for a surgical lung biopsy which showed acute fibrinous and organising pneumonia, noting a prominent reactive interstitial lymphoid infiltrate ( Figure 2). Following this, he was given intravenous methylprednisolone for five days and started to improve clinically. This was followed by a reducing cou...
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