We present a case of a 75-year-old woman who admitted in the internal medicine department for a recent onset of persisting moderate daily fever and fatigue that started 30 days prior to her hospitalization. Her past medical history is remarkable for mild pulmonary fibrosis, megaloblastic anaemia, and hypergammaglobulinaemia of no obvious causes. On presentation, she was febrile (38ºC) and had high ESR and CRP levels, but most of her laboratory tests were within normal levels and had no signs of arthritis or rash. She was hospitalized for suspected lower urinary tract infection and started on antibiotics. During hospitalization, her renal function deteriorated together with microscopic haematuria, proteinuria and granular urine casts in urine analysis and her inflammation markers raised further. A renal biopsy revealed glomerulonephritis with pauci-immune crescents, and serology tests were positive for anti-MPO p-ANCA, both suggesting a diagnosis of microscopic polyangiitis (MPA). While high-dose methylprednisolone pulses and cyclophosphamide were introduced intravenously, there was no remission, but respiratory failure occurred that led to patient's intubation and transfer to the ICU. She died a few days later due to septic shock. Asymptomatic pulmonary fibrosis can precede microscopic polyangiitis for several years and is associated with a poor prognosis.
Background:The current goal of treatment in SLE is remission or low disease activity (LDA) and prevention of flares, achieved with the lowest possible dose of glucocorticoids. Nevertheless, in current clinical practice a significant number of patients still has active disease.1,2Objectives:To assess the current disease activity state of SLE patients during their most recent visit in two centers (Department of Rheumatology in “Asklepieio” Hospital and Rheumatology Unit in “Attikon” Hospital, both in Athens, Greece).Methods:Cross-sectional study of patients with a diagnosis of SLE for at least one year. Patients were divided into four groups: 1) Remission off-therapy: SLE Disease Activity Index (SLEDAI)=0 without prednisone or immunosuppressive drugs (IS), 2) Remission on-therapy: SLEDAI=0, prednisone dose ≤5mg/day and/or IS (conventional and biologic, maintenance phase), 3) LDA: SLEDAI ≤4, prednisone dose ≤7.5mg/day and/or IS (maintenance phase), 4) Active disease: SLEDAI >4 and/or prednisone dose >7.5mg/day and/or IS (induction phase).2 Hydroxychloroquine was allowed in all groups.Results:205 patients were included [95.1% female, mean (SD) age 48.4 (14.9) years and median disease duration (IQR) 6.2 (12.6) years]. A history of lupus nephritis and neuropsychiatric SLE was present in 16.6% and 17.1% of our patients, respectively, and 39% of patients had SLICC/ACR damage index (SDI) > 0. At last visit, remission off-therapy and remission on-therapy was present in 8.3% (n=17) and 15.1% (n=31) of our patients, respectively. Seventy-five patients (36.6%) had LDA, whereas 82 patients (40%) had active disease. More than 85% (86.3%) of patients were in treatment with hydroxychloroquine and 64.4% were receiving immunosuppressive drugs. Regarding glucocorticoids, 50.2% (n=103) were treated with prednisone dose ≤7.5mg/day and over 40% (42.4%, n=87) did not receive prednisone at all. A SLEDAI score 0 and 1-4 was achieved in 24.4% and 42.9% of patients, respectively, but only 3.9% had a SLEDAI > 8, indicative of high disease activity.Conclusion:Although the majority of our patients were treated with hydroxychloroquine and glucocorticoids in acceptable levels of daily dose, four out of ten patients in our practice have active disease during their last visit. Achieving treatment goals in SLE patients remains a challenge for future novel therapies.References:[1]Fanouriakis A, Kostopoulou M, Alunno A, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis 2019; 78: 736–745.[2]Ugarte-Gil MF, Wojdyla D, Pons-Estel GJ, et al. Remission and Low Disease Activity Status (LDAS) protect lupus patients from damage occurrence: data from a multiethnic, multinational Latin American Lupus Cohort (GLADEL). Ann Rheum Dis 2017; 76: 2071–2074.Disclosure of Interests:None declared
Ankylosing spondylitis (AS) is an inflammatory disease affecting mainly the sacroiliac joints and the spine. In long-standing disease, the fused spine of AS patients is susceptible to spinal fractures, even after low impact trauma. We present a 61-year-old man with long-standing AS who presented with anterior and posterior longitudinal ligament rupture and T12 and L1 vertebral endplates fractures (a so called “chalk-stick fracture”) without reporting any prior trauma and discuss relevant issues.
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