Whipple's disease is a chronic infectious systemic disease caused by the bacterium Tropheryma whipplei. Nondeforming arthritis is frequently an initial complaint. Gastrointestinal and general symptoms include marked diarrhoea (with serious malabsorption), abdominal pain, prominent weight loss, and low-grade fever. Possible neurologic symptoms (up to 20%) might be associated with worse prognosis. Diagnosis is based on the clinical picture and small intestinal histology revealing foamy macrophages containing periodic-acid-Schiff- (PAS-) positive material. Long-term (up to one year) antibiotic therapy provides a favourable outcome in the vast majority of cases. This paper provides review of the literature and an analysis of our 5 patients recorded within a 20-year period at a tertiary gastroenterology centre. Patients were treated using i.v. penicillin G or amoxicillin-clavulanic acid + i.v. gentamicin for two weeks, followed by p.o. doxycycline (100 mg per day) plus p.o. salazopyrine (3 g per day) for 1 year. Full remission was achieved in all our patients.

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Summary: Double balloon enteroscopy (DBE) was introduced 15 years ago. The complications of diagnostic DBE are rare, acute pancreatitis is most redoubtable one (incidence about 0.3%). Hyperamylasemia after DBE seems to be a rather common condition respectively. The most probable cause seems to be a mechanical straining of the pancreas. We tried to identify patients in a higher risk of acute pancreatitis after DBE. We investigated several laboratory markers before and after DBE (serum cathepsin B, lactoferrin, E-selectin, SPINK 1, procalcitonin, S100 proteins, alfa-1-antitrypsin, hs-CRP, malondialdehyde, serum and urine amylase and serum lipase). Serum amylase and lipase rose significantly with the maximum 4 hours after DBE. Serum cathepsin and procalcitonin decreased significantly 4 hours after DBE compared to healthy controls and patients values before DBE. Either serum amylase or lipase 4 hours after DBE did not correlate with any markers before DBE. There was a trend for an association between the number of push-and-pull cycles and procalcitonin and urine amylase 4 hours after DBE; between procalcitonin and alfa-1-antitrypsin, cathepsin and hs-CRP; and between E-selectin and malondialdehyde 4 hours after DBE. We found no laboratory markers determinative in advance those patients in a higher risk of acute pancreatitis after DBE.

Scleroderma renal crisis (SRC) is classical renal disease in systemic sclerosis (SSc). SRC is a relatively rare manifestation, approximately in 5% of patients. In terms of severity, manifestation in the form of SRC is the most common cause of acute organ failure. In SSc patients, SRC is defined as a new onset of accelerated arterial hypertension and rapidly progressive anuric or oliguric renal failure. SRC is primarily vascular injury with increased activity of the renin-angiotensin activity. These events lead to release or activation of cytokines and growth factors that result in the typical proliferative vascular lesions. Successful approach is routine use of angiotensin-converting enzyme inhibitors in the treatment of SRC (except prevention) and other advances in renal replacement therapy in SSc management. It is crucial to detect manifestations of SRC early and to manage appropriately in collaboration with intensive care medicine, cardiologists, and nephrologists. In contrast to SRC, clinical presentation of interstitial renal disease is poor, often without evidence of renal abnormality. Interestingly, other renal manifestations are glomerulonephritis and vasculitis. These manifestations are associated with overlapping mechanisms. The objective of this chapter is to focus on actual knowledge about the renal involvement in SSc and current treatment principles and possibilities.

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ObjectivesDiffuse alveolar hemorrhage (DAH) is severe, life-threatening syndrome, affecting the patients with systemic vasculitis and other systemic connective tissue diseases. It can be often the first manifestation of systemic disease. Frequently DAH is the acute incident, which often leads to the rapidly emerging respiratory and/or renal failure with requirement of ventilatory support and with high mortality rate.MethodsDiffuse alveolar hemorrhage was diagnosed on the basis of bronchoscopy and CT examination in patients with rapidly progressive respiratory insufficiency or newly emerged hemoptysis. Artificial ventilation was started in majority of cases.Therapeutically were used high doses of steroids i.v. (1000 mg 3–5 times in the iv infusion), Cyclophosphamide pulse, and plasmapheresis in some cases.ResultsThe group of patients consisted of 32 cases; the average age of patients was 54 years (20–71) years. In half of patients was the episode of DAH the first manifestation of the disease. 16 patients were pro3 ANCA-positive, 7 patients MPO-ANCA positive, 6 patient had known systemic connective tissue disease 46.8% of patients survive 3 years from the acute episode of DAH, including two with repeated hemorrhage. 15 patients died during the acute phase of the disease, 3 more died during next 3 years, two of them on an opportunistic infection. Steroids were used for all patients, cyclophosphamide in 71, plasmapheresis in 56%, and i.v. immunoglobulins for 6.25 percent of patients. Patients who survive 3 years after the acute episode, had significantly lower necessity of artificial ventilation (40 vs. 93%) against the fatal cases, unfavorable prognostic sign was long term connective tissue disease. Residual lung impairment is present in 53% of the surviving patients; the signs of pulmonary arterial hypertension are present in 20% of the surviving group.ConclusionsDiffuse alveolar hemorrhage is a sudden life-threatening event with a high mortality rate (46%) in the acute phase and during the first 3 years after the acute episode (20% of survivors). Up to half of patients the episode of DAH may be the first manifestation of the disease. Survival of patient depends on rapid diagnosis and the initiation of intensive immunosuppressive therapy. Respiratory failure is the major negative quoad vitam prognostic factor.Suspect the diffuse alveolar bleeding is necessary always in the case of a rapidly progressive respiratory failure. Acute bronchoscopy and x-ray examination give diagnosis of DAH, which further specify the acute examination of ANCA antibodies (71% of the report was ANCA antibodies positive.Disclosure of InterestNone declared

BackgroundGout is a common, inflammatory, crystal-induced disease with increasing prevalence and incidence. Typical monoarticular involvement can be quite easily and effectively treated. In recent years, we have observed more frequently gout cases with severe polyarticular manifestation and with systemic inflammatory response syndrome (SIRS). In clinical practice such a complicated gout attack used to be identified with delay and therapy is not adequate.ObjectivesTo assess clinical and laboratory parameters of systemic inflammatory polyarticular gout and to identify risk factors and convenient therapeutic strategy.MethodsRetrospective analysis of 133 consecutive new patients (14 females) with gout (according to the classification criteria ACR/EULAR 2015) on the presence of acute polyarticular involvement (≥ 4 joints) and fullfilling criteria for SIRS.ResultsSeventeen (12.8%) of 133 gout patients had polyarticular manifestation and met SIRS criteria; 2 females and 15 males, average age 73.2±11.3 years, BMI 29.9±3.8. All these patients had to be admitted to inpatient department because of gout severity. Mean CRP was 204±113 mg/l, leucocytes 15.2±6.8 109/l, serum uric acid 401±140 µmol/l. Procalcitonin was slightly positive in 2 patients (with concurrent respiratory infection). Mean 6 joints were affected, 13 (76,5%) patients had involvement of both upper and lower limbs, tophi were present in 7 of 17 patients. Synovial fluid analysis was performed with proving urate crystals and leukocyte count 24.4±17.3 109/l. Typical gout features were found in 70.6% patients on joint X-ray and in 52.9% ones on musculoskeletal ultrasound.Risk factors for systemic inflammatory polyarticular manifestation were preceding renal function impairment, long-term diuretic therapy and alcohol above-average consumption. Monotherapy was not effective in any patients, combination of NSAID with colchicin or systemic glucocorticoids was used with success.ConclusionAcute polyarticular gout with systemic inflammatory response mimics septic status and mostly requires hospitalization. Complex management with combination therapy should be guided by an experienced rheumatologist.Reference[1] Schäfer VS, Krause A, Trauzeddel RF, Schmidt WA. Systemic Inflammatory Polyarticular Gout Syndrome – Description of a Previously Neglected Entity. JSM Arthritis 2017; 2: 1024.Disclosure of InterestsNone declared