[Septic sacroiliitis: early diagnosis due to specific physical examination]. / Septische sacroiliitis.

BACKGROUND: Septic sacroiliitis is an uncommon disease which represents approximately 1-4% of all joint infections, therefore it is difficult to make the right diagnosis and to start early treatment. CASE REPORT: A 18 year old woman was admitted to the emergency room with a fever and pain in the left gluteal region. The patient was considered healthy and had no risk factors for septic arthritis. Edema and a small abscess was found in and around the left sacroiliac joint on pelvic MRI. The patient had positive blood cultures with Staphylococcus Aureus. Antibiotic treatment was initiated and lasted 7 weeks. She recovered completely and had no remaining complaints. CONCLUSION: Physical exam and clinical suspicion are important to consider the diagnosis of septic sacroiliitis. In addition a MRI of the pelvic is the best radiographic exam to conform the diagnosis.

Biphasic Allergic Reactions at a Dutch Emergency Department: A 5-Year Retrospective Cohort Study.

BACKGROUND: A biphasic allergic reaction develops in 0.4-20% of patients with an allergic reaction, but the incidence of severe biphasic reactions is unknown OBJECTIVE: Our objective was to assess the incidence and time of onset of clinically relevant biphasic reactions in a Dutch emergency department (ED) cohort. Furthermore, the characteristics of patients with a biphasic reaction and the mean observation time after an allergy-related ED visit were assessed. METHODS: This was a single-center retrospective cohort study. We collected data from clinical records of adult patients presenting with an allergic reaction to the ED between January 2015 and December 2019. We defined clinically relevant biphasic reactions as biphasic reactions in which the criteria for anaphylaxis were met. RESULTS: Five hundred fifty-seven patients were included. Eight patients (1.4%) developed a biphasic reaction, of which 1 (0.2% of the total inclusions) was clinically relevant. In the subgroup of patients with an anaphylactic reaction (n = 258), those percentages were 2.3% and 0.4%, respectively. The mean time between the initial allergic reaction and the biphasic allergic reaction was 25.4 h (95% CI 13.2-37.6 h). The single clinically relevant biphasic reaction occurred 30 h after the initial reaction. CONCLUSIONS: The incidence of clinically relevant biphasic reactions in our cohort was low, with a mean time between the initial allergic reaction and the biphasic reaction of > 24 h. Based on these single-center retrospective data, routine inpatient monitoring for several hours does not seem warranted for all patients.

Gelatinous bone marrow transformation due to extensive weight loss in median arcuate ligament syndrome.

A 34-year-old man was referred to the outpatient clinic because of progressive abdominal pain, weight loss and pancytopenia. His body mass index (BMI) had fallen to 14.2 kg/m2 A CT angiography (CTA) showed narrowing of the truncus coeliacus with poststenotic dilation, and duodenal biopsy revealed ischaemia establishing a rare diagnosis: median arcuate ligament syndrome (MALS). This explained the postprandial pain and minimal intake. Further pancytopenia workup was performed. The bone marrow displayed gelatinous marrow transformation (GMT), a rare disorder of unknown pathogenesis, which has been associated with severe malnutrition. The final diagnosis was pancytopenia secondary to GMT due to severe malnutrition caused by MALS. The abnormalities in the bone marrow may be reversible by restoring nutritional status. This case emphasises the awareness of GMT in patients with weight loss, malnutrition and cytopenias. To our knowledge, this is the first report demonstrating an association between pancytopenia and MALS.

Hypereosinophilic syndrome with multiorgan involvement: an interdisciplinary work-up.

A previously healthy 40-year-old man was referred to our emergency department with pruritic skin lesions and dyspnoea. Laboratory investigation revealed hypereosinophilia. Further diagnostic work-up confirmed the diagnosis of idiopathic hypereosinophilic syndrome (iHES), a rare myeloproliferative disease with a heterogeneous clinical presentation. We describe a unique case with cardiac, pulmonary, hepatic and cutaneous involvement at time of presentation. This case accentuates the importance of an extensive multidisciplinary diagnostic work-up, since iHES is a condition with potential rapid progressive multiorgan failure which requires prompt analysis and treatment. In addition, this case emphasises the importance of being aware of tunnel vision, especially during the COVID-19 pandemic, which might give rise to an increased risk of missing rare diagnoses. Our patient was treated with prednisolone, after which both his clinical condition and eosinophil concentrations markedly improved.

Blue toe syndrome as a first sign of systemic sclerosis.

We describe an unusual case of blue toe syndrome as the primary and solitary manifestation of systemic sclerosis. The possible cause was long-term occupational exposure in construction work. Blue toe syndrome is a small vessel disease, characterised by the sudden development of painful, blue discolouration in one or more toes. The most common aetiology is atheroembolic disease; however, it can also appear in several conditions ranging from hypercoagulability disorders to underlying systemic diseases such as vasculitis or autoimmune diseases. Here, we describe the case of a 57-year-old man who presented with blue toe syndrome without underlying atheroembolic disease. He was found to have positive anticentromere antibodies, which indicated that systemic sclerosis was the likely primary underlying cause. An extensive systemic evaluation and a thorough physical examination revealed no other symptoms associated with systemic sclerosis. He was prescribed nifedipin and rosuvastatin, and showed complete resolution of symptoms after 3 months.

The interferon type I signature is present in systemic sclerosis before overt fibrosis and might contribute to its pathogenesis through high BAFF gene expression and high collagen synthesis.

BACKGROUND: Interferon (IFN) signature has been reported in definite systemic sclerosis (SSc) but it has not been characterised in early SSc (EaSSc). We aim at characterising IFN type I signature in SSc before overt skin fibrosis develops. METHODS: The expression of 11 IFN type I inducible genes was tested in whole-blood samples from 30 healthy controls (HCs), 12 subjects with primary Raynaud's phenomenon (RP), 19 patients with EaSSc, 7 patients with definite SSc without cutaneous fibrosis, 21 limited cutaneous SSc and 10 diffuse cutaneous SSc subjects. The correlation between IFN activity in monocytes, B cell activating factor (BAFF) mRNA expression and type III procollagen N-terminal propeptide (PIIINP) serum levels was tested. RESULTS: In all the SSc groups, higher IFN scores were observed compared with HC. An IFN score ≥7.09 discriminated HCs from patients with SSc (sensitivity=0.7, specificity=0.88, area under receiving operating characteristic (AUROC)=0.82); the prevalence of an elevated IFN score was: HC=3.3%; RP=33.3%, EaSSc=78.9%, definite SSc=100%, limited cutaneous SSc=42.9%, diffuse cutaneous SSc=70.0%. In monocytes an IFN score ≥4.12 distinguished HCs from patients with fibrotic SSc (sensitivity=0.62, specificity=0.85, AUROC=0.76). Compared with IFN-negative subjects, IFN-positive subjects had higher monocyte BAFF mRNA levels (19.7±5.2 vs 15.20±4.0, p=2.1×10(-5)) and serum PIIINP levels (median=6.0 (IQR 5.4-8.9) vs median=3.9 (IQR 3.3-4.7), p=0.0004). CONCLUSIONS: An IFN type I signature is observed in patients with SSc from the earliest phases of the disease, even before overt skin fibrosis. The presence of IFN type I signature in monocytes is correlated with BAFF mRNA expression and serum PIIINP levels, supporting a contribution in the pathogenesis and progression of SSc.