ObjectiveOur aim was to present our first experience with the use of caplacizumab, an anti-von Willebrand factor monoclonal antibody, in an SLE patient who developed TTP. Data on the use of ...caplacizumab in SLE-associated TTP are scarce.MethodsWe present a single SLE patient with TTP, successfully treated with plasma exchange, caplacizumab, and rituximab.ResultsA 64-year-old patient was admitted to our department in 2023 due to severe thrombocytopenia (15x109/L), multiple hematomas of the extremities, and a history of transient dysarthria lasting 30 minutes the evening prior to admission. Her previous history was remarkable for inactive SLE and splenectomy due to B-non-Hodgkin’s splenic lymphoma of the marginal zone in 2016. Her blood count was compatible with hemolytic anemia (hemoglobin 95 g/L, LDH 984 U/L, bilirubin 17 umol/L, haptoglobin undetectably low). Her anti-dsDNA levels were not increased, antiphospholipid antibodies and complement levels were normal, despite an elevated erythrocyte sedimentation rate (120 mm/h). Her brain CT and CT angiography were unremarkable. Given the negative Coombs test, an urgent peripheral blood smear was ordered, revealing the presence of 12 shistocytes per high power field, compatible with microangiopathic hemolytic anemia. Given that the patient was stable, she was started on pulse-dose iv methylprednisolone the same evening, and a blood ADAMTS13 test was ordered early in the next morning (0,05 kIU/L, reference range: 0.60–1.21). The patient was diagnosed with TTP and was treated with plasma exchange for three consecutive days, as well as caplacizumab 10 mg sc daily for the duration of plasma exchange and 30 days subsequently. She was also treated with rituximab iv 375mg/m2 body surface area (four consecutive weekly doses). Neither the chest-abdomen CT scan nor the bone marrow biopsy revealed signs of lymphoma relapse. Thrombocytopenia was resolved within a week following treatment initiation, and no relapse of TTP or SLE was observed within four months of follow-up.ConclusionsEven a history of transient neurological symptoms should warrant a diagnosis of TTP in the context of SLE with Coombs-negative hemolytic anemia. The role of caplacizumab in the therapeutic algorithm of TTP in SLE patients still needs to be established.
Sjögren’s syndrome (SjS) is a systemic autoimmune disease, most common in middle-aged women, characterized by chronic inflammation
of exocrine glands. It can present by itself or alongside other ...autoimmune diseases. Main symptoms are dry eyes and mouth, but the disease can affect joints, lungs, kidneys, peripheral nervous system (PNS), and rarely central nervous system (CNS). SS-A antibodies in SjS can pass the fetoplacental barrier and cause congenital heart block. Patients with SjS are at higher risk of developing lymphoma.
Članak je zadržan u integralnom obliku i odstupa od standardnog načina citiranja i navođenja izvora koji se primjenjuje u sic-u.Dugo su se ruska i američka kultura percipirale kao potpuno suprotne, ...građene na proturječnim idejama: kolektivizmu i individualizmu, jednakosti i slobodi, na pojmovima komunalnost i „privacy”. Kako bismo se razumjeli, potrebno je primijetiti isti oksimoron u samom izrazu „rusko-američka kultura”, kao i u izrazu „crna ovca”. Mi se, ruski Amerikanci, nalazimo „na kraju perspektive koja iščezava”, u točki konvergencije suprotnosti i dužni smo ih iznova razrješavati sami u sebi, u svojem iskustvu i stvaralaštvu. Magnetno polje rusko-američke kulture nabijeno je svim onim intelektualnim i emocionalnim proturječjima koja su donedavno spomenute kulture činile neprijateljima i suparnicima.Dakle to je, prema svojem potencijalu, zapravo velika kultura koja ne pripada u potpunosti ni američkoj, a ni ruskoj tradiciji, već nekoj fantastičnoj kulturi budućnosti, onoj Amerusiji koja je prikazana u Nabokovljevoj Adi. Rusko-američka kultura ne može se svesti na svoje zasebne komponentne, već ih nadrasta, poput krošnje, na kojoj će se daleko razgranate grane zajedničkog indoeuropskog drveta jednom ponovno ispreplesti, spoznati svoje srodstvo, baš kao što se u ruskoj riječi sam i engleskoj same mutno prepoznaje srodstvo indoeuropskih korijena.
The aim of the study was to determine the serum vitamin D levels in patients with psoriatic arthritis (PsA) and compare it with patients with rheumatoid arthritis (RA) and with osteoarthritis (OA), ...as well as to explore the relationship of the vitamin D level with indices of disease activity and functional ability in a real-life setting in a South-European country.
In a cross-sectional study, 120 adult patients with established diagnosis of PsA, RA and OA were consecutively enrolled. Serum 25-hydroxyvitamin D and intact parathyroid hormone were determined. Parameters of disease activity and functional ability were obtained using standard instruments.
Serum vitamin D insufficiency (≤ 75 nmol/L) was found in 74% of patients with PsA, 94% patients with RA and 97% of patients with OA, whereas vitamin D deficiency (≤ 25 nmol/L) was found in 13% of patients with PsA, 39% of patients with RA and in 38% of patients with OA. Compared with RA, patients with PsA had significantly higher serum vitamin D (P = 0.002), and when controlling for age and gender, their serum vitamin D level was significantly associated with disease activity and functional activity.
In the group of rheumatic patients, a high prevalence of serum vitamin D insufficiency/deficiency was found regardless of the type of arthritis. Patients with PsA might have higher levels of vitamin D than patients with RA, and this was associated with disease activity and functional ability. The results of this study indicate that prophylactic supplementation with vitamin D might be recommended for all rheumatic patients.
Eosinophilia is characterized by more than 0.5
x 10
eosinophils per liter in the full blood count. A wide
range of conditions, from asthma to parasitic infections,
autoimmune diseases, and certain ...forms of cancer, have
been known to trigger abnormally high amount of eosinophils.
It is essential to reach the correct diagnosis and
treat the underlying disease aggresively. Definition of the
eosinophilia-myalgia syndrome was offered in 1980s by
Centers for Disease Control and Prevention for surveillance
purposes, and criteria were revised in 2001, with
high specificity.
We report a case of 59-year old female who started a
special weight-reducing diet regimen that included excessive
cashew nut ingestion. Several months after she has
presented with periferal blood eosinophilia and constitutional
symptoms. Detailed work-up has not found elements
for haematological, systemic autoimmune, neoplastic or
infectious disease. She was diagnosed with eosinophilia-
myalgia syndrome due to extreme L-tryptophan intake,
a compound found in the cashew nut’s oil. She responded
well to cashew nut withdrawal and steroid therapy. In the
follow-up period she remained stable with normal eosinophil
count and there was not a need for any specific
therapy
Primopredaja bolesnika smatra se jednim od najdelikatnijih postupaka u medicini i najvažnijim sprječivim uzrokom medicinske pogreške. Ovaj se proces zbiva na svim razinama zdravstvene zaštite: ...primarnoj,
sekundarnoj i tercijarnoj, od kojih svaka ima svoje specifičnosti. Unaprjeđenje kvalitete primopredaje na svakoj razini izrazito je važno. Prikladan i precizan prijenos informacija između medicinskog osoblja nuždan je za sigurnost bolesnika i kontinuiranu zdravstvenu zaštitu. U ovom su članku opisane preporuke za uspješnu i kvalitetnu primopredaju bolesnika te su istaknute pogreške pri komunikaciji i postupku prijelaza u skrb drugom liječniku. Poseban je osvrt dan na primopredaju reumatološkog bolesnika i na prijelaz iz pedijatrijske skrbi u adultnu reumatološku skrb. Osobito je naglašena potreba poučavanja pri komunikaciji i prenošenju podataka tijekom prijelaza bolesnika od jednog liječnika drugomu, iz ustanove u ustanovu. Poučavanje komunikacijskih vještina danas se provodi na medicinskim fakultetima na diplomskoj i poslijediplomskoj razini. Na Medicinskom fakultetu Sveučilišta u Zagrebu uveli smo longitudinalni 6-godišnji predmet Temelji liječničkog umijeća u kojem studente poučavamo komunikaciji. Ipak, ističemo nedovoljan broj nastavnih sadržaja specijalističkog usavršavanja koji obuhvaćaju izobrazbu o primopredaji bolesnika i vještinama otpusta bolesnika.
Eosinophilia is characterized by more than 0.5 × 109 eosinophils per liter in the full blood count. A wide range of conditions, from asthma to parasitic infections, autoimmune diseases, and certain ...forms of cancer, have been known to trigger abnormally high amount of eosinophils. It is essential to reach the correct diagnosis and treat the underlying disease aggresively. Definition of the eosinophilia-myalgia syndrome was offered in 1980s by Centers for Disease Control and Prevention for surveillance purposes, and criteria were revised in 2001, with high specificity. We report a case of 59-year old female who started a special weight-reducing diet regimen that included excessive cashew nut ingestion. Several months after she has presented with periferal blood eosinophilia and constitutional symptoms. Detailed work-up has not found elements for haematological, systemic autoimmune, neoplastic or infectious disease. She was diagnosed with eosinophilia-myalgia syndrome due to extreme L-tryptophan intake, a compound found in the cashew nut’s oil. She responded well to cashew nut withdrawal and steroid therapy. In the follow-up period she remained stable with normal eosinophil count and there was not a need for any specific therapy.
To validate Systemic Lupus International Collaborating Clinics (SLICC)-12 and American College of Rheumatology (ACR)-97 classification criteria on a patient cohort from the University Hospital Center ...Zagreb.
This retrospective study, conducted from 2014 to 2016, involved 308 patients with systemic lupus erythematosus (SLE) (n=146) and SLE-allied conditions (n=162). Patients' medical charts were evaluated by an expert rheumatologist to confirm the clinical diagnosis, regardless of the number of the ACR-97 criteria met. Overall sensitivity and specificity, as well as the sensitivity and specificity according to disease duration, were compared between ACR-97 and SLICC-12 classifications. Predictive value for SLE for both classifications was assessed using logistic regression and receiver operating characteristic (ROC) curves.
The SLICC-12 criteria had significantly higher sensitivity in early disase, which increased with disease duration. The ACR-97 criteria had higher specificity. The specificity of the SLICC-12 criteria was low and decreased with disease duration. Regression analysis demonstrated the superiority of the SLICC-12 classification criteria over the ACR-97 criteria, with areas under the ROC curve of 0.801 and 0.780, respectively.
Although the SLICC-12 criteria were superior to the ACR-97 and were more sensitive for diagnosing early SLE, their specificity in our population was too low. The sensitivity of the SLICC-12 classification is increased by better defined clinical features within each criterion. Our results contribute to the current initiative for developing new criteria for SLE.
Aim To validate Systemic Lupus International Collaborating Clinics (SLICC)-12 and American College of Rheumatology (ACR)-97 classification criteria on a patient cohort from the University Hospital ...Center Zagreb. Methods This retrospective study, conducted from 2014 to 2016, involved 308 patients with systemic lupus erythematosus (SLE) (n = 146) and SLE-allied conditions (n = 162). Patients' medical charts were evaluated by an expert rheumatologist to confirm the clinical diagnosis, regardless of the number of the ACR-97 criteria met. Overall sensitivity and specificity, as well as the sensitivity and specificity according to disease duration, were compared between ACR97 and SLICC-12 classifications. Predictive value for SLE for both classifications was assessed using logistic regression and receiver operating characteristic (ROC) curves. Results The SLICC-12 criteria had significantly higher sensitivity in early disase, which increased with disease duration. The ACR-97 criteria had higher specificity. The specificity of the SLICC-12 criteria was low and decreased with disease duration. Regression analysis demonstrated the superiority of the SLICC-12 classification criteria over the ACR-97 criteria, with areas under the ROC curve of 0.801 and 0.780, respectively. Conclusion Although the SLICC-12 criteria were superior to the ACR-97 and were more sensitive for diagnosing early SLE, their specificity in our population was too low. The sensitivity of the SLICC-12 classification is increased by better defined clinical features within each criterion. Our results contribute to the current initiative for developing new criteria for SLE.
PDF
