Choroba IgG4 zależna manifestująca się jako izolowana zmiana w obrębie żołądka– przegląd literatury

Wstęp: Choroba IgG4-zależna (IgG4-RD) jest niedawno opisaną jednostką chorobową o nieustalonej etiologii i patogenezie charakteryzującą się występowaniem bogatych w komórki IgG4 dodatnie nacieków tkankowych z włóknieniem. Zmiany w jej przebiegu lokalizują się w różnych narządach, jednak w obrębie przewodu pokarmowego występują rzadko. Rozpoznany w naszej Klinice przypadek izolowanego zajęcia żołądka przez IgG4-RD stał się punktem wyjścia do wykonania przeglądu aktualnej literatury w celu przybliżenia lekarzom tej nowej jednostki chorobowej. Materiały i metody: Przeglądu literatury dokonano w oparciu o bazę PubMed. Do przeglądu włączono 8 prac dotyczących choroby IgG4-zależnej ograniczonej do żołądka opublikowanych w latach 2001–2017 oraz przypadek IgG-RD rozpoznany przez autorów pracy. Wyniki: U 3 z 9 chorych występowały objawy ze strony przewodu pokarmowego, w innych przypadkach zmiany zostały wykryte przypadkowo. U większości pacjentów miały one charakter guza podśluzówkowego. Tylko w jednym przypadku zaobserwowano zmianę w postaci owrzodzenia. Najczęstszą lokalizacją zmiany był trzon żołądka. Wszystkie zmiany zostały usunięte chirurgicznie z powodu podejrzenia nowotworu złośliwego, a diagnozę postawiono na podstawie typowego obrazu choroby w badaniu histologicznym i immunohistochemicznym. Podwyższony poziom IgG4+ w surowicy stwierdzono u jednej pacjentki. W żadnym przypadku nie zaobserwowano wznowy ani pojawienia się innych zmian o typie IgG4-RD. Wnioski: Choroba IgG4-zależna może manifestować się w postaci zmian ograniczonych do żołądka i powinna być brana pod uwagę w diagnostyce różnicowej. Postawienie ostatecznego rozpoznania przed wykonaniem badania histopatologicznego jest trudne, co przekłada się na pomyłki diagnostyczne i terapeutyczne. IgG4-RD charakteryzuje się dobrą odpowiedzią na glikokortykosteroidy. W tym aspekcie konieczne są jednak dalsze badania

IgG4-related disease manifesting as an isolated gastric lesion- a literature review

Introduction: IgG4-related disease (IgG4-RD) is a newly recognised disorder of unknown etiology and pathogenesis, characterised by dense IgG4+ cells infiltration and fibrosis. IgG4-RD can affect various organs, but gastrointestinal tract involvement is rare. First case of isolated gastric IgG4-RD reported in polish population was diagnosed in our Clinic and became the reason for conducting a literature review. Materials and methods: A literature review was performed using PubMed database. Eight studies of isolated gastric IgG4-RD, published between 2011 and 2017, and a case diagnosed by the authors were included. Results: Three out of nine analysed patients had gastrointestinal complaints. In other cases lesions were detected accidentally. The majority of them were submucosal tumors while only one was a gastric ulcer. The most commonly affected was the stomach body. In all cases malignancy had been suspected, and the lesions were surgically removed. Diagnosis was based on the histopathology image and immunohistochemical staining. Only one patient had elevated IgG4 serum level. No case of recurrence or other organ involvement was reported. Conclusion: IgG4-related disease may manifest as an isolated gastric lesion and should be taken in consideration in differential diagnosis. Making an ultimate diagnosis without histopathological specimen examination seems to be difficult and can lead to misdiagnosis followed by inappropriate treatment. IgG4-RD responds well to steroid therapy. However, on this matter further studies are needed

Artificial neural network identifies nonsteroidal anti‐inflammatory drugs exacerbated respiratory disease (N‐ERD) cohort

Background: To date, there has been no reliable in vitro test to either diagnose or differentiate nonsteroidal anti-inflammatory drug (NSAID)–exacerbated respiratory disease (N-ERD). The aim of the present study was to develop and validate an artificial neural network (ANN) for the prediction of N-ERD in patients with asthma. Methods: This study used a prospective database of patients with N-ERD (n = 121) and aspirin-tolerant (n = 82) who underwent aspirin challenge from May 2014 to May 2018. Eighteen parameters, including clinical characteristics, inflammatory phenotypes based on sputum cells, as well as eicosanoid levels in induced sputum supernatant (ISS) and urine were extracted for the ANN. Results: The validation sensitivity of ANN was 94.12% (80.32%-99.28%), specificity was 73.08% (52.21%-88.43%), and accuracy was 85.00% (77.43%-92.90%) for the prediction of N-ERD. The area under the receiver operating curve was 0.83 (0.71-0.90). Conclusions: The designed ANN model seems to have powerful prediction capabilities to provide diagnosis of N-ERD. Although it cannot replace the gold-standard aspirin challenge test, the implementation of the ANN might provide an added value for identification of patients with N-ERD. External validation in a large cohort is needed to confirm our results

Biomarkers for predicting response to aspirin therapy in aspirin-exacerbated respiratory disease

BACKGROUND: Aspirin desensitization followed by daily aspirin use is an effective treatment for aspirin‐exacerbated respiratory disease (AERD). OBJECTIVE: To assess clinical features as well as genetic, immune, cytological and biochemical biomarkers that might predict a positive response to high‐dose aspirin therapy in AERD. METHODS: We enrolled 34 AERD patients with severe asthma who underwent aspirin desensitization followed by 52‐week aspirin treatment (650 mg/d). At baseline and at 52 weeks, clinical assessment was performed; phenotypes based on induced sputum cells were identified; eicosanoid, cytokine and chemokine levels in induced sputum supernatant were determined; and induced sputum expression of 94 genes was assessed. Responders to high‐dose aspirin were defined as patients with improvement in 5‐item Asthma Control Questionnaire score, 22‐item Sino‐Nasal Outcome Test (SNOT‐22) score and forced expiratory volume in 1 second at 52 weeks. RESULTS: There were 28 responders (82%). Positive baseline predictors of response included female sex (p = .002), higher SNOT‐22 score (p = .03), higher blood eosinophil count (p = .01), lower neutrophil percentage in induced sputum (p = .003), higher expression of the hydroxyprostaglandin dehydrogenase gene, HPGD (p = .004) and lower expression of the proteoglycan 2 gene, PRG2 (p = .01). The best prediction model included Asthma Control Test and SNOT‐22 scores, blood eosinophils and total serum immunoglobulin E. Responders showed a marked decrease in sputum eosinophils but no changes in eicosanoid levels. CONCLUSIONS AND CLINICAL RELEVANCE: Female sex, high blood eosinophil count, low sputum neutrophil percentage, severe nasal symptoms, high HPGD expression and low PRG2 expression may predict a positive response to long‐term high‐dose aspirin therapy in patients with AERD