• Klíčová slova
• Goodův syndrom,
• MeSH
• běžná variabilní imunodeficience imunologie   komplikace   MeSH
• deficience IgA imunologie   komplikace   MeSH
• dospělí * MeSH
• lidé MeSH
• opožděná diagnóza MeSH
• primární imunodeficience * diagnóza   terapie   MeSH
• Check Tag
• dospělí * MeSH
• lidé MeSH
• Publikační typ
• přehledy MeSH

Screening for celiac disease (CD) dramatically improved when techniques able to measure blood autoantibodies against tissue transglutaminase 2 (TTG) were developed. Although typically increased in CD, these antibodies are not pathognomonic since they are also detected in several other autoimmune processes. IgA deficiency among celiac patients is more frequent than in general population (up to 25% vs 1-3%). This led to develop kits able to measure IgG-TTG, which until today represent a helpful diagnostic tool during diagnosis of CD in IgA deficient individuals. Today, commercial kits measuring IgG- TTG (and other) antibodies are widely available, are frequently used and create confusion in diagnosing CD in IgA-sufficient individuals. This is attributed to the fact that sensitivity and specificity of IgG-TTG is lower when applied to IgA-sufficient persons, and also because IgG-TTG is detected in several autoimmune disorders, with variable frequency and isotypes depending on the condition. Evidence analyzed indicate that to date available data: i) is insufficient to understand the difference of classes and subclasses detected in CD and other autoimmune conditions; ii) does not support the use of IgG-TTG for diagnosing CD in IgA-sufficient individuals and therefore iii) IgG should not be used in the routine diagnostic process of CD.

• MeSH
• autoprotilátky krev   MeSH
• celiakie * diagnóza   patofyziologie   prevence a kontrola   MeSH
• deficience IgA * komplikace   MeSH
• imunoglobulin A krev   MeSH
• imunoglobulin G krev   MeSH
• lidé MeSH
• prediktivní hodnota testů MeSH
• sérologické testy MeSH
• transglutaminasy imunologie   krev   MeSH
• Check Tag
• lidé MeSH

Concomitant occurrence of celiac disease (CeD) and Crohn's disease (CD) is rare and has been reported in several case reports and series. We report a case of a 63-year-old man with proximal CD, CeD and selective immunoglobulin (Ig) A deficiency (SIgAD). The patient was admitted in our Clinic for evaluation of epigastric pain, nausea, vomiting and weight loss (up to 7 kg) appeared in the last 3 months. The imaging findings from abdominal ultrasound and CT enterography raised suspicion of a lymphoproliferative disease of the jejunum. To exclude the development of lymphoma based on long standing untreated atypical CeD, upper endoscopy with biopsies and serological tests were conducted, showing and subtotal villous atrophy and elevated levels of IgG tissue transglutaminase tTG. Histopathology documented CD (A3,L4,B2 according to Montreal's classification).

• MeSH
• celiakie * imunologie   patologie   terapie   MeSH
• Crohnova nemoc chirurgie   imunologie   patologie   MeSH
• deficience IgA diagnóza   komplikace   MeSH
• komorbidita MeSH
• lidé středního věku MeSH
• lidé MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• kazuistiky MeSH

Monoclonal gammopathy-associated IgA pemphigus is a debilitating skin disorder with inconsistent response to treatment. A 61-year-old woman with IgA pemphigus and monoclonal gammopathy of unknown significance had been treated unsuccessfully with cyclophosphamide/dexamethasone and then with rituximab. When the monoclonal gammopathy progressed to multiple myeloma, the patient received treatment with cyclophosphamide/doxorubicin/dexamethasone but there was no clinical response. Second-line therapy with a thalidomide/cyclophosphamide/dexamethasone combination led to severe exacerbation of the skin disorder. However, therapy with a combination regimen that included bortezomib, cyclophosphamide and dexamethasone resulted in complete and durable remission of multiple myeloma and IgA pemphigus. This suggests that bortezomib-based therapy is useful for the treatment of the rare dermatologic disorder associated with IgA gammopathy.

• MeSH
• cyklofosfamid aplikace a dávkování   MeSH
• deficience IgA komplikace   prevence a kontrola   MeSH
• dexamethason aplikace a dávkování   MeSH
• indukce remise MeSH
• kyseliny boronové aplikace a dávkování   MeSH
• lidé středního věku MeSH
• lidé MeSH
• mnohočetný myelom komplikace   farmakoterapie   MeSH
• paraproteinemie komplikace   prevence a kontrola   MeSH
• pemfigus komplikace   prevence a kontrola   MeSH
• protokoly protinádorové kombinované chemoterapie aplikace a dávkování   MeSH
• pyraziny aplikace a dávkování   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• práce podpořená grantem MeSH

• MeSH
• dárci krve MeSH
• deficience IgA epidemiologie   komplikace   MeSH
• dítě MeSH
• dospělí MeSH
• infekce dýchací soustavy MeSH
• lidé MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé MeSH
• Geografické názvy
• Česká republika MeSH

• MeSH
• alergie MeSH
• deficience IgA komplikace   krev   MeSH
• dítě MeSH
• imunoglobuliny krev   MeSH
• lidé MeSH
• nemoci dýchací soustavy imunologie   MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• Publikační typ
• srovnávací studie MeSH

• MeSH
• běžná variabilní imunodeficience imunologie   krev   terapie   MeSH
• deficience IgA imunologie   komplikace   krev   MeSH
• imunoglobulin G imunologie   krev   MeSH
• imunoglobulin M imunologie   krev   MeSH
• lidé MeSH
• lymfocyty metabolismus   MeSH
• mladiství MeSH
• pasivní imunizace MeSH
• progrese nemoci MeSH
• Check Tag
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• Publikační typ
• kazuistiky MeSH