) Microbiology (direct culture of pathogens, PCR, antigen ELISA) Purulent sputum: ascertain colonizing pathogens and resistance pattern to antibiotics In case of chronic bronchiectasis, manage of colonising pathogens (Pseudomonas sppHaemophilius influenzae, Streptococcus pneumoniae, Staphylococcus aureus, Candida spp. and other individuals) with sensitivity to antibiotics Lungs Spirometry, CO diffusion test, blood gases Chest X-ray HR-CT in case of confirmed GILD Bronchoscopy + BAL in case of suspicion of GILD Lymphoproliferation Abdomen sonography CT-AbdomenMRT Lymph node biopsy Gastrointestinal tract Oesophogastroscopy Monthly trough levels – months At diagnosis Initially and repeatedly in case of suspected combined immunodeficiency At diagnosis At diagnosis On demand Intervals (-) months; additional normally in case of identified autoimmune cytopenia On demand On demand at diagnosis or throughout follow-up- months and on demand months (-) months months and on demand At diagnosis; on demand months On demand; in case of suspected lymphoma In case of clinical symptoms and each months in case of elevated risk for creating intestinal malignancy On demand On demandColonoscopy Central nervous system MRT, liquor evaluation in case of neurological symptoms (exclusion of enteroviral infection)BAL, bronchoalveolar lavage; CT, computed tomography; GILD, FG9065 site granulomatous interstitial lung disease; HR-CT, high-resolution computed tomography; MRT, magnetic resonance computed tomography.vaccinated for diagnostic purposes before the start of immunoglobulin substitution. The subsequent stage of diagnosis is flow cytometric analysis of lymphocyte subpopulations, such as total T, B and natural killer cells, to distinguish late manifesting Xlinked PubMed ID:http://www.ncbi.nlm.nih.gov/pubmed/28422762?dopt=Abstract agammaglobulinemia (B cells) and combined immunodeficiencies (CD cells l). The classification of CVID patients with all the separation of Bcell subpopulations is reserved for specialized immunodeficiency centers. A bone marrow biopsy ought to be performed in sufferers with low B-cell numbers 
and if lymphoma or myelodysplasia is suspected. Furthermore, numerous diagnostic procedures at first check out and throughout follow-ups are indicated for the control of achievable secondary complications (summarized in Table).Therapy, organic course and prognosis Present therapy of CVID could be categorized as follows: regular and enough substitution with immunoglobulins (IgG trough levelsgL); targeted antibiotic therapy of (breakthrough) infections; sufficient treatment of complications; and in selected sufferers with severe hematological changes (chronic transfusion need, leukopenia, thrombocytopenia), secondary malignancies and suspected combined immunodeficiency, allogeneic peripheral stem cell transplantation is becoming deemed in skilled centersThe immunoglobulin replacement therapy will be the mainstay of therapy; of CVID individuals are on either intravenous (IVIg) or subcutaneous (SCIg) therapy -. Intramuscular MedChemExpress GNE-140 (racemate) administration is no longerSalzer et al. Arthritis Investigation Therapy , : http:arthritis-researchcontentPage ofrecommended since this route will not assure helpful serum levels but is associated with a greater price of unwanted effects. The current regular dosage when administered intravenously is to mgkg every single to weeks. For subcutaneous administration, this corresponds to to mgkg per week. The goal may be the handle of infections, which is reached at different person IgG trough levelsAs a target value, IgG trough levels of far more than gL are desirable befo.) Microbiology (direct culture of pathogens, PCR, antigen ELISA) Purulent sputum: establish colonizing pathogens and resistance pattern to antibiotics In case of chronic bronchiectasis, control of colonising pathogens (Pseudomonas sppHaemophilius influenzae, Streptococcus pneumoniae, Staphylococcus aureus, Candida spp. and other individuals) with sensitivity to antibiotics Lungs Spirometry, CO diffusion test, blood gases Chest X-ray HR-CT in case of confirmed GILD Bronchoscopy + BAL in case of suspicion of GILD Lymphoproliferation Abdomen sonography CT-AbdomenMRT Lymph node biopsy Gastrointestinal tract Oesophogastroscopy Monthly trough levels – months At diagnosis Initially and repeatedly in case of suspected combined immunodeficiency At diagnosis At diagnosis On demand Intervals (-) months; extra usually in case of recognized autoimmune cytopenia On demand On demand at diagnosis or in the course of follow-up- months and on demand months (-) months months and on demand At diagnosis; on demand months On demand; in case of suspected lymphoma In case of clinical symptoms and just about every months in case of enhanced risk for building intestinal malignancy On demand On demandColonoscopy Central nervous system MRT, liquor evaluation in case of neurological symptoms (exclusion of enteroviral infection)BAL, bronchoalveolar lavage; CT, computed tomography; GILD, granulomatous interstitial lung illness; HR-CT, high-resolution computed tomography; MRT, magnetic resonance computed tomography.vaccinated for diagnostic purposes prior to the commence of immunoglobulin substitution. The subsequent stage of diagnosis is flow cytometric analysis of lymphocyte subpopulations, like total T, B and natural killer cells, to distinguish late manifesting Xlinked PubMed ID:http://www.ncbi.nlm.nih.gov/pubmed/28422762?dopt=Abstract agammaglobulinemia (B cells) and combined immunodeficiencies (CD cells l). The classification of CVID individuals using the separation of Bcell subpopulations is reserved for specialized immunodeficiency centers. A bone marrow biopsy should be performed in patients with low B-cell numbers and if lymphoma or myelodysplasia is suspected. Moreover, several diagnostic procedures at first go to and during follow-ups are indicated for the handle of probable secondary complications (summarized in Table).Therapy, organic course and prognosis Existing therapy of CVID is usually categorized as follows: typical and adequate substitution with immunoglobulins (IgG trough levelsgL); targeted antibiotic remedy of (breakthrough) infections; adequate therapy of complications; and in chosen sufferers with extreme hematological alterations (chronic transfusion will need, leukopenia, thrombocytopenia), secondary malignancies and suspected combined immunodeficiency, allogeneic peripheral stem cell transplantation is being regarded in skilled centersThe immunoglobulin replacement therapy 
is the mainstay of therapy; of CVID sufferers are on either intravenous (IVIg) or subcutaneous (SCIg) remedy -. Intramuscular administration is no longerSalzer et al. Arthritis Research Therapy , : http:arthritis-researchcontentPage ofrecommended mainly because this route will not ensure effective serum levels but is connected using a greater rate of unwanted effects. The current regular dosage when administered intravenously should be to mgkg each to weeks. For subcutaneous administration, this corresponds to to mgkg per week. The target may be the handle of infections, which is reached at unique person IgG trough levelsAs a target worth, IgG trough levels of much more than gL are desirable befo.