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TABLE IX-44 Evidence for CRS management with immunodeficiency treatment Study Year LOE Study Design Study Groups Clinical Endpoint Conclusions Tas 1280 1990 2 Randomized control trial Double-blind cross-over trial (n = 20) TP-1 then placebo Placebo then TP-1 Endoscopy, DTH skin test, lymphocyte subsets, MIF assay, and other laboratory tests. Refractory CRS patients were successfully treated TP-1, restoring some laboratory parameters phosphoinositide 3-kinase delta syndrome (APDS) Clinical manifestations, immunological phenotypes, treatment modalities examined. APDS should be suspected in patients with history of recurrent respiratory

infections, lymphoproliferation, and raised IgM levels. 25.9% patients had RS. The majority of APDS patients were placed on long-term Ig

replacement therapy. Hematopoietic stem cell transplantation was used in 12.8% of patients.

IVIG is more effective in reducing lower respiratory infections than reducing RS.

Immune dysfunction CRS patients had similar outcomes as control CRS patients.

Medical therapy, particularly Ig replacement therapy, appears to be

most effective when administered at high doses early in the disease course. The addition of surgery is less clearly

supported, but may also provide benefit if performed early.

High dose IVIG therapy was more effective than low dose IVIG.

Ig level, lymphocyte subsets, culture test, CT

Data was collected pertaining to immune dysfunction in patients with CRS, the

clinical workup for these patients, and the

effectiveness of medical

and surgical treatments.

Endoscopy, sputum cultures, Ig level, chest and sinus radiographs, spirometry. QoL measurement nasal endoscopy, sinusCT.

Khalid 954 2010 4 Case-control study CRS with immune dysfunction or autoimmune disease (n = 22) CRS control (n = 22)

CVID patients on IVIG for a mean of 11.5 years. (n = 224)

Mazza 1284 2016 3 Systematic review 39 studies, predominantly level 4 evidence, of patients with primary immunodeficiency and CRS met inclusion criteria. Quinti 40 2007 3 Multicenter prospective study Roifman 951 1988 3 Prospective cross-over study

6 months of: 1. High dose (0.6 g/kg/mo) IVIG 2. Low dose (0.2 g/kg/mo) IVIG

Jamee 1283 2019 3 Systematic review 243 patients with activated