Change in Referral Diagnoses and Diagnostic Delay in Hypogammaglobulinaemic Patients. Jiri Litzman Dept. Clin. Immunol. Allergol Masaryk University, Brno, Czech Republic

Clinical Manifestation of Immunoglobulin Deficiency Frequent and complicated infections of the respiratory tract. Patients suffer from recurrent attacks of bronchitis, sinusitis, pneumonia, otitis. Infections are caused mainly by encapsulated bacteria: Heamohilus influenzae, Str. pmeumoniae, St. aureus, Pseudomanonas sp. Typical complications are bronchiectasis and/or lung fibrosis.

Dept. Clin. Immunol. Allergol. St Anne´s University Hospital in Brno Founded in Tertiary referral center for South Moravia (approx inhabitants). Also patients from North Moravian region (approximately inhabitants) are frequently, but not constantly referred.

Regions of the Czech Republic

Spectrum of Primary Hypogamamglobulinemic Patients Diagnosed between PeriodPatientsCVIDX-LAOthers Before x non-X-linked HIMS x HIMS, 1x -chain def x Good syndrome Total967810

Diagnostic Delay and Number of Pneumonias before the Diagnosis of PID Was Made TotalDiagnostic delay*No. of pneumonias* ; 9,6 (0-38)5; 6,3 (0-25) ; 11,7 (0-44)1; 2,2 (0-10) ; 6,6 (0-36)0; 2,6 (0-15) Mann-Whitney test: Diagnostic delay: vs : P= 0, vs : P=0, vs P=0,0054 *Expressed as median; SD (range)

Diagnostic Delay in Hypogammaglobulinemic Patients Referred between P=0,628 P=0,005 P=0.032

Diagnostic delay Year of Diagnosis Diagnostic Delay in Hypogammaglobulinemic Patients Referred between Time trend in the length of the diagnostic delay was fitted using the exponencial regression method." Spearman´s correlation coefficient R= -0,359 (P < 0.001)

Diagnostic Delay and Number of Pneumonias before the Diagnosis of PID Was Made TotalDiagnostic delay*No. of pneumonias* ; 9,6 (0-38)5; 6,3 (0-25) ; 11,7 (0-44)1; 2,2 (0-10) ; 6,6 (0-36)0; 2,6 (0-15) Mann-Whitney test: Number of pneumonias: vs : P= vs : P= vs P= 0,0002 *Expressed as median; SD (range)

Number of Pneumonia Episodes during Diagnostic Delay in Hypogammaglobulinemic Patients Referred between P<0.001P=0,0317 P<0.001 Number of pneumonia episodes

Number of Pneumonias Number of Pneumonia Episodes durin Diagnostic Delay in Hypogammaglobulinemic Patients Referred between Time trend in the number of pneumonias during the diagnostic delay was fitted using the polynomial regression method." Spearman´s correlation coefficient R = -0,520 (P < 0.001).

Number of Hypogammaglobulinemic Patients who Experienced Pneumonia before Immunological Investigation was Made 17/19 25/37 13/33 89% 68% 39% P= P= P=0.012

Age at the time of diagnosis Year of Diagnosis Age when the Diagnose of PID was Made Spearman´s correlation coefficient R = 0,0075 (N.S)

Number of Patients Referred after previous Immunoglobulin Determination Total No. of Patients No of Patients in whom Igs Were Determined (37%) (54%) (55%)

Referral Diagnoses Different from Respiratory-Tract Infections Prior to 1980 : (total 7 referred patients): none : (total 19 referred patients): none : (total 37 referred patients) 1x hemolytic anemia, 1xhepatopathy, 1x herpes zoster, 1x family study in IgAD family, consequent progression of IgAD to CVID.

Referral Diagnoses Different from Respiratory-Tract Infections : (total 33 patients) 2x progression of IgA deficiency (both previously referred for recurrent RTI) 1x asthma, 1x herpes zoster, 1x problem with blood group determination, 1x generalized fatigue, 1x arthralgia, 1x family study in IgAD patient, 1x allopecia areata.

Hypogammaglobulinemic Patients without Severe Repiratory Tract Infections in the Time of Diagnosis

Conclusion The diagnostic delay in patients with primary antibody deficiency has markedly shortened during the observation period. Currently majority of patients did not experience pneumonia before the diagnose of hypogammaglobulinemia is made. A significant diagnostic delay in some patients was observed, even in the recently referred patients.

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