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Change in R eferral D iagnoses and D iagnostic D elay in H ypogammaglobulinaemic P atients. Jiri Litzman Dept. Clin. Immunol. Allergol Masaryk University, Brno, Czech Republic. Clinical M anifestation of I mmunoglobulin D eficiency.
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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 1981. • Tertiary referral center for South Moravia(approx 2 237 000 inhabitants). • Also patients from North Moravian region (approximately 1 910 000 inhabitants) are frequently, but not constantly referred.
Spectrum of Primary Hypogamamglobulinemic Patients Diagnosed between 1965-2008
Diagnostic Delay and Number of Pneumonias before the Diagnosis of PID Was Made *Expressed as median; SD (range) Mann-Whitney test: Diagnostic delay: 1981-1990 vs1991-2000: P= 0,6280 1991-2000 vs 2001-2008: P=0,0352 1981-1990 vs 2001-2008 P=0,0054
Diagnostic Delay in Hypogammaglobulinemic Patients Referred between 1981-2008 P=0,005 P=0.032 P=0,628
Diagnostic delay Year of Diagnosis Diagnostic Delay in Hypogammaglobulinemic Patients Referred between 1981-2008 Spearman´scorrelation coefficient R= -0,359(P< 0.001) Time trend in the length of the diagnostic delay was fitted using the exponencial regression method."
Diagnostic Delay and Number of Pneumonias before the Diagnosis of PID Was Made *Expressed as median; SD (range) Mann-Whitney test: Number of pneumonias: 1981-1990 vs1991-2000: P=0.0311 1991-2000 vs 2001-2008: P=0.0211 1981-1990 vs 2001-2008 P= 0,0002
Number of Pneumonia Episodes during Diagnostic Delay in Hypogammaglobulinemic Patients Referred between 1981-2008 P<0.001 P<0.001 P=0,0317 Number of pneumonia episodes
Number of Pneumonias Number of Pneumonia Episodes durin Diagnostic Delay in Hypogammaglobulinemic Patients Referred between 1981-2008 Spearman´s correlation coefficientR = -0,520 (P< 0.001). Time trend in the number of pneumonias during the diagnostic delay was fitted using the polynomial regression method."
Number of Hypogammaglobulinemic Patients who Experienced Pneumonia before Immunological Investigation was Made P=0.0003 P=0.0546 P=0.012 68% 39% 89% 17/19 13/33 25/37
Age at the time of diagnosis Year of Diagnosis Age when the Diagnose of PID was Made Spearman´scorrelation coefficient R = 0,0075 (N.S)
Number of Patients Referred after previous Immunoglobulin Determination
Referral Diagnoses Different from Respiratory-Tract Infections • Prior to 1980 : (total 7 referred patients): none • 1981- 1990: (total 19 referred patients): none • 1991-2000: (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 • 2001-2008: (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.
