Incidence of Pneumocystis jiroveci Pneumonia among Groups at Risk in HIV-negative Patients.

[waldenström macroglobulinemia]

Pneumocystis jiroveci pneumonia in HIV-negative immunocompromised patients is associated with high mortality rates . Although trimethoprim-sulfamethoxazole (TMP-SMX ) provides a very effective prophylaxis , pneumocystosis still occurs and may even be emerging , due to sub-optimal characterization of patients most at risk , hence precluding targeted prophylaxis .We retrospectively analyzed all cases of documented pneumocystosis in HIV-negative patients admitted in our institution , a referral center in the area , from January 1990 to June 2010 , and extracted data on their underlying condition (s ). To estimate incidence rates within each condition , we estimated the number of patients followed-up in our area for each condition , by measuring the number of patients admitted with the corresponding international classification diagnostic code , through the national hospital discharge database (PMSI ).From 1990 to 2010 , 293 cases of pneumocystosis were documented , of whom 154 (52 .6 %) tested negative for HIV . The main underlying conditions were hematological malignancies (32 .5 %), solid tumors (18 .2 %), inflammatory diseases (14 .9 %), solid organ transplant (12 .3 %), and vasculitis (9 .7 %). Estimated incidence rates could be ranked in three categories : i ) high risk (incidence rates > 45 cases per 100 ,000 patient-year ): polyarteritis nodosa , granulomatosis with polyangiitis , polymyositis /dermatopolymyositis , acute leukemia , chronic lymphocytic leukemia , and non-Hodgkin lymphoma ; ii ) intermediate risk (25 -45 cases per 100 ,000 patient-year ): Waldenström macroglobulinemia , multiple myeloma , and central nervous system cancer ; and iii ) low risk (< 25 cases per 100 ,000 patient-year ): other solid tumors , inflammatory diseases , and Hodgkin lymphoma .These estimates may be used as a guide to better target pneumocystosis prophylaxis in the groups most at risk .

Diseases
Validation

Diseases presenting "high risk" symptom

22q11.2 deletion syndrome

acute rheumatic fever

adrenal incidentaloma

alpha-thalassemia

aniridia

canavan disease

congenital diaphragmatic hernia

congenital toxoplasmosis

cowden syndrome

cushing syndrome

cutaneous mastocytosis

esophageal adenocarcinoma

esophageal carcinoma

esophageal squamous cell carcinoma

fabry disease

harlequin ichthyosis

heparin-induced thrombocytopenia

hirschsprung disease

hodgkin lymphoma, classical

homocystinuria without methylmalonic aciduria

hydrocephalus with stenosis of the aqueduct of sylvius

krabbe disease

legionellosis

liposarcoma

locked-in syndrome

oligodontia

oral submucous fibrosis

papillon-lefèvre syndrome

pendred syndrome

phenylketonuria

primary hyperoxaluria type 1

severe combined immunodeficiency

sneddon syndrome

waldenström macroglobulinemia

werner syndrome

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