Determining the incidence of Pneumocystis pneumonia in patients with autoimmune blistering diseases not receiving routine prophylaxis

Kyle T. Amber, Aniek Lamberts, Farzan Solimani, Arianna F. Agnoletti, Dario Didona, Ilona Euverman, Emanuele Cozzani, Lee Haur Yueh, Giovanni Di Zenzo, Yael Anne Leshem, Daniel Mimouni, Michael Hertl, Barbara Horvath

Research output: Contribution to journalArticle

Abstract

IMPORTANCE: Pneumocystis pneumonia (PCP) is a potentially lethal opportunistic infection that primary prophylaxis can help prevent. The risk of prophylactic therapy must be weighed against the incidence of PCP in the patient population. Prophylaxis most frequently involves trimethoprim-sulfamethoxazole, with second-line therapies, including atovaquone, dapsone, and pentamide. The indication for prophylaxis in immunocompromised patients without HIV is less well defined. Previously, an incidence of at least 3.5% has been proposed as a cutoff to justify prophylaxis. OBJECTIVE: To assess the incidence of PCP in patients with autoimmune blistering diseases receiving no routine prophylaxis. DESIGN, SETTING, AND PARTICIPANTS: This was a retrospective analysis of patient medical records to determine the incidence of PCP infections. The multicenter study was performed at tertiary care centers that provide care for patients with autoimmune blistering disease in Germany, Italy, Singapore, Israel, and the Netherlands. Patients had a confirmed diagnosis of pemphigus vulgaris/foliaceus, bullous pemphigoid, epidermolysis bullosa acquisita, mucous membrane pemphigoid/cicatricial pemphigoid, or anti-p200 pemphigoid. MAIN OUTCOMES AND MEASURES: To determine the incidence of PCP defined as patients with the International Classification of Diseases, Ninth Revision (ICD-9), code 136.3, for PCP, or free text documentation of PCP occurring based on characteristic radiographic findings with elevated lactate dehydrogenase, or hospitalization for pneumonia with bronchioalveolar lavage demonstrating Pneumocystis jiroveci on confirmatory stains. RESULTS: A total of 801 patients with autoimmune blistering diseases were included in this study; their mean (SD) age was 66.5 (17.6) years, and a total of 465 (58%) were female. Only 1 patient developed PCP, resulting in an incidence rate of 0.1%. This incidence significantly fell below the recommended threshold of 3.5% (0.1% vs 3.5%, χ1 2 = 27.0; P < .001). This incidence was significantly lower than the previously reported incidence of PCP in all immunosuppressed dermatologic patients (0.1% vs 1.3%; χ1 2 = 8.2; P = .004). CONCLUSIONS AND RELEVANCE: Routine Pneumocystis prophylaxis for patients with autoimmune blistering diseases does not seem to be warranted. Patients with autoimmune blistering disease seem to have a lower risk of PCP than the general population of immunosuppressed dermatology patients. Risks of routine prophylaxis include hyperkalemia, hypoglycemia, photosensitivity, thrombocytopenia, and more rare adverse reactions.

Original languageEnglish
Pages (from-to)1137-1141
Number of pages5
JournalJAMA Dermatology
Volume153
Issue number11
DOIs
Publication statusPublished - Nov 1 2017

Fingerprint

Pneumocystis Pneumonia
Autoimmune Diseases
Incidence
Bullous Pemphigoid
International Classification of Diseases
Pemphigus
Epidermolysis Bullosa Acquisita
Atovaquone
Pneumocystis
Benign Mucous Membrane Pemphigoid
Pneumocystis Infections
Pneumocystis carinii
Dapsone
Hyperkalemia
Singapore
Opportunistic Infections
Sulfamethoxazole Drug Combination Trimethoprim
Immunocompromised Host
Bronchoalveolar Lavage
Israel

ASJC Scopus subject areas

  • Dermatology

Cite this

Amber, K. T., Lamberts, A., Solimani, F., Agnoletti, A. F., Didona, D., Euverman, I., ... Horvath, B. (2017). Determining the incidence of Pneumocystis pneumonia in patients with autoimmune blistering diseases not receiving routine prophylaxis. JAMA Dermatology, 153(11), 1137-1141. https://doi.org/10.1001/jamadermatol.2017.2808

Determining the incidence of Pneumocystis pneumonia in patients with autoimmune blistering diseases not receiving routine prophylaxis. / Amber, Kyle T.; Lamberts, Aniek; Solimani, Farzan; Agnoletti, Arianna F.; Didona, Dario; Euverman, Ilona; Cozzani, Emanuele; Yueh, Lee Haur; Di Zenzo, Giovanni; Leshem, Yael Anne; Mimouni, Daniel; Hertl, Michael; Horvath, Barbara.

In: JAMA Dermatology, Vol. 153, No. 11, 01.11.2017, p. 1137-1141.

Research output: Contribution to journalArticle

Amber, KT, Lamberts, A, Solimani, F, Agnoletti, AF, Didona, D, Euverman, I, Cozzani, E, Yueh, LH, Di Zenzo, G, Leshem, YA, Mimouni, D, Hertl, M & Horvath, B 2017, 'Determining the incidence of Pneumocystis pneumonia in patients with autoimmune blistering diseases not receiving routine prophylaxis', JAMA Dermatology, vol. 153, no. 11, pp. 1137-1141. https://doi.org/10.1001/jamadermatol.2017.2808
Amber, Kyle T. ; Lamberts, Aniek ; Solimani, Farzan ; Agnoletti, Arianna F. ; Didona, Dario ; Euverman, Ilona ; Cozzani, Emanuele ; Yueh, Lee Haur ; Di Zenzo, Giovanni ; Leshem, Yael Anne ; Mimouni, Daniel ; Hertl, Michael ; Horvath, Barbara. / Determining the incidence of Pneumocystis pneumonia in patients with autoimmune blistering diseases not receiving routine prophylaxis. In: JAMA Dermatology. 2017 ; Vol. 153, No. 11. pp. 1137-1141.
@article{56353983f42d4d43a521b4f200343863,
title = "Determining the incidence of Pneumocystis pneumonia in patients with autoimmune blistering diseases not receiving routine prophylaxis",
abstract = "IMPORTANCE: Pneumocystis pneumonia (PCP) is a potentially lethal opportunistic infection that primary prophylaxis can help prevent. The risk of prophylactic therapy must be weighed against the incidence of PCP in the patient population. Prophylaxis most frequently involves trimethoprim-sulfamethoxazole, with second-line therapies, including atovaquone, dapsone, and pentamide. The indication for prophylaxis in immunocompromised patients without HIV is less well defined. Previously, an incidence of at least 3.5{\%} has been proposed as a cutoff to justify prophylaxis. OBJECTIVE: To assess the incidence of PCP in patients with autoimmune blistering diseases receiving no routine prophylaxis. DESIGN, SETTING, AND PARTICIPANTS: This was a retrospective analysis of patient medical records to determine the incidence of PCP infections. The multicenter study was performed at tertiary care centers that provide care for patients with autoimmune blistering disease in Germany, Italy, Singapore, Israel, and the Netherlands. Patients had a confirmed diagnosis of pemphigus vulgaris/foliaceus, bullous pemphigoid, epidermolysis bullosa acquisita, mucous membrane pemphigoid/cicatricial pemphigoid, or anti-p200 pemphigoid. MAIN OUTCOMES AND MEASURES: To determine the incidence of PCP defined as patients with the International Classification of Diseases, Ninth Revision (ICD-9), code 136.3, for PCP, or free text documentation of PCP occurring based on characteristic radiographic findings with elevated lactate dehydrogenase, or hospitalization for pneumonia with bronchioalveolar lavage demonstrating Pneumocystis jiroveci on confirmatory stains. RESULTS: A total of 801 patients with autoimmune blistering diseases were included in this study; their mean (SD) age was 66.5 (17.6) years, and a total of 465 (58{\%}) were female. Only 1 patient developed PCP, resulting in an incidence rate of 0.1{\%}. This incidence significantly fell below the recommended threshold of 3.5{\%} (0.1{\%} vs 3.5{\%}, χ1 2 = 27.0; P < .001). This incidence was significantly lower than the previously reported incidence of PCP in all immunosuppressed dermatologic patients (0.1{\%} vs 1.3{\%}; χ1 2 = 8.2; P = .004). CONCLUSIONS AND RELEVANCE: Routine Pneumocystis prophylaxis for patients with autoimmune blistering diseases does not seem to be warranted. Patients with autoimmune blistering disease seem to have a lower risk of PCP than the general population of immunosuppressed dermatology patients. Risks of routine prophylaxis include hyperkalemia, hypoglycemia, photosensitivity, thrombocytopenia, and more rare adverse reactions.",
author = "Amber, {Kyle T.} and Aniek Lamberts and Farzan Solimani and Agnoletti, {Arianna F.} and Dario Didona and Ilona Euverman and Emanuele Cozzani and Yueh, {Lee Haur} and {Di Zenzo}, Giovanni and Leshem, {Yael Anne} and Daniel Mimouni and Michael Hertl and Barbara Horvath",
year = "2017",
month = "11",
day = "1",
doi = "10.1001/jamadermatol.2017.2808",
language = "English",
volume = "153",
pages = "1137--1141",
journal = "JAMA Dermatology",
issn = "2168-6068",
publisher = "American Medical Association",
number = "11",

}

TY - JOUR

T1 - Determining the incidence of Pneumocystis pneumonia in patients with autoimmune blistering diseases not receiving routine prophylaxis

AU - Amber, Kyle T.

AU - Lamberts, Aniek

AU - Solimani, Farzan

AU - Agnoletti, Arianna F.

AU - Didona, Dario

AU - Euverman, Ilona

AU - Cozzani, Emanuele

AU - Yueh, Lee Haur

AU - Di Zenzo, Giovanni

AU - Leshem, Yael Anne

AU - Mimouni, Daniel

AU - Hertl, Michael

AU - Horvath, Barbara

PY - 2017/11/1

Y1 - 2017/11/1

N2 - IMPORTANCE: Pneumocystis pneumonia (PCP) is a potentially lethal opportunistic infection that primary prophylaxis can help prevent. The risk of prophylactic therapy must be weighed against the incidence of PCP in the patient population. Prophylaxis most frequently involves trimethoprim-sulfamethoxazole, with second-line therapies, including atovaquone, dapsone, and pentamide. The indication for prophylaxis in immunocompromised patients without HIV is less well defined. Previously, an incidence of at least 3.5% has been proposed as a cutoff to justify prophylaxis. OBJECTIVE: To assess the incidence of PCP in patients with autoimmune blistering diseases receiving no routine prophylaxis. DESIGN, SETTING, AND PARTICIPANTS: This was a retrospective analysis of patient medical records to determine the incidence of PCP infections. The multicenter study was performed at tertiary care centers that provide care for patients with autoimmune blistering disease in Germany, Italy, Singapore, Israel, and the Netherlands. Patients had a confirmed diagnosis of pemphigus vulgaris/foliaceus, bullous pemphigoid, epidermolysis bullosa acquisita, mucous membrane pemphigoid/cicatricial pemphigoid, or anti-p200 pemphigoid. MAIN OUTCOMES AND MEASURES: To determine the incidence of PCP defined as patients with the International Classification of Diseases, Ninth Revision (ICD-9), code 136.3, for PCP, or free text documentation of PCP occurring based on characteristic radiographic findings with elevated lactate dehydrogenase, or hospitalization for pneumonia with bronchioalveolar lavage demonstrating Pneumocystis jiroveci on confirmatory stains. RESULTS: A total of 801 patients with autoimmune blistering diseases were included in this study; their mean (SD) age was 66.5 (17.6) years, and a total of 465 (58%) were female. Only 1 patient developed PCP, resulting in an incidence rate of 0.1%. This incidence significantly fell below the recommended threshold of 3.5% (0.1% vs 3.5%, χ1 2 = 27.0; P < .001). This incidence was significantly lower than the previously reported incidence of PCP in all immunosuppressed dermatologic patients (0.1% vs 1.3%; χ1 2 = 8.2; P = .004). CONCLUSIONS AND RELEVANCE: Routine Pneumocystis prophylaxis for patients with autoimmune blistering diseases does not seem to be warranted. Patients with autoimmune blistering disease seem to have a lower risk of PCP than the general population of immunosuppressed dermatology patients. Risks of routine prophylaxis include hyperkalemia, hypoglycemia, photosensitivity, thrombocytopenia, and more rare adverse reactions.

AB - IMPORTANCE: Pneumocystis pneumonia (PCP) is a potentially lethal opportunistic infection that primary prophylaxis can help prevent. The risk of prophylactic therapy must be weighed against the incidence of PCP in the patient population. Prophylaxis most frequently involves trimethoprim-sulfamethoxazole, with second-line therapies, including atovaquone, dapsone, and pentamide. The indication for prophylaxis in immunocompromised patients without HIV is less well defined. Previously, an incidence of at least 3.5% has been proposed as a cutoff to justify prophylaxis. OBJECTIVE: To assess the incidence of PCP in patients with autoimmune blistering diseases receiving no routine prophylaxis. DESIGN, SETTING, AND PARTICIPANTS: This was a retrospective analysis of patient medical records to determine the incidence of PCP infections. The multicenter study was performed at tertiary care centers that provide care for patients with autoimmune blistering disease in Germany, Italy, Singapore, Israel, and the Netherlands. Patients had a confirmed diagnosis of pemphigus vulgaris/foliaceus, bullous pemphigoid, epidermolysis bullosa acquisita, mucous membrane pemphigoid/cicatricial pemphigoid, or anti-p200 pemphigoid. MAIN OUTCOMES AND MEASURES: To determine the incidence of PCP defined as patients with the International Classification of Diseases, Ninth Revision (ICD-9), code 136.3, for PCP, or free text documentation of PCP occurring based on characteristic radiographic findings with elevated lactate dehydrogenase, or hospitalization for pneumonia with bronchioalveolar lavage demonstrating Pneumocystis jiroveci on confirmatory stains. RESULTS: A total of 801 patients with autoimmune blistering diseases were included in this study; their mean (SD) age was 66.5 (17.6) years, and a total of 465 (58%) were female. Only 1 patient developed PCP, resulting in an incidence rate of 0.1%. This incidence significantly fell below the recommended threshold of 3.5% (0.1% vs 3.5%, χ1 2 = 27.0; P < .001). This incidence was significantly lower than the previously reported incidence of PCP in all immunosuppressed dermatologic patients (0.1% vs 1.3%; χ1 2 = 8.2; P = .004). CONCLUSIONS AND RELEVANCE: Routine Pneumocystis prophylaxis for patients with autoimmune blistering diseases does not seem to be warranted. Patients with autoimmune blistering disease seem to have a lower risk of PCP than the general population of immunosuppressed dermatology patients. Risks of routine prophylaxis include hyperkalemia, hypoglycemia, photosensitivity, thrombocytopenia, and more rare adverse reactions.

UR - http://www.scopus.com/inward/record.url?scp=85034657945&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85034657945&partnerID=8YFLogxK

U2 - 10.1001/jamadermatol.2017.2808

DO - 10.1001/jamadermatol.2017.2808

M3 - Article

C2 - 28854309

AN - SCOPUS:85034657945

VL - 153

SP - 1137

EP - 1141

JO - JAMA Dermatology

JF - JAMA Dermatology

SN - 2168-6068

IS - 11

ER -