Download PDF
Exp Clin Endocrinol Diabetes 2008; 116(9): 515-519
DOI: 10.1055/s-2008-1062729
Article

© J. A. Barth Verlag in Georg Thieme Verlag KG Stuttgart · New York

Fatal Pneumocystis Jirovecii Pneumonia in a Case of Ectopic Cushing's Syndrome due to Neuroendocrine Carcinoma of the Kidney

A. Arlt 1 , B. Harbeck 1 , M. Anlauf 2 , I. Alkatout 2 , G. Klöppel 2 , U. R. Fölsch 1 , B. Bewig 1 , H. Mönig 1
  • 11st Department of Medicine, University Hospital Schleswig-Holstein, Campus Kiel, Kiel, Germany
  • 2Institute of Pathology, University Hospital Schleswig-Holstein, Campus Kiel, Kiel, Germany
Further Information

Publication History

received 08.10.2007 first decision 20.12.2007

accepted 13.02.2008

Publication Date:
01 April 2008 (online)

Also available at
    Permissions and Reprints

Abstract

Immunosuppression with subsequent opportunistic infections is a well-recognized complication of severe hypercortisolism. We report a case of fatal pneumocystis jirovecii pneumonia (formerly pneumocystis carinii pneumonia) in a case of ectopic Cushing's syndrome caused by a neuroendocrine carcinoma of the kidney. The 36-year old male patient had consulted a physician because of weight gain. Further endocrine diagnostic work-up revealed ACTH-dependent hypercortisolism of non-pituitary origin. Because of rapid clinical deterioration therapy with metyrapone was initiated. A neuroendocrine carcinoma of the right kidney with regional lymph node infiltration was identified and was suspected to be the source of the ACTH excess. Before any causal therapy could be initiated, the patient developed severe pneumocystis jirovecii pneumonia and died shortly thereafter from multiorgan failure one month after he first consulted a physician. Pneumocystosis has been reported in only a few cases of Cushing's syndrome. There seems to be a relationship between the degree of hypercortisolism and the susceptibility to opportunistic infections. Since ACTH concentrations may be excessively high in ectopic Cushing's syndrome and pneumocystosis may deteriorate as a consequence of decreasing circulating cortisol levels under adrenolytic therapy, prophylaxis against pneumocystis jirovecii infection should be considered.

Key words

cancer - glucocorticoids - hormones

References

  • 1 Anthony LB, Greco FA. Pneumocystis carinii pneumonia: a complication of Cushing's syndrome.  Ann Intern Med. 1981;  94 488-489
    CrossrefPubMedSearch in Google Scholar
  • 2 Ayala AR, Basaria S, Udelsman R, Westra WH, Wand GS. Corticotropin-independent Cushing's syndrome caused by an ectopic adrenal adenoma.  J Clin Endocrinol Metab. 2000;  85 2903-2906
    CrossrefPubMedSearch in Google Scholar
  • 3 Bakker RC, Gallas PR, Romijn JA, Wiersinga WM. Cushing's syndrome complicated by multiple opportunistic infections.  J Endocrinol Invest. 1998;  21 329-333
    CrossrefPubMedSearch in Google Scholar
  • 4 Cushing HW. The basophil adenomas of the pituitary body and their clinical manifestations (pituitary basophilism).  Bull Johns Hopkins Hosp. 1932;  50 137-195
    PubMedSearch in Google Scholar
  • 5 Drake WM, Perry LA, Hinds CJ, Lowe DG, Reznek RH, Besser GM. Emergency and prolonged use of intravenous etomidate to control hypercortisolemia in a patient with Cushing's syndrome and peritonitis.  J Clin Endocrinol Metab. 1998;  83 3542-3544
    CrossrefPubMedSearch in Google Scholar
  • 6 Findling JW, Buggy BP, Segerson TP, Raff H. Pneumocystis carinii pneumonia complicating intermittent Cushing's syndrome.  Wis Med J. 1986;  85 23-25
    PubMedSearch in Google Scholar
  • 7 Frenkel JK, Good JT, Shultz JA. Latent Pneumocystis infection of rats, relapse, and chemotherapy.  Lab Invest. 1966;  15 1559-1577
    PubMedSearch in Google Scholar
  • 8 Fulkerson WJ, Newman JH. Endogenous Cushing's syndrome complicated by Pneumocystis carinii pneumonia.  Am Rev Respir Dis. 1984;  129 188-189
    PubMedSearch in Google Scholar
  • 9 Graham BS, Tucker Jr WS. Opportunistic infections in endogenous Cushing's syndrome.  Ann Intern Med. 1984;  101 334-338
    PubMedSearch in Google Scholar
  • 10 Hannah J, Lippe B, Lai-Goldman M, Bhuta S. Oncocytic carcinoid of the kidney associated with periodic Cushing's syndrome.  Cancer. 1988;  61 2136-2140
    CrossrefPubMedSearch in Google Scholar
  • 11 Isidori AM, Kaltsas GA, Pozza C, Frajese V, Newell-Price J, Reznek RH, Jenkins PJ, Monson JP, Grossman AB, Besser GM. The ectopic adrenocorticotropin syndrome: clinical features, diagnosis, management, and long-term follow-up.  J Clin Endocrinol Metab. 2006;  91 371-377
    CrossrefPubMedSearch in Google Scholar
  • 12 Kaltsas GA, Giannulis MG, Newell-Price JD, Dacie JE, Thakkar C, Afshar F, Monson JP, Grossman AB, Besser GM, Trainer PJ. A critical analysis of the value of simultaneous inferior petrosal sinus sampling in Cushing's disease and the occult ectopic adrenocorticotropin syndrome.  J Clin Endocrinol Metab. 1999;  84 487-492
    CrossrefPubMedSearch in Google Scholar
  • 13 Keenan N, Dhillo WS, Williams GR, Todd JF. Unexpected shortness of breath in a patient with Cushing's syndrome.  Lancet. 2006;  367 446
    CrossrefPubMedSearch in Google Scholar
  • 14 Kim DS, Park SK, Choi WH, Kim TW, Choi YY, Jeon SC, Ryu JS. Pneumocystis carinii pneumonia associated with a rapid reduction of cortisol level in a patient with ectopic ACTH syndrome treated by octreotide and ketoconazole.  Exp Clin Endocrinol Diabetes. 2000;  108 146-150
    Thieme ConnectPubMedSearch in Google Scholar
  • 15 MacQuillen DP, Schroy PC, Hesketh PJ, Sugar AM. Pneumocystis carinii pneumonia complicating somatostatin therapy of Cushing's syndrome in a patient with metastatic pancreatic islet cell carcinoma and Zollinger-Ellison syndrome.  Am J Gastroenterol. 1991;  86 512-514
    PubMedSearch in Google Scholar
  • 16 Oldfield EH, Doppman JL, Nieman LK, Chrousos GP, Miller DL, Katz DA, Cutler Jr GB, Loriaux DL. Petrosal sinus sampling with and without corticotropin-releasing hormone for the differential diagnosis of Cushing's syndrome.  N Engl J Med. 1991;  325 897-905
    Search in Google Scholar
  • 17 Oosterhuis JK, Berg G van den, Monteban-Kooistra WE, Ligtenberg JJ, Tulleken JE, Meertens JH, Zijlstra JG. Life-threatening Pneumocystis jiroveci pneumonia following treatment of severe Cushing's syndrome.  Neth J Med. 2007;  65 215-217
    PubMedSearch in Google Scholar
  • 18 Pareja JG, Garland R, Koziel H. Use of adjunctive corticosteroids in severe adult non-HIV Pneumocystis carinii pneumonia.  Chest. 1998;  113 1215-1224
    CrossrefPubMedSearch in Google Scholar
  • 19 Plotz CM, Knowlton AI, Ragan C. The natural history of Cushing's syndrome.  Am J Med. 1952;  13 597-614
    CrossrefPubMedSearch in Google Scholar
  • 20 Russi E, Speich R, Hess T, Meili E, Walter E. Pneumocystis carinii pneumonia after transsphenoidal removal of microadenoma causing Cushing's syndrome.  Lancet. 1993;  341 1348-1349
    PubMedSearch in Google Scholar
  • 21 Sarlis NJ, Chanock SJ, Nieman LK. Cortisolemic indices predict severe infections in Cushing syndrome due to ectopic production of adrenocorticotropin.  J Clin Endocrinol Metab. 2000;  85 42-47
    CrossrefPubMedSearch in Google Scholar
  • 22 Schulte HM, Mönig H. Hormone measurement in blood from inferior petrosal sinus: clinical and experimental implications.  J Endocrinol Invest. 1993;  16 647-653
    CrossrefPubMedSearch in Google Scholar
  • 23 Sepkowitz KA. Opportunistic infections in patients with and patients without Acquired Immunodeficiency Syndrome.  Clin Infect Dis. 2002;  34 1098-1107
    CrossrefPubMedSearch in Google Scholar
  • 24 Stringer JR, Beard CB, Miller RF, Wakefield AE. A new name (Pneumocystis jiroveci) for Pneumocystis from humans.  Emerg Infect Dis. 2002;  8 891-896
    CrossrefPubMedSearch in Google Scholar
  • 25 Sufrin G, Chasan S, Golio A, Murphy GP. Paraneoplastic and serologic syndromes of renal adenocarcinoma.  Semin Urol. 1989;  7 158-171
    PubMedSearch in Google Scholar
  • 26 Thomas Jr CF, Limper AH. Pneumocystis pneumonia.  N Engl J Med. 2004;  350 2487-2498
    CrossrefPubMedSearch in Google Scholar

Correspondence

A. ArltMD 

1st Department of Medicine, UKSH-Campus Kiel

Schittenhelmstrasse 12

24105 Kiel

Germany

Phone: +49/431/597 13 93

Fax: +49/431/597 13 02

Email: aarlt@1med.uni-kiel.de