Atypical Presentation of Scedosporium Pneumonia

Gabriel Johnson, DOLeslie Spikes, MD

Department of Internal MedicineUniversity of Kansas Medical Center

Kansas City, KS

Introduction Provide a brief overview of scedosporium

epidemiology

Present an unusual case of a life-threatening Scedosporium infection in a patient without typical risk factors for fungemia

Identify diagnostic and therapeutic challenges

Scedosporium Infections in Humans Localized infections:

Bronchiectatic lungs Mycetomas

Disseminated infections: Transplant wards

Up to 10% of cystic fibrosis patients colonized in transplant wards

Near drowning events Rarely in the immuno-competent

Cortez et. Al. Infections Caused by Scedosporium spp. Clin Microbiol Rev. 2008 January; 21(1): 157–197.

Complication of organ transplant Study of 80 cases of scedosporium infection in

transplant patients at 5 academic institutions

23 hematopoietic stem cell transplants 57 solid organ transplants

Disseminated infection 2 noncontiguous organs or + blood culture 69% of HSCT with scedosporium 53% of SOT with scedosporium

Husein et. al. Infections due to Scedosporium in Transplant Recipients: Clinical Characteristics. Clinical Infectious Disease 2005 Jan 1;40

Scedosporium - overview Ubiquitous white mold

Tolerates aerobic and anaerobic conditions and wide range of temperature and osmolarity

Transmission Direct inoculation (mycetoma) Inhalation of airborne particles

Williamson et. al. Genetic Epidemiogy of Scedosporium in Patients with Chronic Lung Disease. J Clin Microbiol. 2001 January; 39(1): 47–50.

Species Scedosporium apiospermum

Typically sensitive to multi-agent antifungal therapy

Voriconazole associated with survival improvement over amphotericin

Scedosporium prolificans Treatment generally requires immunosuppression

reversal and surgical intervention.

Cortez et. Al. Infections Caused by Scedosporium spp. Clin Microbiol Rev. 2008 January; 21(1): 157–197.

American Society for Microbiology: Clinical Microbiology Reviews

Infection sites 2000-2007

Cortez et. Al. Infections Caused by Scedosporium spp. Clin Microbiol Rev. 2008 January; 21(1): 157–197.

Lungs 59%Sinuses 36%Bone/joint 8%Eyes 7%Hands 4%Feet 4%CNS 3%Blood 3%Abdomen 2%

Case Report A 72 year old woman presented to ER

3 months of progressive hemoptysis

Diffuse pulmonary nodules on recent imaging

5 days of fever, chills, and myalgias

Past Medical History Pulmonary arterial hypertension

Diagnosed 2 years prior Likely secondary to chronic pulmonary emboli On continuous infusion intravenous treprostinil On warfarin for chronic thromboemboli

Breast cancer Right mastectomy and radiation 8 years prior

No history of atypical or recurrent infections

Recent Medical History CT guided needle biopsy of pulmonary nodule had

been performed 3 weeks prior

Histology: necrotic tissue, peribronchial fibrosis and chronic inflammatory changes without granulomas

Gram stain/culture: no bacterial or fungal growth

Cytology: no malignant cells

Social History Independently performs activities of daily living

25 pack years but quit 2 years prior

No occupational or environmental exposures

Physical ExamT 36.7 BP 121/70 P 99 R 24 Pulse ox: 95% on room air

HEENT – UnremarkableChest – right sided indwelling Hickman catheterHeart – UnremarkableLungs Diminished breath sounds bilaterally, no rales, rhonchi, or wheezingAbdomen: UnremarkableExtremities/Skin: Unremarkable

Laboratory Data Fungitell: 257 (41 previously) [Normal < 40] Blood Culture: Scedosporium elements

WBC 12.9Hgb 12.3Plt 239Neut 80%INR 3.4

Histoplasma Ab HIV screenGalactomannan CMV, EBV pcrAspergillus Ab RVPANA Hep A,B,CScl70 MycoplasmaAnti-dsDNA Chlamydia

The following were negative

3 weeks prior

Hospital Course Sudden hemoptysis of 600 ml frank blood

Resolved with reversal of anticoagulation

Bronchoscopy with lavage performed Hemorrhagic fluid with negative cultures

Repeat CT guided biopsy of left lobe nodule Pathologic findings unchanged and unremarkable

Hickman catheter removed No fungal or bacterial growth on tip culture

Hospital Course Amphotericin and voriconazole initiated

Repeat CT 2 weeks later showed progression

Patient requested to discontinue all IV medications and go home with home health care Oral voriconazole and terbinafine Oral sildenafil New 2 L oxygen requirement

Speciation and Sensitivity Speciation: Scedosporium Apiospermum

Sensitivity testing: Amphotericin R Caspofungin R Micafungin S Voriconazole S Itraconazole S Posaconazole S

Resolution Patient’s hypoxia improved and she was able to

titrate off oxygen

No recurrence of fever or hemoptysis

She completed 6 months of antifungal therapy with voriconazole and terbinafine

Radiographic regression

4 months later

Case Summary Atypical presentation of a rare fungal pathogen

Diagnostic difficulties

Voriconazole as preferred agent

Questioning her risk factors

AcknowledgmentsDr. Leslie Spikes

Associate Professor of Internal Medicine University of Kansas Medical Center

European Society for Imunodeficiencies

Unusual infections or unusually severe course of infections

T lymphocyte deficiency WAS STAT1 deficiency Hypermorphic mutations in IκBαX–linked lymphoproliferative syndrome

DeVries et.al. Clinical & Experimental Immunology vol. 145, iss. 2.pages 204–214, August 2006