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Clinico-Pathological Conference #2 October 6, 2009 The Patient • 52 yr old female with SLE – scleroderma overlap – Inflammatory polyarthritis – Sclerodermatous skin changes with Raynauds – Extensive GI dysmotility- TPN – Glomerulonephritis – Restrictive lung disease – Hypocomplementemia – Inflammatory myositis / myocarditis SLE-scleroderma • 2006– Polyarthritis – Weakness with myositis • 2/2008 – Esophageal dysmotility • TPN + IV methylprednisolone (30 mg/day) HPI • 1 month PTA- volume overload, cardiac dysfunction – R / L cardiac cath: no pulmonary arterial hypertension or coronary artery disease – Endomyocardial biopsy: inflammatory cells, scaring: mycophenolate, methylpred (60mg/dy) Medications Lasix 20 mg daily Hydrochlorquine 200 mg twice daily Lisinopril 5 mg daily Metoprolol 25 mg twice daily Prednisolone 60 mg intravenously in the morning Reglan 10 mg four times daily Myfortic 180 mg twice daily Dilaudid 2mg as needed Dapsone 100 mg every Monday-Wednesday-Friday Flagyl 500 mg every 8 hours for bacterial overgrowth Protonix 40 mg twice daily Ambien 10 mg as needed Ergocalciferol 1000 International Units daily Course • 3 weeks later– OSH: SOB, fever, chills, rigors, cough – Bilateral infiltrates – Rapid decline with sepsis, multi-organ failure despite • Vancomycin, pip/tazo, oral flagyl, caspofungin • T: 38.5, HR: 108, BP:92/62 on vasopressors, SaO2: 91% on FiO2=0.5 General: intubated, sedated, acutely ill, appears somnolent but is arousable. HEENT: clear oropharynx, no thrush, no ulceration. Lungs: coarse breath sounds throughout. CV: rapid and regular heart sounds. Abdomen: Soft, infrequent bowel sounds. Extremities: marked violaceous discoloration of fingers and toes, diffusely and bilaterally; no acute digital infarcts. Skin: Thickening in the trunk and extremities, sclerodactyly. Neuro: moves all four extremities on command, Intact bilateral dorsiflexion and plantar flexion. • Laboratory Values on Transfer Na 127, K 3.7, Cl 87, bicarbonate 23, BUN 74, creatinine 2.6. Calcium 8.5, protein 5.2, albumin 2.8 AST 2859, ALT 1416, alkaline phosphatase 1154, total bilirubin 5.3, ammonia 57, CPK 179.0, troponin 1.96, CK-MB 2%, lactic acid 2.7 LDH 3610 WBC 23,110, hemoglobin 7.8, hematocrit 26.2, platelet 221,000, polymorphonuclear cells 91% PT 16.1, INR 1.6, aPTT 31.6 Urinalysis: 2 WBC/PHF, 65 RBCs/PHF Cultures- Blood cultures negative, Sputum culture positive for yeast, urine culture negative, CSF cultures negative Legionella DFA-negative, Urine Strep antigen-negative, Serum galactomannan- 0.5 Course • Bronchosopy: – Ulcerations with vesicular appearance throughout the main airways – Purulence RLL bronchus • Dies 7 days later despite “broad spectrum antibiotic” therapy, aggressive supportive care – 13 days after presentation to hospital – 4 weeks after initiation of current symptoms Summary Moderately immunocompromised patient with ‘gradual’ respiratory decline, fever (4 week) bilateral pneumonia with acute sepsis – multi-organ dysfunction Ulcerative lesions in airway Chronic severe paralytic ileus Important Issues Contributing to Differential • Degree of immunocompromise – Chronic steroids + pulse, MMF • Duration of illness – “sub-acute” progression of pneumonia with sepsis • Appearance of infiltrates – Nodules, ground glass, focal lobar infiltrates • • • • Time of year: Feb – March Previous smoker Chronic GI dysmotility: aspiration Where is she from? Other risks for infection? Questions posed • What are the risk factors for pneumonia in this patient? – Corticosteroid exposure (+MMF?) • Other prior therapies (TNF-a inhibitors, rituxan?) – Hypocomplementemia – Underlying airway disease – smoking (ulcerations?) – Underlying structural lung disease – disruption of alveoli (alveolar hemorrhage / pneumonitis) – GI dysmotility – aspiration – Time of year / antecedent respiratory virus ? • Myocarditis / myositis 1 month PTA Differential Non-infectious • Osler (1904) suggested that pulmonary involvement as part of SLE – Alveolar hemorrhage / damage – Lupus pneumonitis / progressive interstitial lung disease – Progressive pulmonary hypertension – Pulmonary embolism – Malignancy Differential Infectious (syndrome) • Community-acquired pneumonia • Progressive hospital-acquired pneumonia • Aspiration pneumonia • Multiple / sequential infections – Respiratory virus followed by bacterial pneumonia • Influenza – S. pneumonia / S. aureus • Respiratory virus – fungal pneumonia • Disseminated viral infection – Influenza – Herpes virus (HSV, VZV) – Adenovirus Infectious Differential • Bacterial – – – – – – – – – – – • Viral common S. pneumoniae H. influenzae common S. aureus Rapidly progressive, necrotizing Group A / B Streptococcus • Mixed anaerobes (aspiration) Enterobacteriaceae Increased colonization Other organ involvement? Liver / myocarditis? – Disseminated Herpes virus – “Respiratory virus” (RSV, influenza, para-influenza, adenovirus, HMPV, coxsackievirus) Fungal – Aspergillosis (airway to invasive) – Cryptococcosis • E. coli (ESBL?) Airway lesions + GM Prior hospitalization • K. pneumonia (ESBL?) – Misc. filamentous fungus Suboptimal therapy Endemicity (?) Underlying disease (e.g. Zygomycetes) P. aeruginosa (MDR) common – Endemic fungus (Histoplasma, Legionella spp. Coccidiomycosis, Blastomyces) Multiple other atypicals: Tm / slf - dapsone? – Pneumocystis M. pneumoniae, Chlamydia Nocardia spp. Tm / slf - dapsone? • Parasitic Tm / slf - dapsone?– Toxoplasma gondii Mycobacteria – Strongyloides stercoralis Deductive reasoning • Moderately immunosuppressed, sub-acute pneumonia + gram stain / AFB stain negative, with ulcerative airway lesions, + galactomannan – Assumptions: • Other organ dysfunction (liver, heart) really as described • Omission of sputum / BAL Afb and gram stain not purposeful (high burden of disease for stain-negative bacterial process) • “Yeast” in sputum = Candida, not from endemic region Tracheobronchial + invasive aspergillosis Rapidity of death / sepsis: likely a secondary bacterial pneumonia