Shortness of breath, fevers, chills and rigors.

The patient is a 52-year-old woman with a history of systemic lupus erythematosis (SLE) and scleroderma overlap syndrome characterized by inflammatory polyarthritis, sclerodermatous skin changes, dilated nailfold capillaries, esophageal dysmotility, small intestine dysmotility requiring on home TPN for nutrition, inflammatory myopathy, Raynaud's phenomenon, glomerulonephritis, hypocomplementemia, and restrictive lung disease. She was recently admitted to JHH from 2/16/2009 to 2/27/2009 for a flare of her autoimmune disease with inflammatory myositis and myocarditis on heart biopsy that was treated with Prednisone 60 mg/day and mycophenolate mofetil.

Her autoimmune illness began in the summer of 2006 with inflammatory polyarthritis affecting her knees, shoulders, wrists, and finger joints. In the fall of 2006 into 2007, she developed proximal weakness and found to have elevated CPK and aldolase, an abnormal EMG, and histologic evaluation of muscle showing an inflammatory myositis. Serologically, she was found to have positive ANA and RNP, and low positive double-stranded DNA with positive Smith and positive Rho antibodies. In 02/08 she was admitted to an outside hospital with abdominal pain, nausea, and vomiting. She had evidence of esophageal dysmotility and small bowel obstruction and underwent exploratory laparotomy during a 7-day admission. Since that time she had been receiving parenteral nutrition and intravenous methylprednisolone (30 mg/day).

Last month, the patient was admitted to Johns Hopkins from 02/16/09 to 02/27/09 because of volume overload and newly diagnosed cardiac dysfunction. Over the past 2 months prior to admission she reported worsening functional status, dyspnea on exertion, and lower extremity edema. She underwent extensive cardiology evaluation with right and left-sided catheterizations. Notably, she did not have evidence of pulmonary arterial hypertension nor of coronary artery disease. Endomyocardial biopsy did reveal numerous infiltrative CD-3 positive cells with a marked increase in these inflammatory cells in areas of scarring thought to be consistent with myocarditis from scleroderma. She was discharged on mycophenolate and intravenous methylprednisolone (60 mg/day).

On 03/05/2009 she presented to an outside hospital with shortness of breath, fevers, chills and rigors. She reported having worsening fatigue since discharge from JHH and 2-3 days of cough productive of clear to yellow phlegm. In the outpatient setting she was noted to have a temperature of 101F. She was admitted to the hospital and placed on antibiotics. A chest radiograph showed bilateral infiltrates. Over the next 72 hours she developed hypotension requiring vasopressors, worsening respiratory failure, requiring increasing inspired oxygen concentration, and renal insufficiency. She was treated with broad spectrum antibiotics (vancomycin, piperacillin/tazobactam, po metronidazole, and caspofungin) and stress dose steroids (hydrocortisone 100mg/Q8h). In the setting of worsening multi-system organ failure (renal failure, respiratory failure, shock liver), she was transferred to JHH on 03/11/2009 for further management.

SLE/Scleroderma overlap syndrome
    Myocarditis, Cardiomyopathy, nephritis, Inflammatory myositis/polyarthritis
Pancreatitis
H/O esophageal dysmotility, esophageal candidiasis
Methotrexate associated hepatitis
Small bowel obstruction s/p exploratory laparotomy- requiring chronic TPN
S/P c-section x 3

Notable for father who died at age 35 from an MVA; her mother is 73. One sister and three brothers all have hypertension. She has 3 children who are well. There is no family history of autoimmune disease.

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

No known drug allergies.

Gradual decline with increased breathlessness and weakness; a new cough which was productive of yellow and white sputum; fever.

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.

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

Normal sinus rhythm, left axis deviation, possible lateral infarct, age undetermined nonspecific T wave changes

See Images 1-6

The patient was admitted to the MICU and was subsequently intubated on vasopressors (dopamine). She was on renal replacement therapy. A diagnostic bronchoscopy was performed on 3/13/09 for bilateral ground glass opacities noted on CT chest. Ulcerations with vesicular appearance were noted throughout the main airways. Purulent secretions were found in the right lower lobe bronchus. Culture of bronchoalveolar lavage was performed, as were assays for viral pathogens. Her MICU course was notable for worsening multiorgan system failure with evidence of both septic and cardiogenic shock. Despite broad spectrum antibiotics, her clinical status declined with escalating vasopressor requirements. Ultimately, on 3/18/09 her family made the decision to make her comfort care. The patient expired on 3/18/09 at 9:45 am.

1.What are the risk factors for pneumonia in this patient?
2.What is the most likely etiology of the pneumonia?