Progressive shortness of breath.

The patient is a 39 year old African American female who was diagnosed with pulmonary sarcoidosis 1.5 years prior to JHH admission after presenting with shortness of breath. At that time, radiologic studies showed parenchymal infiltrates and mediastinal adenopathy. The pathology of a lung wedge resection 1 year prior to admission was reviewed at JHH and showed granulomatous inflammation. Her FVC was 70% predicted and DLCO 57% predicted. She began oral corticosteroid therapy without significant symptomatic improvement. Six months prior to admission, the patient had continued dypsnea and a transthoracic ultrasound and right heart catheterization showed the presence of pulmonary hypertension with mean PA pressure 50-55 mmHg. She was started on an oral endothelin antagonist (Bosentan) which was discontinued after 5 months due no improvement in symptoms. The patient was admitted two separate times to an outside hospital 3 and 1 month prior to JHH admission for suspected pneumonia. At that time, during a 6 minute walk test, her SaO2 fell from 94% to 83% while inspiring 4 L/min nasal oxygen.
Two days prior to JHH admission, the patient was again admitted to the critical care unit of an outside hospital for worsening shortness of breath and increased oxygen need. By report, a chest x-ray showed a bilateral lung infiltrate with the left lung being worse than the right. She was started on cefotaxime and azithromicin for bacterial pneumonia and due to a persistent pulmonary infiltrate, the patient was transferred to JHH for further evaluation and management

Sarcoidosis
Pulmonary hypertension
Multinodular goiter
Hypercholestolemia

The patients mother is alive and has coronary artery disease and hypertension. Her father died at an early age of a myocardial infarction that was thought to be drug related. There is also a history of breast and gastric cancer in her family.

The patient lives at home with her husband, daughter, and son. She is a registered nurse. Prior to her decline in health, she had worked with mentally handicapped individuals. The patient has a remote smoking history of one year. She occasionally drinks alcohol.

Bactrim 1 single strength/day
Prednisone 20 mg/day
KCl
Lasix 20 mg/day
Nystatin
Oxygen 4 L/min

No known drug allergies.

The patient reports pink frothy sputum production, wheezing, and a 3 pillow orthopnea. The patient does not report chest pain.

• T: 36.2 C BP: 99/79 P: 94 RR: 33 SaO2: 95% on 4L NC
• General: NAD, obese African American female, awake and alert.
• HEENT: Normocephalic, atraumatic, extra-ocular movements intact, sclera anicteric, mucosal membrane moist. A white papular lesion was present on the glossal surface.
• CV: Regular rate and rhythm. Jugular venous distension to the mid-neck while reclining at 30o. Normal S1 with a wide S2 and prominent P2. There is a 2-3/6 systolic murmur at the midsternum that is not heard at the apex or the axilla.
• Lungs: No wheezes, crackles, or rhonci.
• Abdomen: Soft, nontender, nondistended, positive bowel sounds.
• Extremities: No clubbing, cyanosis, or edema, capillary refill <2s, 2+ pulses throughout.
• Neuro: Alert and oriented x 3, cranial nerves II-XII intact, sensation intact to fine touch, normal muscle bulk and tone, coordination grossly normal.

• Na 134; K 4.5; Cl 101; HCO3 18; BUN 20; Cr 1.0; Glucose 159; Calcium 7.6
• WBC 12.6;Hct 34.8%; Platelet 233k;

The patient was admitted and treated for a suspected sarcoid flare with increasing doses of prednisone. Piperacillin/tazobactam and azithromycin were given for possible pneumonia. Trimethoprim/sulfamethoxazole was also given for possible pneumocystis pneumonia. Sildenafil was started for the management of pulmonary hypertension. The patient was also given lasix, nebulizer treatments, and was continued on oxygen 4L NC. A chest CT 1 day after admission (Images 1-3) showed a patchy ground glass infiltrate involving the left lung, particularly in the upper lobe. The right lung was also involved, but to a lesser degree. Adenopathy was present in hilar, mediastinal, paratracheal, and supraclavicular nodes. The patient also continued to report shortness of breath. A portable chest x-ray (Image 4) showed a diffuse infiltrate of the left lung and no definitive infiltrate in the right lung. At around 11 am, the patient had rapid onset of weakness and decreased mental status. The patient lost consciousness and was found to be in PEA arrest. Resuscitation was not successful.

Images 1-3: Chest CT shows bilateral ground glass opacities, with patchy consolidative soft tissue density in left lung. Diffuse lymphadenopathy in mediastinum and neck.

Image 4: Plain film shows diffuse consolidative pattern in left lung plus mediastinal widening.

What is the differential diagnosis of granulomatous inflammation in the lung? What is the most likely cause of her pulmonary hypertension? What are the possible causes of the patients worsening shortness of breath over months and eventual demise?