A 20-year-old girl, born in Pakistan and living in Italy for 8 years, presented to the emergency room for fatigue, fever, and dry cough, lasting for 1 week. The medical history of the patient revealed coeliac disease. She was never-smoker. On physical examination, the patient was cachectic without any other pathological findings, in particular, no superficial lymphoadenopathies were appreciable. Blood examination showed leukocytosis (15,240/mmc), microcytic anaemia (hemoglobin [Hb] 10.4 g/dL), thrombocytosis (593,000/mmc), increased levels of C-reactive protein and erythrocyte sedimentation rate. Chest X-ray [Figure 1] displayed micronodular interstitial disease, with diffuse interstitial-alveolar infiltrates. In the suspect of miliary tuberculosis (TB), the patient was admitted to the Division of Infectious Diseases and it was started immediately anti-TB therapy: Rifampin, isoniazid, ethambutol, and pyrazinamide, firstly intravenous and then per os. Blood exams were repeated and showed normal leukocytes count with a slight monocytosis (12%) and confirmed microcytic iron deficiency anemia (Hb: 9.3 g/dL, ferritin: 7 ng/mL, iron: 25 mcg/dL) and thrombocytosis (485,000/mmc). Moreover, other laboratory tests confirmed cachexia (low levels of triglycerides, cholesterol, albumin, and creatine kinase).
Despite the prompt beginning of antitubercular therapy, the clinical condition worsened day by day. Persistent fever was present and after 5 days since admission, respiratory failure developed requiring oxygen-therapy. Blood culture for Gram-positive and negative bacteria resulted negative.
To clarify the involvement of the lung, it was performed a bronchoscopy with bronchial washing. The evaluation at the microscope on this specimen resulted negative for mycobacterium. Furthermore also QuantiFERON-TB resulted negative. Chest CT [Figure 2] displayed lung hilar and mediastinal lymphadenopathies (up to 3 cm) associated to diffuse parenchymal opacities (ground glass) and small nodules. The radiological features were not typical for miliar TB, but could be an expression of other diseases, such as lymphoproliferative disorders.
Fifteen days after the admission, patient's clinical condition worsened further with respiratory failure, hypotension, and tachycardia, and she was moved to the Intensive Care Unit. Despite the continuous positive airway pressure (CPAP) with inspiratory oxygen fraction 80% applied, PaO2 was lower than 50 mmHg. Clinical and echocardiographic parameters required to make a diagnosis of ARDS were satisfied. Intravenous therapies with methylprednisolone 20 mg twice a day and levofloxacin 500 mg twice a day were started. Bronchoscopy was repeated one more time performing bronchoalveolar lavage (BAL) and transbronchial biopsies (TBBs).
After the onset of the therapy, respiratory and cardiovascular parameters improved abruptly. CPAP treatment was discontinued the day after, dyspnea got better progressively. On serological examinations human immunodeficiency virus-test and lymphocyte typing were negative. Instead angiotensin conversion enzyme and lactic dehydrogenase were slightly increased. These results and the clinical response to steroid therapy suggested sarcoidosis.
This diagnosis was confirmed by BAL fluid examination which revealed lymphocytic alveolitis with CD4/CD8 ratio 1.2 and TBB was positive for nonnecrotizing granulomas consistent with sarcoidosis [Figure 3]. Antitubercular therapy and levofloxacin were stopped, and methylprednisolone was continued.
After 6 days, the patient was discharged from the Intensive Care Unit in good clinical condition and without oxygen support. A chest X-ray of control showed a reduction of the lung infiltrates bilaterally.
The patient was finally discharged with a diagnosis of ARDS in pulmonary sarcoidosis (Stage II) and the prescription of prednisone 25 mg a day.
The patient was followed in our interstitial lung diseases surgery. Chest X-ray and CT scan, performed 6 and 16 months after the discharge respectively, demonstrated almost complete regression of lymphadenopathies and pulmonary abnormalities [Figure 4]. During this period also the symptoms progressively improved, and steroid therapy (prednisone) could be tapered to a dose of 5 mg once a day.
Computed tomography images demonstrate subcarinal adenopathy (asterisk) and diffuse ground-glass opacities with small nodules (arrows) which tend to be confluent in some areas of the lung (box).