A 42-year-old female with a past medical history of rheumatoid arthritis diagnosed three months prior to admission, managed with methotrexate with poor adherence, was admitted due to psychomotor agitation without focal neurological deficits and fever (100.4 F) with neutrophilia.
Lumbar puncture showed a low glucose level in the cerebrospinal fluid (CSF) (glucose CSF 24.8 mg/dl, glucose serum: 78 mg/dl), 23 common germs in FilmArray (multiplex PCR system), as well as Gram and Chinese ink negative.
The patient lived in a tuberculosis endemic zone. Antibiotic therapy, antituberculosis drugs, and antifungal agents were initiated despite negative results.
The patient showed a sudden improvement in consciousness three days after starting treatment. However, she presented a new episode of altered mental status two days later, and it was associated with transaminases elevation 100 times above the reference value. Liver enzymes remained elevated despite discontinuation of antituberculosis agents. Brain CT scan of the brain showed ischemic areas in the corpus callosum and bilateral parietal lobes.
One day later, our patient presented severe hemodynamic decompensation with metabolic acidosis, acute respiratory failure, and pancytopenia. The patient was transferred to the MICU to provide mechanical ventilation and vasopressors.
Simultaneously, a progressive drop in hemoglobin was identified, so a bronchial tract lavage (not alveolar) was performed obtaining bloody secretions. Chest X-ray showed diffuse bilateral infiltrates. Patient's critical condition disabled him to undergo chest CT scan. Autoimmune markers were requested due to the suspicion of immune alveolar hemorrhage showing anti-cardiolipin antibodies IgM 0.3 MPL-U/ml (negative: <7 MPL-U/ml), IgG 100 MPL-U/ml (POSITIVE: >17 GPL-U/ml), Anti-DNA 29 IU/ml (positive: >20 IU/ml), and both positive anti-nuclear antibodies and lupus anticoagulant. Schistocytes were identified in peripheral blood smear. There was no evidence of lupus nephritis.
The patient met the criteria for definite CAPS associated with alveolar hemorrhage. Intravenous pulses of methylprednisolone 1 g daily IV and enoxaparin 60 mg daily SubQ to maintain isocoagulation state due to risk of hemorrhagic conversion of ischemic strokes were added to the already placed ventilatory support. The patient's critical condition prevented her from underwent bronchoscopy or pulmonary biopsy.
Despite the initial clinical improvement, the patient died in later days due to a new deterioration of respiratory function, renal failure, and encephalopathy.