A 20-year-old Caucasian man with no prior medical history was admitted to the emergency department for hoarseness, stridor, and pyrexia. The patient did not take any specific medications. Symptoms developed over a period of 2-3 months before admission and were associated with exhaustion, gonalgia, and depression. Three months before the current situation, the patient had undergone severe psychological stress. Physical examination revealed numerous submandibular polyadenopathy, attenuated heart sounds, and bilateral pulmonary crackles. Chest X-ray revealed cardiomegaly, and cardiac ultrasonography confirmed the presence of a 400 ml circumferential pericardial effusion and a thickened mitral valve but no hemodynamic changes. Initial laboratory findings showed increased C-reactive protein level at 4.5 mg/dl, normocytic anemia with hemoglobin of 9.2 g/dl, low serum albumin at 1.9 mg/dl, serum urea at 55 mg/dl, and serum creatinine at 1.0 mg/dl. The 24-hour urine collection showed increased proteinuria of 11 g. Autoimmune evaluation tests revealed increased antinuclear autoantibodies at a titre of 1 : 5000 and the presence of high titres of anti-dsDNA as well as the anti-Sm autoantibodies. The complement levels (C3 and C4) were diminished, and the Coombs test was positive. Anti-EBV and anti-CMV IgG were identified in absence of specific IgM. The patient was HIV seronegative. A renal biopsy was performed, and histological analysis revealed focal extramembranous proliferative glomerulonephritis. Cervical computed tomography and laryngoscopy showed left vocal cord paresis as well as hypomobility of the right vocal cord (Figure 1). The major involvement of both vocal cords required urgent endotracheal intubation followed by tracheotomy. Chest CT-scan showed bilateral pleural effusions and confluent opacities within the left pulmonary lobe. Recurrent nerve compression was reasonably excluded in the absence of mediastinal adenopathies. Fine-needle aspiration (FNA) was performed in a left submandibular adenopathy and revealed cytological features suggestive of follicular and paracortical lymph node hyperplasia without any signs of malignancy or granuloma. Cytologic examination of one of the samples obtained from bronchoalveolar lavage revealed numerous acid-fast bacilli. Because of high suspicion of active tuberculosis (TB), anti-TB chemotherapy (Isoniazid, rifampicin, pyrazinamide, and ethambutol) was started in association with glucocorticosteroids (pulse corticoids 1 g/day for 3 days). The patient's condition improved within a few days with the disappearance of vocal cord palsy. However, additional investigations (auramine staining and cultures of BAL and pleural fluids) as well as sustained cultures of the FNA of the adenopathy sample remained negative for mycobacterium, making the diagnosis of TB unlikely. The above clinical vignette encompassing serositis, glomerulonephritis, increased titres of antinuclear antibodies, the presence of antidsDNA, anti-Sm autoantibodies, and autoimmune anemia is typical of SLE. In the light of this atypical presentation, the vocal cord dysfunction was attributed to laryngeal involvement due to SLE.
As recurrent nerve palsy was reasonably ruled out, the diagnosis of cricoarytenoiditis was retained to explain the vocal cord dysfunction. Accordingly, immunosuppressive therapy including glucocorticoids (methylprednisolone 64 mg/d) and mycophenolate mofetil (2 g/d) was started.