This case describes a patient presenting with acute onset papilledema, subacute strokes resulting in upper extremity weakness and numbness, arthritis, maculopapular rash, depressed C4 and CH50, and a high titer anti-double-stranded DNA antibody. The patient was given the diagnosis of probable systemic lupus erythematosus, which was supported by the Systemic Lupus International Collaborating Clinics (SLICC) criteria. He was aggressively treated for neuropsychiatric lupus (NPSLE) with pulse dose steroids and a dose of intravenous cyclophosphamide. Blood cultures drawn on admission later grew out 2/4 bottles of Gram-variable bacteria, speciated as
Brucellosis, secondary to
A 50-year-old male with no past medical history presented to the hospital with one week of painless blurry vision of the right eye. He had also been having intermittent fevers, headache, body aches, and a nonpruritic maculopapular rash on the bilateral lower extremities for 6 months. On further review of systems, the patient noted one isolated episode of left knee swelling as well as testicular swelling in the past. The patient otherwise denied any neck stiffness, nausea, vomiting, Raynaud’s phenomenon, oral ulcerations, chest pain, shortness of breath, abdominal pain, or photosensitivity. He worked as a flooring installer, and he did not have any toxic habits such as smoking, drinking, or illicit drug use.
The patient’s vital signs were normal. On physical exam, the patient was found to have bilateral papilledema and optic nerve erythema, right greater than left, right inferior nasal quadrant visual field defect, and a right afferent pupillary defect. Muscle strength was 5/5 throughout, and reflexes were 2+ throughout. Sensation to light touch, pinprick, vibration, and proprioception was intact. The bilateral lower extremities demonstrated a maculopapular rash (Figure
The admitting labs were notable for a microcytic anemia (Hb 11.6 gm/dL (ref 13.6–17.3); Hct 35.3% (ref 39.8–50.7); MCV 76.9 fL (ref 80.3–98.1)), hyponatremia (133 mmol/L (ref 136–144)), elevated ESR (33 mm/hr (ref 0–15)), and elevated CRP (13.3 mg/L (ref 0.0–7.0)). Urinalysis did not show protein or blood. Lumbar puncture was colorless/clear with 2/cumm RBC (ref 0), 56/cumm WBC (ref 0–9), 39% segmented neutrophils (ref 0–2), 53% lymphocytes (ref 40–80%), 30 glucose (ref 40–70), 69 protein (ref 15–45), with presence of oligoclonal bands, an elevated IgG index (+19.8 mg/24 hr (ref −9.9 to +3.3)), and normal opening pressure (16 cm H20 (ref 10–25 cm H20)). The initial CT scan of brain and orbits demonstrated no acute intracranial process, and the MRI of the orbits was also unremarkable.
Given the patient’s history of fever, myalgia, rash, and joint pain with CSF studies showing both a neutrophilic and lymphocytic pleocytosis, there was concern for infectious etiologies, including both bacterial and viral infections, as well as autoimmune etiologies. The differential diagnoses included neuromyelitis optica, multiple sclerosis, neuropsychiatric SLE, HIV, syphilis, tuberculosis, coccidioidomycosis, cryptococcus, Lyme, and West Nile virus.
Further investigation was pursued to work up the aforementioned etiologies, and the patient was found to have a positive double-stranded DNA (>1 : 640), low C4 (10 mg/dL (ref 16–47)), low CH50 (13 U/mL (ref 31–60)), normal C3, negative ANA by immunofluorescence assay (repeated twice) and negative anti-Sm/RNP, anti-SSA/B, Coombs antibody, anti-beta2 glycoprotein, anticardiolipin, and lupus anticoagulant. ANCA, ACE, and cryoglobulin were negative. Rheumatoid factor was positive (38 IU/mL (ref < 14)). The infectious disease service was consulted, and the infectious workup including HIV, hepatitis antibodies, cocci antibodies, RPR, cryptococcus antibodies, Lyme antibodies, West Nile virus antibodies, and Quantiferon Gold were all negative. CSF cultures showed no growth. Skin biopsy of the lower extremity rash was done, pending results.
The presence of a high-titer positive double-stranded DNA antibody raised concern for an autoimmune etiology, although in the setting of a negative ANA the validity of the dsDNA titer was questioned with a high concern for a false-positive test. Given the lack of other findings to suggest autoimmune disease, the rheumatology service requested additional studies. Given the negative infectious workup to date, the neurology service recommended initiation of pulse corticosteroids with methylprednisolone 1,000 mg intravenous daily for which the patient received 2 doses, with improvement in his symptoms, which was then followed by oral prednisone taper.
Approximately one week later, the patient returned to the hospital with acute onset right arm weakness and numbness. On physical exam, the patient was found to have 4/5 muscle strength in the right upper extremity with decreased sensation to light touch over the fourth and fifth digits of the right hand. MRI of the brain showed multiple subacute infarcts in the left parietal lobe (Figure
After the patient was discharged, the blood cultures that had been initially reported as no growth, were later found to have 2/4 bottles positive for Gram-variable bacteria. The cultures were sent to Public Health for confirmation, and the organism was speciated as
On follow-up, the patient’s serum
Brucellosis is a multisystem disease with a wide variety of clinical manifestations making the diagnosis in nonendemic areas very challenging. Acute brucellosis manifestations are often nonspecific and can resemble other neurologic and rheumatologic diseases as demonstrated in the case above. Neurobrucellosis is a rare but serious complication of brucellosis infection, with an incidence that ranges between 0.5 and 25% [
The diagnosis of neurobrucellosis can be difficult given the diverse neurologic characteristics and lack of specific radiographic or serologic findings [
Neurobrucellosis manifesting as vasculitis, as seen in our patient, is an unusual but well-described manifestation of brucellosis [
Our patient’s main complaint was visual and neurological symptoms, but the patient also gave an additional history of maculopapular rash on the lower extremities, intermittent fevers, headaches, body aches, an episode of left knee, and testicular swelling. All of these additional symptoms are well-described symptoms of acute brucellosis infection. In Burzgan et al.’s study of 1,028 patients with brucellosis, the most frequently seen symptoms in acute brucellosis were arthralgias (73.7%), myalgia (37.6%), headache (18.8%), and fever (76.9%). Scrotal swelling had an incidence of 3.8% and skin lesions 2.4% [
The presence of the high-titer positive dsDNA antibody described in the above case contributed to a much higher suspicion for SLE than would have otherwise been attributed to the case, thus highlighting the significance of the positive anti-dsDNA antibodies in delaying the correct diagnosis and treatment. Brucellosis-induced autoantibody production, has been described, as brucellosis has been shown to be a triggering factor for immunologic reaction [
While autoantibody production has been described in brucellosis patients, the possibility of lab error was also considered. Measurement of the dsDNA antibodies was done using the
In conclusion, we present a diagnostically challenging case of an infection mimicking an autoimmune disease, thus highlighting the importance of ruling out infectious diseases when evaluating a patient with features of systemic vasculitis. This case also emphasizes the risk of associated production of autoantibodies in the setting of infection, as well as the risk of lab error and false-positive test results. While epidemiologic advances and cultural habits have resulted in low rates of brucellosis in the United States, the large movement of people and goods between the United States and endemic areas makes it increasingly important for healthcare providers to recognize brucellosis’ diverse clinical presentations capable of mimicking other diseases.
The case clinical data used to support the findings of this study are included within the article.
The authors declare that there are no conflicts of interest regarding the publication of this paper.