regions, as well as Louisiana and Mississippi.
resulting positive. He was also found to be positive for antinuclear antibodies and anti-SSA. He was thus started on intravenous corticosteroids. His mental status gradually but significantly improved, allowing him to be transferred out of the ICU. Discussion: While Neuroinvasive WNV is a rare complication, it is quite serious with a 10% mortality rate. Our case underlines not only how severe it can be, but two important points. First, what factors predisposed this patient to contract the rare complication? Second, further studies are needed to assess both the efficacy of corticosteroids and alternative therapies. At this moment, there is no definitive cure or treatment for the infection, and the standard of care remains supportive measures. Few case studies have shown the benefit of corticosteroid treatment, and many other reports detail the ineffectiveness of other potential therapies including ribavirin, interferon- alpha, and intravenous immunoglobulin.
Case: A 68-year-old man who presented with altered mental status and progressive motor weakness. Initial testing revealed a small subdural hematoma which was deemed nonsurgical by neurosurgery. The patient’s encephalopathy gradually worsened and he developed ascending motor weakness with acute hypoxic respiratory failure requiring transfer to the intensive care unit (ICU). Because magnetic resonance imaging and electroencephalogram were negative, neurology empirically started the patient on intravenous immunoglobulin therapy for presumptive Guillain- Barre Syndrome. However, the patient’s clinical status continued to deteriorate and further work- up was done, including heavy metal, autoimmune and infectious studies. Because cerebrospinal fluid (CSF) studies were inconclusive and a basic meningitis panel was negative, Lyme serology, fungitell, and West Nile Virus CSF polymerase chain reaction (PCR) tests were added, with WNV CSF PCR
ANTI-MDA5 DERMATOMYOSITIS: A WINDED RASH Anuj Thakre, Bruno Alvarez Concejo; Department of Medicine, Louisiana State University, Shreveport, LA.
Introduction: Anti-melanoma differentiation- associated gene 5 (anti-MDA5) dermatomyositis (DM) is a rare subtype of idiopathic inflammatory myositis, characterized by a phenotype of clinically amyotrophic DM (CADM), characteristic cutaneous features, and rapidly progressive- interstitial lung disease (RP-ILD). Case: A 49-year-old male with hypertension presented with chronic progressive rash, arthralgias, and joint stiffness associated with fatigue, 60 lbs. unintentional weight loss, and dysphagia to solids. Symptoms had started six months prior. The rash involved the face and chest, with marked peri-orbital swelling. Joints affected included shoulders, wrists, fingers, and knees bilaterally. He reported having been diagnosed with “oral thrush” recently, which was being treated medically. Physical examination showed alopecia, an erythematous facial rash with peri-orbital swelling, hyperpigmented rash in the anterior chest wall, and asymmetric swelling of proximal interphalangeal joints of hands, as well as scattered crackles in the pulmonary examination. Investigations showed normocytic anemia with a hemoglobin of 11.8 mg/dL, ferritin 1520 ng/ml, low
iron saturation. His chemistry demonstrated K 3.0, HCO3 38, Cr 0.85, AST 99, ALT 126, total bilirubin 0.8, ESR 45 mm/hr, CRP 0.77 mg/dl, and normal levels of CK and aldolase. He had positive ANA, ANA IgG 1:640, anti-Histone Ab, RNP Ab, anti-MDA and SMA, and normal C3 & C4. UA had +3 protein, 24-h Urine protein 1.4g. Other laboratories included negative anti-smith, anti-ds-DNA, anti-SSA, anti-SSB, anti-centromere, anti- SCL70, P-ANCA, and C-ANCA. CXR showed bibasilar infiltrates, and XR hands showed no evidence of erosive arthritis. Non-contrast chest CT with findings suggestive of Usual Interstitial Pneumonia (UIP). CT A/P w/wo contrast ruled-out other primary malignancies. Renal biopsy was negative for immune- complex deposition. Treatment was initiated with pulse-dose steroids, and he was discharged on oral steroids. In the outpatient setting, he was initiated on Mycophenolate Mofetil (MM) and Rituximab infusions with slowly tapered steroids. MM was later replaced by Tacrolimus due to gastrointestinal side effects. The patient had interval progression of ILD over the next few months but remained off supplemental oxygen. Discussion: Anti-MDA5 phenotype is known for its RP-ILD. Early recognition is imperative, as disease 35
Made with FlippingBook Digital Publishing Software