Urine biomarker score captures response to induction therapy with lupus nephritis.

BACKGROUND: The Renal Activity Index for Lupus (RAIL) consists of urine protein assessment of neutrophil gelatinase-associated lipocalin, kidney injury molecule-1, monocyte chemotactic protein 1, adiponectin, hemopexin, and ceruloplasmin, which non-invasively identifies lupus nephritis (LN). We aimed to delineate RAIL scores with inactive versus active LN and changes over time with response to LN induction therapy. METHODS: There were 128 pediatric patients with systemic lupus erythematosus (SLE) and age-matched healthy controls recruited in a prospective case control study, with kidney biopsy confirmation of LN. Laboratory and clinical information was recorded and urine collected at diagnosis and end of induction and during maintenance therapy. Response to therapy was assessed by repeat kidney biopsy or laboratory parameters. Urine was assayed for RAIL biomarkers and the RAIL score calculated. RESULTS: Pediatric RAIL (pRAIL) scores from 128 children and young adults with SLE (with/without LN: 70/38) including 25 during LN induction therapy, differentiated clinically active LN from inactive LN or without LN, and controls (all p < 0.0017). pRAIL scores significantly decreased with complete LN remission by 1.07 ± 1.7 (p = 0.03). CONCLUSIONS: The RAIL biomarkers differentiate LN patients based on activity of kidney disease, with decreases of ≥ 1 in pRAIL scores indicating complete response to induction therapy. Significantly lower RAIL scores in healthy controls and in SLE patients without known LN raise the possibility of subclinical kidney disease. A higher resolution version of the Graphical abstract is available as Supplementary information.

Rare Diagnostic and Clinical Manifestations in an Acute Hepatitis A Infection: A Case Report.

The hepatitis A virus (HAV) is a common cause of infectious hepatitis worldwide. In adults, clinical manifestations typically involve fever, nausea/vomiting, fatigue, abdominal pain, and jaundice, although rarer manifestations may be observed. Acute hepatitis A infection is detected via anti-HAV IgM antibodies, which are present in almost all patients at symptom onset. In this case, we present a patient who not only tested negative for acute HAV infection at symptom onset, but also presented with uncommon, extrahepatic manifestations including maculopapular skin rash and polyarthralgia. Wariness of such a presentation can facilitate the timely diagnosis of atypical cases of HAV infection. We report the case of a 51-year-old man who presented with fever, abdominal pain, headaches, and diarrhea for one week with elevated liver enzymes and leukocytosis. Workup consisting of viral hepatitis panels, various infectious studies, and rheumatologic antibody titers did not initially reveal an etiology for the patient's presentation. Computed tomography (CT) abdomen and pelvis, abdominal ultrasound, magnetic resonance cholangiopancreatography (MRCP), and hepatobiliary iminodiacetic acid (HIDA) scan did not reveal acute pathology. The patient's symptoms worsened over the following days, and he additionally developed bilateral wrist pain, digital arthralgias, paraspinal back pain, diffuse muscular weakness, and a pruritic maculopapular rash affecting the flanks and extremities. Eventually, viral hepatitis studies were repeated which revealed elevated levels of anti-HAV IgM antibodies, indicating acute hepatitis A infection. The patient was treated supportively while hospitalized with subsequent improvement of symptoms and lab abnormalities. Since discharge, the patient had not experienced persistent sequelae of the disease. This case of acute viral hepatitis A infection is notable for two reasons: (1) the patient experienced uncommon, delayed, extrahepatic manifestations of disease, and (2) the initial viral hepatitis studies revealed undetectable anti-HAV IgM levels despite having experienced symptoms of illness for several days. This case suggests that repeat viral hepatitis testing may be warranted in patients who continue to experience manifestations of the infection after initially testing negative. It also emphasizes the importance of recognizing potential atypical manifestations of acute hepatitis A infection.

Tertiary Syphilis Masquerading as Oropharyngeal Cancer.

Syphilis is re-emerging in the United States. Treponema pallidum, the spirochete bacterium responsible for syphilis, has immunoevasive properties that facilitate pathogenesis and widespread tissue involvement. Host immune status, particularly the presence of HIV/AIDS, can influence the presentation and severity of the disease. Patients co-infected with HIV and syphilis may develop atypical lesions, including those involving the oropharynx. Any immunocompromised patient with tongue lesions and lymphadenopathy is presumed to have a wide differential diagnosis, and tissue sampling with histopathologic analysis is indicated. We present a patient with gumma of the tongue as the initial manifestation of tertiary syphilis.