title: The diffuse infiltrative lymphocytosis syndrome (DILS). A comprehensive review.
abstract: The Diffuse Infiltrative Lymphocytosis Syndrome (DILS) is a rare multisystemic syndrome described in HIV-infected patients. It is characterised by CD8(+) T-cell lymphocytosis associated with a CD8(+) T-cell infiltration of multiple organs. DILS is usually seen in uncontrolled or untreated HIV infection but can also manifest itself independently of CD4(+) T-cell counts. The syndrome may present as a Sjögren-like disease that generally associates sicca signs with bilateral parotiditis, lymphadenopathy, and extraglandular organ involvement. The latter may affect the lungs, nervous system, liver, kidneys, and digestive tract. Anomalies of the respiratory system are often identified as lymphocytic interstitial pneumonia. Facial nerve palsy, aseptic meningitis or polyneuropathy are among the more frequent neurological features. Hepatic lymphocytic infiltration, lymphocytic interstitial nephropathy and digestive tract lymphocytic infiltration account for more rarely noted complications. Sicca syndrome, organomegaly and/or organ dysfunction associated with polyclonal CD8(+) T-cell organ-infiltration are greatly suggestive of DILS in people living with HIV. Labial salivary gland biopsy is therefore helpful when the focus score is equal or greater than 1 (or Chisholm Score ≥ 3). Primary Sjögren syndrome, chronic HCV or HTLV1 infection, graft versus host disease, IgG4-related disease, and immune reconstitution inflammatory syndrome are among the differential diagnoses that need to be considered. Treatment consists in highly active anti-retroviral therapy (HAART), which is usually effective in resolving clinical signs and symptoms. Steroids, however, may also be occasionally required when organ infiltration does not respond to HAART. This review should provide an insight into this rare entity complicating the course of HIV infection.
authors: DHU i2B, Service de Médecine Interne, Hôpital Saint-Antoine, AP-HP, 184 Rue du Faubourg Saint-Antoine, 75012 Paris, France. Electronic address: etienne.ghrenassia@gmail.com.
authors: Etienne
authors: E
authors: Ghrenassia
authors: Service de Médecine Interne, Hôpital l'Archet, Centre Hospitalier Universitaire de Nice, 151 Route Saint-Antoine de Ginestière, 06200 Nice, France. Electronic address: martis.n@chu-nice.fr.
authors: Nihal
authors: N
authors: Martis
authors: Service d'Anatomo-Pathologie, Hôpital l'Archet, Centre Hospitalier Universitaire de Nice, 151 Route Saint-Antoine de Ginestière, 06200 Nice, France. Electronic address: boyer.j@chu-nice.fr.
authors: Julien
authors: J
authors: Boyer
authors: Service d'Anatomo-Pathologie, Hôpital Pasteur, Centre Hospitalier Universitaire de Nice, 30 Avenue de la Voie Romaine, 06000 Nice, France. Electronic address: burel-vandenbos.f@chu-nice.fr.
authors: Fanny
authors: F
authors: Burel-Vandenbos
authors: DHU i2B, Service de Médecine Interne, Hôpital Saint-Antoine, AP-HP, 184 Rue du Faubourg Saint-Antoine, 75012 Paris, France. Electronic address: arsene.mekinian@sat.aphp.fr.
authors: Arsène
authors: A
authors: Mekinian
authors: Service d'Hématologie Clinique, Hôpital Saint-Antoine, AP-HP, 184 Rue du Faubourg Saint-Antoine, 75012 Paris, France. Electronic address: paul.coppo@sat.aphp.fr.
authors: Paul
authors: P
authors: Coppo
mesh_terms: D000818:Animals; D023241:Antiretroviral Therapy, Highly Active; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology* / Q000821:virology; D002465:Cell Movement; D003937:Diagnosis, Differential; D006678:HIV / Q000276:immunology*; D015658:HIV Infections / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D008206:Lymphatic Diseases / Q000175:diagnosis* / Q000188:drug therapy; D008218:Lymphocytosis / Q000175:diagnosis* / Q000188:drug therapy; D010309:Parotitis / Q000175:diagnosis* / Q000188:drug therapy; D012859:Sjogren's Syndrome / Q000175:diagnosis*; D013256:Steroids / Q000627:therapeutic use; D013577:Syndrome
chemical_list: D013256:Steroids
keywords: AIDS; CD8(+) infiltration; Diffuse infiltrative lymphocytosis syndrome; HAART; HIV; Sicca signs