Keywords

Overview

  • Definition

    • Immune-mediated inflammation involving the kidney and the eye

    • Uveitis is usually bilateral, non-granulomatous anterior uveitis, but posterior segment involvement has been reported

  • Symptoms

    • Pain

    • Photophobia

    • Blurry vision

  • Laterality

    • Bilateral

  • Course

    • Nephritis is self-limited and rarely recurs, but uveitis can become recurrent in 40%

    • In 65% of cases, acute nephritis precedes uveitis by weeks to months; 20% uveitis precedes nephritis; 15% nephritis and uveitis occur concurrently

  • Age of onset

    • Children and adolescent, with median age of 15 years

  • Gender/race

    • F:M = 3:1

    • No racial predilection

  • Systemic association

    • Tubulointerstitial nephritis: injury to renal tubules and interstitium (not involving the glomeruli), leading to decreased renal function

    • Several HLA haplotypes have been associated with TINU, but HLA-DRB1*0102 represents the strongest association

Exam: Ocular

Anterior segment

  • Non-granulomatous AC inflammation, ranging from mild to severe

Posterior Segment

  • Uncommon: papillitis, cystoid macular edema, retinal pigment epithelial detachments, retinal vascular sheathing, vitritis, neuroretinitis, multifocal choroiditis

Exam: Systemic

  • Abdominal or flank pain

  • Fatigue

  • Fever

  • Headache

  • Anorexia and weight loss

  • BP usually normal

Imaging

  • OCT

    • CME (rare)

  • FA

    • Optic nerve leakage or vascular staining (rare)

Laboratory and Radiographic Testing

  • Renal biopsy (for definitive diagnosis): tubulointerstitial nephritis

  • Urinalysis: elevated beta-2 microglobulin, mild proteinuria, eosinophilia, pyuria or hematuria without infection, normoglycemic glycosuria, white cell casts

  • Bloodwork: elevated serum creatinine or decreased creatinine clearance, anemia, abnormal LFTs, elevated ESR

Differential Diagnosis

Inflammatory diseases that can affect the kidney and the eye:

  • Systemic lupus erythematosus

  • Sarcoidosis

  • Granulomatosis and polyangiitis

  • Adamantiades-Behcet’s disease

  • Sjögren’s syndrome

  • IgA nephropathy (Berger’s disease)

  • Post-streptococcal uveitis

  • Syphilis

  • Leptospirosis

  • Brucellosis

  • Tuberculosis

  • Drug-induced TINU: NSAIDs, Chinese herb Goreisan, acetaminophen, codeine phosphate, lamotrigine, smoking synthetic cannabinoid

Treatment

  • Nephritis responds favorably to systemic corticosteroids

  • Uveitis responds well to topical or regional steroids, but in recurrent cases systemic IMT is employed, including methotrexate, azathioprine, and cyclosporine

Referral/Co-management

  • Nephrology