Anterior Non-granulomatous Uveitis: Differential Diagnosis 10.1007/978-81-322-2506...Anterior Non-granulomatous Uveitis: ... Case 4: Uveitis Related to a Streptococcal Infection/

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  • Anterior Non-granulomatous Uveitis:Differential Diagnosis

    Marina Papadia, Naoual Jennane, and Carl P. Herbort

    ContentsIntroduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2Characteristics of Non-granulomatous Versus

    Granulomatous Uveitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2Differential Diagnosis of Non-granulomatous Anterior

    Uveitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6

    Case 1: HLA-B27 Uveitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8

    Case 2: Uveitis Associated with Juvenile IdiopathicArthritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9

    Case 3: Uveitis Mistakenly Described as Non-granulomatous with Very DeleteriousConsequences . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11

    Case 4: Uveitis Related to a Streptococcal Infection/Para-infectious Uveitis . . . . . . . . . . . . . . . . . . . . . . . . . . . 13

    Further Comments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14

    Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15

    Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15

    M. Papadia (*)Department of Ophthalmology, Ospedale Antero Micone,Genoa, Italy

    Retinal and Inflammatory Eye Diseases, Centre forOphthalmic Specialized Care (COS), Teaching CentreClinic Montchoisi, Lausanne, Switzerlande-mail: marinapapadia@yahoo.com

    N. JennaneRetinal and Inflammatory Eye Diseases, Centre forOphthalmic Specialized Care (COS), Teaching CentreClinic Montchoisi, Lausanne, Switzerland

    Private Practice, Rabat, Moroccoe-mail: jennane.naoual@yahoo.com

    C.P. HerbortRetinal and Inflammatory Eye Diseases, Centre forOphthalmic Specialized Care (COS), Teaching CentreClinic Montchoisi, Lausanne, Switzerland

    University of Lausanne, Lausanne, Switzerlande-mail: carl.herb@bluewin.ch; cph@herbortuveitis.ch

    # Springer India 2016V. Gupta et al. (eds.), The Uveitis Atlas,DOI 10.1007/978-81-322-2506-5_12-1

    1

    mailto:marinapapadia@yahoo.commailto:jennane.naoual@yahoo.commailto:carl.herb@bluewin.chmailto:cph@herbortuveitis.ch

  • Introduction

    The anatomical classification of uveitis into ante-rior, intermediate, and posterior forms is veryuseful to conduct the work-up and eventually toreach a diagnosis, even though inflammation doesnot always respect these anatomical boundaries.Anterior uveitis is the term used for the group ofinflammatory disorders for which the preponder-ant part of the inflammation is situated at the levelof the pars plicata of the ciliary body, the retro-iridal space, the iris, and the anterior chamber.

    The anatomical diagnosis of anterior uveitisshould first be verified by excluding spilloverinflammation associated with uveitis of the poste-rior segment (intermediate or posterior uveitis).To exclude posterior involvement, pupil dilatationis mandatory in all cases. Secondly, the type ofclinical presentation has to be characterized asnon-granulomatous or granulomatous in order tocorrectly orient work-up and differentialdiagnosis.

    Characteristics of Non-granulomatousVersus Granulomatous Uveitis

    Non-granulomatous uveitis is characterizedmainly by the type of keratic precipitates (KPs)that present as fine KPs producing endothelialdusting. In severe cases fibrinous clotting orhypopyon can occur depending on whether pro-tein influx or cellular infiltration is predominant(Figs. 1, 2, and 3). In case of severe inflammation,it is also common to find posterior synechiae, andpressure tends to be more often decreased thanincreased (Fig. 2).

    In contrast, granulomatous uveitis is character-ized by KPs that are larger than the dusty KPs ofnon-granulomatous uveitis. These KPs are betterindividualized but their size varies depending onthe inflammatory process. The medium- andlarge-size granulomatous KPs are called mutton-fat KPs. Other characteristic features of granulo-matous uveitis are Koeppe and Busacca nodulesmade of inflammatory cells at the pupillary mar-gin (Koeppe) or within the iris stroma (Busacca).

    Synechiae are common in more pronouncedinflammation. Pressure changes when present areusually characterized by increased intraocularpressure. In this context it is important to insiston the fact that Fuchs uveitis is a granulomatousuveitis unlike what is written in several textbooks.Fuchs KPs are structured, usually in a stellatefashion; they can be easily individualized andare larger than just dust (Fig. 4). The presence ofKoeppe nodules in a substantial number of casesis further confirming the granulomatous characterof Fuchs uveitis. The term of granulomatous

    Fig. 1 Fibrinous clot in the anterior chamber in typicalcase of HLA-B27-related uveitis

    Fig. 2 Posterior irido-lenticular synechiae. The ring ofpigment deposits on the crystalline lens shows where theiris was attached (synechiae). There is one remainingsynechia at 6 oclock on the verge of detaching itselffollowing the administration of massive dilating drops.The presence of pigment deposits on crystalline lens isuseful to confirm previous episodes of anterior uveitis

    2 M. Papadia et al.

  • uveitis is in fact a misnomer because a histopath-ologic term is used to describe clinical conditionsbased on certain clinical features including spe-cific KPs and iris nodules, among other clinicalsigns. Originally the clinical term of granulomatousuveitis was still based on the histopathologic pres-ence of granulomatous lesions which today is nomore always the case. It has become a clinicalcategory, a clinical terminology for which in somecases an underlying granulomatous histopathologycan be found such as in sarcoidosis and tuberculo-sis, but this clinical terminology has extended toother conditions where the underlying histopathol-ogy is not granulomatous such as in Fuchs uveitisor birdshot retinochoroiditis or not always granulo-matous such as in Vogt-Koyanagi-Harada disease.

    Although this clinical distinction betweengranulomatous and non-granulomatous is a veryuseful classification, the subdivision is not anabsolute one. A granulomatous uveitis may ini-tially present as non-granulomatous before takingits granulomatous aspect. On the other hand, when

    dusty KPs are very numerous and thick, they maybe mistaken as granulomatous (Table 1).

    Symptoms and Signs of Non-granulomatous Anterior UveitisThe severity of symptoms in anterior uveitisranges from no symptoms in chronic diseasesuch as anterior uveitis related to juvenile idio-pathic arthritis (JIA) to very severe symptoms inacute uveitis such as HLA-B27-related uveitis.Symptoms of acute anterior uveitis include pho-tophobia, redness, pain, decreased vision, andtearing in the absence of discharge.

    Fig. 3 Hypopyon. Sedimented white cells form a level atthe bottom of the anterior chamber, associated with a ringof fibrin on the surface of the crystalline lens (top) indicat-ing broken synechiae. One remaining synechia on themeridian of 7 oclock

    Table 1 Common causes of non-granulomatousuveitis

    HLA-B27-related uveitis and HLA-B27-associateddiseases

    Behets uveitis

    Uveitis associated with juvenile idiopathic arthritis

    Uveitis associated with scleritis

    Uveitis associated with streptococcal infection

    Uveitis associated with tubulointerstitial nephritis anduveitis (TINU)

    Rare entity psoriatic uveitis (Tanaka et al. 2015),relapsing polychondritis uveitis

    Fig. 4 Stellate KPs, typical of Fuchs uveitis

    Anterior Non-granulomatous Uveitis: Differential Diagnosis 3

  • The Signs of Anterior Non-granulomatousUveitis (Listed in Table 2)

    1. Conjunctival injection in anterior uveitis canbe diffuse or localized circumferentially at thelimbus (perikeratic injection) or mixed (diffuseand perikeratic injection).

    2. Keratic precipitates (KPs) are small and dif-fuse causing dusting of the endothelium. IfKPs are larger than dust and can easily beindividualized, even if they are small, theyshould be considered as granulomatous suchas in Fuchs uveitis presenting micro-granulomatous stellate KPs.

    3. Anterior chamber flare is caused by exudationof proteins into the normally clear aqueoushumor from iris vessels or across the ciliarybody epithelium following the breakdown ofthe blood-aqueous barrier. The intensity offlare is measured in a standard fashion follow-ing the grading system proposed by the ProctorGroup in San Francisco in 1959 (Table 3). Thisgrading system is however only qualitative.When the concentration of proteins in theaqueous is very high, they agglomerate andform fibrinous clots, a finding more commonin acute non-granulomatous uveitis (Fig. 1).

    4. Posterior synechiae: Depending on the amountand composition of aqueous inflammatory pro-teins, adherences between the iris and anteriorcapsule of the crystalline lens can form (poste-rior synechiae) (Figs. 13).

    Since a few years, it is now possible tomeasure flare in a quantitative and objectivefashion, using laser flare photometry (LFP)(Fig. 5). This new technology makes flare theonly quantitative parameter to measure intra-ocular inflammation. So far cells were esti-mated to be more accurate to measureinflammatory activity in uveitis. At best thismeasurement is however only semiquantita-tive. LFP was shown to be more sensitivethan slit-lamp assessment of cells to measurethe evolution of inflammatory activity, makingflare the new gold standard to assess intraocu-lar inflammation. LFP allows to detect subclin-ical flare intensities and changes that can bepredictive of clinical recurrence. A closerfollow-up of therapy is so possible, leading toincrease of treatment in case of r