Ординатура / Офтальмология / Английские материалы / Practical Manual of Intraocular Inflammation_Dick, Okada, Forrester_2008
.pdf50 |
Chapter 2 |
2.Nussenblatt RB, Palestine AG, Chan CC, et al. Standardization of vitreal inflammatory activity in intermediate and posterior uveitis. Ophthalmology 1985; 92(4):467–471.
3.Chylack LT Jr., Wolfe JK, Singer DM, et al. The Lens opacities classification system III. The Longitudinal Study of Cataract Study Group. Arch Ophthalmol 1993; 111(6):831–836.
4.Jabs DA, Rosenbaum JT. Guidelines for the use of immunosuppressive drugs in patients with ocular inflammatory disorders: recommendations of an expert panel. Am J Ophthalmol 2001; 131(5):679.
5.Cassoux N, Giron A, Bodaghi B, et al. IL-10 measurement in aqueous humor for screening patients with suspicion of primary intraocular lymphoma. Invest Ophthalmol Vis Sci 2007; 48(7):3253–3259.
6.Cimino L, Auer C, Herbort CP. Sensitivity of indocyanine green angiography for the follow-up of active inflammatory choriocapillaropathies. Ocul Immunol Inflamm 2000; 8(4):275–283.
7.Antcliff RJ, Stanford MR, Chauhan DS, et al. Comparison between optical
coherence tomography and fundus fluorescein angiography for the detection of cystoid macular edema in patients with uveitis. Ophthalmology 2000; 107(3): 593–599.
8.Francis PJ, Marinescu A, Fitzke FW, et al. Acute zonal occult outer retinopathy: toward a set of diagnostic criteria. Br J Ophthalmol 2005; 89(1):70–73.
9.Holder GE, Robson AG, Pavesio C, et al. Electrophysiological characterisation and monitoring in the management of birdshot chorioretinopathy. Br J Ophthalmol 2005; 89(6):709–718.
3
Practical Evaluation of the Patient in the Clinic
When one is faced with a patient having posterior segment intraocular inflammation (PSII) for the first time, the process of generating a list of differential diagnoses depends greatly on the predominant ocular signs or features. Although most diseases present with a degree of many features of inflammation, such as vitritis, retinal vasculitis, and/or choroiditis, there are often one or two features that predominate over the others. For example, retinal vasculitis may be the predominating feature in an eye that also has some vitritis and some papillitis. Retinal vasculitis as the predominant feature would lead us to consider the more likely diagnoses of Behc¸et’s disease, sarcoidosis, and tuberculosis rather than other diseases. In this manner, distilling all the ocular signs into only one or two predominant clinical features can greatly aid in narrowing down the differential diagnosis. Of course, the differential diagnosis should also be tailored to other clinical characteristics of the patient’s history and ocular examination, in particular, whether or not anterior segment inflammation is present.
Chapter 2 has already defined and offered hints on how to describe the major posterior uveitic features of vitritis, retinal vasculitis, and chorioretinitis. While this establishes the diagnosis of uveitis and its classification, and indeed recognizes sight-threatening lesions (further discussed in chap. 4), this chapter gives a concrete framework for the reader to generate differential diagnoses based on these and other predominant ocular features. Again, it should be emphasized that all ocular features should be graded and reported according to the Standardization of Uveitis Nomenclature (SUN) guidelines (1). The decision to begin treatment is not based on specific diagnosis but on deciding infective (where then a specific diagnosis of infective agent is imperative) or noninfective etiology, and whether immediately sight threatening or not. The descriptions within this chapter will help achieve this.
51
52 |
Chapter 3 |
Nevertheless, making a diagnosis in noninfectious cases is important for determining long-term outcome and appropriate choices, where necessary, of immunosuppressive therapy.
DIFFERENTIAL DIAGNOSIS BASED ON PREDOMINANT OCULAR FEATURE
The following is an outline of the most likely candidates for specific disease entities based on the predominant posterior segment feature. As you might expect, there is overlap of clinical signs among many of the conditions since the ocular tissues have a limited set of responses to injury, but the key to diagnosis often lies in the pattern of signs as they develop both in time and space. The lists are subdivided based on additional characteristics, such as whether significant (1þ or greater) vitritis and/or anterior segment inflammation accompanies the predominant feature (Fig. 1). Infectious diseases are listed first followed by noninfectious
Figure 1 Predominant clinical features in posterior segment intraocular inflammation.
Practical Evaluation of the Patient in the Clinic |
53 |
disorders and, where appropriate, the neoplastic disease intraocular lymphoma. Diseases that can present with many different predominant features appear in several sections, which only emphasizes the spectrum of phenotype each diagnosis may include and why treatment decisions cannot be based on making a diagnosis alone. Finally, a section on panuveitis in which the predominant feature is the presence or development of both posterior and anterior segment inflammation is also included. Rare diseases or ill-defined entities and rare presentations of common diseases are not included. Nonspecific features of chronic inflammation, such as cystoid macular edema and epiretinal membrane, are also left out of these differential diagnosis lists because they can occur in virtually any type of intraocular inflammation. Finally, the lists pertain mainly to clinical settings in developed countries worldwide. Diseases of the developing world, most notably tropical infections, as well as rare diseases are included in the second half of this chapter describing the workup for specific disease entities.
Vitritis
Some degree of vitritis is a feature of many diseases with PSII. But if vitritis is the predominant feature, the disorder is often classified as intermediate uveitis, and certain conditions and diseases, listed below, may present with it. The purpose of this chapter is to emphasize that while classifying the patient anatomically (e.g., intermediate vs. posterior vs. panuveitis), characteristics of the vitritis may assist in diagnosis of specific conditions, particularly in differentiating infective versus noninfective causes. For example, in general, acute or episodic vitritis would hint toward bacterial endophthalmitis (in a painful red eye), toxoplasmosis (with a chorioretinal scar present) (Fig. 2), or noninfectious PSII such as Behc¸et’s
Figure 2 What can underlie a vitritis? (A) Characteristic Toxoplasma chorioretinal lesion with vitritis. (B) Numerous snowball-like opacities in the inferior vitreous in a patient with sarcoidosis.
54 |
Chapter 3 |
disease (history of painful oral ulcers, and skin or genital lesions), whereas a chronic or indolent vitritis would suggest diseases such as sarcoidosis (often no systemic symptoms), malignant lymphoma (patient more than 50 years of age), and fungal endophthalmitis (recent hospitalization or surgery). Furthermore, chronic vitritis with snowball-like clumps inferiorly is characteristic of sarcoidosis (Fig. 2).
On the other hand, chronic vitritis just behind an intraocular lens with posterior capsular opacification is suspicious of delayed-onset postoperative endophthalmitis (particularly, Propionibacterium acnes infection after cataract surgery). The more typically ascribed intermediate uveitis is considered in a separate category below, since this classically and accurately represents a distinct clinical picture of chronic vitritis in the presence of exudates or snowbanking over the pars plana and/or peripheral retina, often accompanied by peripheral retinal vasculitis (see chap. 1). Looking in more detail at the anatomical distribution of inflammation, we can describe: “anterior vitritis” implying prominent vitreous cells centered behind the lens/iris diaphragm, observed best with the plain slit lamp, and “intermediate uveitis,” “focal posterior vitritis” and “diffuse vitritis” best appreciated by contact lens biomicroscopy using the slit lamp or indirect ophthalmoscopy (Box 1).
Papillitis
As with vitritis, papillitis may be observed to some degree in any inflamed eye. However, it is the predominant feature in just a few diseases of PSII as listed below (Box 2), depending on accompanying ocular signs. An enlarged disc with prominent cellular infiltration may also represent a disc granuloma, suggestive of sarcoidosis (Fig. 3) or tuberculosis. Of course, papillitis or inflammation of the optic nerve head must always be differentiated from disorders of vascular ischemia (anterior ischemic optic neuropathy, giant cell arteritis, impending central retinal vein occlusion, diabetes mellitus), of acute demyelination (idiopathic optic neuritis), or of a genetic nature (Leber’s hereditary optic neuropathy). These occur in eyes with no other evidence of intraocular inflammation in patients with characteristic medical or family history and are not included here. Finally, papillitis must be distinguished from papilledema, which occurs in disorders associated with increased intracranial pressure and would prompt an entirely different line of workup. Fluorescein angiography can be helpful here.
Retinal Vasculitis
Inflammation may predominantly involve retinal arteries or retinal veins. The classic teaching is that inflammation of retinal arteries is characteristic of diseases such as systemic lupus erythematosus and syphilis, while inflammation of retinal
Practical Evaluation of the Patient in the Clinic |
55 |
Box 1 Conditions Causing a Predominant Vitritis
Anterior vitritis
l |
Delayed-onset postoperative endophthalmitis |
l |
Toxocariasis |
Intermediate uveitis with prominent vitritis |
|
l |
Human T lymphocyte virus type 1 uveitis |
l |
Lyme disease |
l |
Syphilis |
l |
Toxocariasis |
l |
Sarcoidosis |
l |
Multiple sclerosis |
l |
Pars planitis |
l |
Intraocular lymphoma |
Focal posterior vitritis |
|
l |
Fungal endophthalmitis |
l |
Toxoplasmosis |
l |
Toxocariasis |
Diffuse vitritis |
|
l |
Human T lymphocyte virus type 1 uveitis |
l |
Bacterial endophthalmitis |
l |
Lyme disease |
l |
Fungal endophthalmitis |
l |
Toxoplasmosis |
l |
Toxocariasis |
l |
Sarcoidosis |
l |
Behc¸et’s disease |
l |
Immune recovery uveitis |
l |
Intraocular lymphoma |
veins is characteristic of diseases such as tuberculosis, sarcoidosis, and Behc¸et’s disease. However, in practice, this distinction is of limited use since the division is far from foolproof, and most eyes show evidence of inflammation in both types of vessels. Signs suggestive of active retinal vasculitis (Fig. 4) include vascular
56 |
Chapter 3 |
Box 2 Conditions Causing a Predominant Papillitis
With retinal exudates
l |
Bartonella infection (cat scratch disease, neuroretinitis) |
With retinal vasculitis
l |
Toxoplasmosis |
l |
Multiple sclerosis |
With choroiditis, vitritis and/or anterior segment inflammation
l |
Tuberculosis |
l |
Syphilis |
l |
Vogt-Koyanagi-Harada disease |
l |
Sarcoidosis |
Figure 3 Fundus color photograph of swollen optic nerve head secondary to sarcoid granuloma.
tortuosity, dilation of retinal veins, and narrowing of retinal arteries. Active retinal vasculitis may be accompanied by varying degrees of retinal hemorrhage, perivascular cellular infiltrates, perivascular exudates, vitritis, posterior hyaloid traction over vessels, and/or cotton wool spots. The presence of cotton wool spots suggests retinal ischemia, and areas of retinal nonperfusion may be observed on
Practical Evaluation of the Patient in the Clinic |
57 |
Figure 4 Fundus color photographs of features of retinal vasculitis. (A) Retinal hemorrhages (*) associated with retinal periphlebitis in a patient with tuberculosis. (B) Small occlusive vasculitis and cotton wool spot (arrow). (C) Prominent vascular sheathing (arrow) is seen in this patient with SLE being treated with immunosuppression. (D) Patient with retinal vasculitis shows the phenomenon of “cuffing” around vessels (arrow), representing active perivascular inflammation. (C and D reproduced with permission Bunkodo Publishing Company, Tokyo)
fluorescein angiography. Long-standing vascular ischemia may lead to neovascularization at the disc or in the retina. Well-demarcated sheathing of retinal vessels or ghost vessels (total occlusion) is indicative of chronic inflammation or inactive disease. Sheathing, which is irregular or patchy, and, importantly, produces a hazy, less well-defined “cuffing” around a segment of the vessel denotes active vasculitis.
Frosted branch angiitis is a particular form of retinal vasculitis associated with cytomegalovirus (CMV) and other infections. Although retinal vasculitis is a clinical feature observed in many disorders of intraocular inflammation, the list of diseases in which retinal vasculitis is the predominant feature is relatively short (Box 3).
Retinal Infiltrates
Cellular retinal infiltrates (retinitis) are the predominant feature in a surprisingly small number of disease entities, the overwhelming majority of which are
58 |
Chapter 3 |
Box 3 Conditions Causing a Predominant Retinal Vasculitis
With significant vitritis and/or anterior segment inflammation
l |
Tuberculosis |
l |
Behc¸et’s disease |
l |
Sarcoidosis |
Without significant vitritis and/or anterior segment inflammation
l |
Whipple’s disease |
l |
Systemic lupus erythematosus |
l |
Multiple sclerosis |
l |
Frosted branch angiitis |
Box 4 Conditions Causing a Predominant Retinal Infiltrate
With significant vitritis and/or anterior segment inflammation
l |
Acute retinal necrosis |
l |
Bacterial endophthalmitis |
l |
Fungal endophthalmitis |
l |
Toxoplasmosis |
l |
Behc¸et’s disease |
Without significant vitritis and/or anterior segment inflammation
l |
Cytomegalovirus retinitis |
l |
Progressive outer retinal necrosis |
infections (Box 4). With the exception of fungal endophthalmitis and CMV retinitis, all of the diseases have a rather acute onset, and except for CMV retinitis and progressive outer retinal necrosis (PORN), all are usually accompanied by a significant degree of vitritis and/or anterior segment inflammation. All of the infections are progressive in nature until specific antimicrobial treatment is initiated. The one noninfectious entity on the list, Behc¸et’s disease, occurs in acute bursts of inflammation (Fig. 5) that are self-limiting but recurrent.
Practical Evaluation of the Patient in the Clinic |
59 |
Figure 5 Fundus photograph of a typical posterior attack in a patient with Behc¸et’s disease, showing several retinal infiltrates, disc hyperemia and edema, and engorgement of the vascular arcades. (With permission, Bunkodo Publishing Company, Tokyo)
Choroiditis (Choroidal White Dots and Other White Lesions)
White lesions in the choroid may appear in numerous intraocular inflammatory diseases, but are the hallmark of the diseases listed below (Box 5). Diseases commonly referred to as white dot syndromes (Fig. 6) include multifocal evanescent white dot syndrome (MEWDS), acute posterior multifocal placoid pigment epitheliopathy (APMPPE), and punctate inner choroidopathy (PIC). However, many textbooks include other diseases such as Vogt-Koyanagi-Harada (VKH) disease, and there is no definitive etiological, clinical, or anatomical reason for classifying one disease as “white dot disease” versus another disease as “multifocal choroiditis.” Therefore, the following list ignores the designation of “white dot syndrome” and is divided in a more clinically relevant manner of whether significant vitritis and/or anterior segment inflammation accompanies the choroidal findings. Some diseases such as tuberculosis can present either with or without significant vitritis and/or anterior segment inflammation, and therefore appear in both sections of the list. Acute lesions tend to have indistinct borders, while old atrophic lesions have distinct borders often with pigmentation. Lesions that grow in size and coalesce may suggest acute retinal necrosis (ARN) due to herpes simplex virus (HSV) or varicella-zoster virus (VZV) infection (Fig. 7).
Many diseases that present with vitritis and/or anterior segment inflammation in the acute phase will only have atrophic choroidal lesions in the quiescent phase in absence of vitreous or anterior chamber cells. Therefore, one must