Noninfectious Inflammatory Disorders PDF

Idiopathic Orbital Inflammatory Disease Benign, non-infectious and non-neoplastic clinical syndrome that displays a nonspecific inflammatory process No known local or systemic etiology, and often occurs between the third and fifth decades of life, without any gender or race p...

Idiopathic Orbital Inflammatory Disease Benign, non-infectious and non-neoplastic clinical syndrome that displays a nonspecific inflammatory process No known local or systemic etiology, and often occurs between the third and fifth decades of life, without any gender or race preferences Formerly termed orbital pseudotumor 16% of all cases of unilateral ptosis in adults IOIS occurs in 6% and 17% of the pediatric population; 45% of these cases present bilaterally The third most common orbital disease behind Graves’ disease and lymphoproliferative disorders (LPDs) ○ Think about bone marrow cancerous disorders May be associated w/ systemic vasculitis such as: ○ Granulomatosis w/ polyangiitis (Wegener’s granulomatosis) (small and middle vessel) vasculitis that affects the lungs, kidneys, and other organs Small and middle: Wegener’s High c. arteritis Giant-cell arteritis (GCA), also known as temporal arteritis, is a primary systemic autoimmune disease characterized by infiltration of moderate-size and large blood vessels is the cause of arteritic ischemic optic neuropathy Characterized by non-neoplastic, non-infectious, space-occupying, diffuse orbital inflammation Histology: pleomorphic cellular inflammatory infiltration followed by fibrosis Inflammatory process may involve any or all the orbital soft tissues resulting in myositis, dacryoadenitis, optic perineuritis, or scleritis Signs and symptoms ○ Painful (dolor), warmth (calor), red (rubor), swelling (tumor) ○ Diplopia, VA loss, and proptosis ○ Lack of fever** (no infectious process) ○ Normal WBC* ○ Ocular motility restrictions and cranial nerve palsies (III, IV, V1- ophthalmic division and in this area, causes efferent autonomic response and contains sensory deprivation and may have loss of sensation in this, VI) ○ Palpable orbital mass and lacrimal gland enlargement Done by retropulsion test ○ Might have increased IOP (because pressure on the globe), hyperopic shift (pressure behind the eye), ON swelling or atrophy, decreased sensitivity of CN V first division, conjunctival chemosis, and injection ○ CT shows ill-defined orbital opacification and loss of contents definition ○  ○ (CT axial: R eye, looking at feet towards the head) Less common signs and symptoms ○ Uveitis ○ Elevated IOP by compression of the globe ○ ON swelling/atrophy ○ Retinal edema, choroidal folds, hyperopic shift, posterior scleritis- causes inflammation to sclera and causes choroidal folds and retinal edema ○ Decrease or loss of vision Work up ○ Case history ○ Ophthalmological evaluation ○ Lab tests as needed ESR, CBC w/ differential ESR elevated: you have to suspect vasculitis************** ANA, BUN (because urea is what forms when muscle breaks down; autoimmune diseases such as SLP or Wegener’s may affect kidney function and have elevated BUN) Creatinine (R/O vasculitis) Extraocular muscles may have excessive muscle breakdown and create uric acid in excess ○ FBS (R/O mucormycosis- infectious) ○ Antineutrophil cytoplasmic antibody (ANCA) test (to detect autoimmune vasculitis) Specifically Wegener’s Treatment/Management (R/O everything and it is an idiopathic orbital disease) ○ Prednisone 80-100 mg PO QD daily and antiulcer medication (any H2 blocker; Famotidine (Pepcid) or even an PPI- (Omeprazole)) Then taper* because if not, you will get a rebound ○ Low-dose radiation if there is no response to steroids when the disease recurs as steroids are tapered or pose a significant risk If it recurs, we are missing something ○ F/U Re-evaluate in 3-5 weeks because dosage is about 2 weeks If responsive, maintain the initial dose for 1-2 weeks and then taper off slowly for several months- monitor the IOP closely Due to oral medications, not as much as a problem than topical but still monitor (incidence is not as prevalent) No response to steroids, order biopsy Idiopathic orbital myositis Rare inflammatory disorder of single or multiple extraocular eye muscles in the absence of thyroid eye disease, vascular abnormalities, or infiltrative lesion - variation of idiopathic; may involve one EOM or various ones - No identifiable local or systemic cause - A subtype of idiopathic orbital inflammatory disease (IOID) Chronic inflammatory cellular infiltrate in muscle fibers Symptoms: acute pain in early adults, that is exacerbated w/ EOM movements, diplopia Signs: lid edema, ptosis, chemosis, diplopia Vascular injection over the muscle: ○ Mild proptosis ○ Permanent restrictive myopathy- chronic cases Dx test: CT scan shows fusiform enlargement of the muscles, with or without tendon involvement DDX ○ Graves’ ophthalmopathy (aka TED; Thyroid Eye Disease): also can cause restrictive myopathy but painless on eye motility, associated w/ systemic manifestation (Grave’s disease) ○ Acute non-recurrent cases may resolve in 6 weeks w/o need of anything ○ Chronic is characterized by a single episode that persists for more than 2 months or recurrent attacks Treatment ○ NSAID: mild cases ○ Systemic steroids: cause dramatic improvement, can have recurrences ○ Radiotherapy: effective, particularly in limiting recurrences Tolosa-Hunt Syndrome (THS) - diagnosis of exclusion Nonspecific granulomatous inflammation within the cavernous sinus or superior orbital fissure, possibly extending into the orbital apex ○ Cavernous sinus: venous circulation; space created by veins that are incasing sella turcica and pituitary gland; internal carotid artery, CN 3, 4, V1/V2, 6 in here Causes acute, constant orbital pain, which characterizes the onset of this disorder Dx of exclusion, an uncommon condition In people older than 20 years = no gender preference The granulomatous inflammation causes ophthalmoparesis due to palsies of CN 3, 4, and 6  Pupillary dysfunction may be present, related to injury to the pupillary fibers of CN III Trigeminal nerve involvement (primarily V1) may cause paresthesia of the forehead Signs ○ CN 3, 4, V1/V2, 6 palsy- complete/incomplete CN 3 palsy w/ or w/o pupillary sparing ○ Ptosis may be observed related to CN 3 ○ Mild proptosis and/or optic disc edema may be noted if the orbit is involved (SOF/orbit) ○ Involvement of the sympathetic nerves may produce (POST-GANGLIONIC) Horner syndrome w/ miosis (anterior to CS) Central, preganglionic, postganglionic (3 trajectories) Hyoid bone- superior cervical ganglion from there towards the eye, it is postganglionic ○ CN 3 palsy and miosis (Horner syndrome) indicates inflammation could be at the cavernous sinus If eye is shut and you pry open the eye and the eye is down and out & pupil is miotic, [inferior] complete cranial nerve 3 palsy Symptoms ○ Painful ophthalmoplegia (hallmark), retrobulbar type ○ Double vision ○ Eyelid droop ○ Facial pain or numbness Treatment ○ Corticosteroids are the treatment of choice: usually, pain relief is obtained w/in 24-72 hours of therapy initiation Granulomatosis w/ Polyangiitis (Wegener Granulomatosis) Formerly known as Wegener granulomatosis Rare multisystem autoimmune disease of unknown etiology Hallmark features ○ Necrotizing granulomatous inflammation (patchy necrosis in arteries and veins) ○ Pauci-immune vasculitis in small- and medium-sized blood vessels Pauci: involving multiple Septic arthritis can cause monoarthritis 8 y/o complaining of pain in wrist and knee, juvenile arthritis in females (3-4 joints are always involved, Pauci arthritis involving 3-4) Involvement of the paranasal sinuses: most of the characteristic clinical features, followed by pulmonary and renal disease Test: c-ANCA- antineutrophil cytoplasmic antibody test Beware of patients w/ chronic sinusitis w/ recurrent exacerbations (initial complaint in 67% of cases w/ failure to respond to conventional tx) Other ENT manifestations ○ Rhinitis (22%)- characteristic of Wegener’s  ○ Epistaxis (11%) ○ Collapse of nasal support, resulting in saddle nose deformity (common) ○ Serous otitis media and hearing loss ○ So-called strawberry gingival hyperplasia Ocular signs ○ Proptosis ○ Eyelid edema ○ Ophthalmoplegia ○ Scleritis ○ Conjunctivitis ○ Uveitis ○ Episcleritis Systemic signs ○ Fevers, night sweats ○ Fatigue, lethargy ○ Loss of appetite ○ Weight loss ○ 85% of patients develop glomerulonephritis Affects the glomerulus Signs of orbital involvement ○ Proptosis, orbital congestion, ophthalmoplegia ○ Dacryoadenitis and nasolacrimal duct obstruction Can cause epiphora (tearing) and may involve the lacrimal gland Even dacryocystitis Diagnostic test ○ C-ANCA test (anti-neutrophilic cytoplasmic antibody test which is found in 90% of patients with active disease) Antibody produced by the body that acts against one of its own proteins Binding to antigen targets throughout the neutrophil cytoplasm MC protein target being proteinase 3 (PR3) A granular pattern of cytoplasmic stainings produced  Treatment ○ Systemic Cyclophosphamide (immunosuppressor- Cytoxan) and steroids Resistant cases: Azathioprine, Rituximab, plasma exchange Surgical: orbital decompression in severe cases of orbital involvement If decompression affected optic nerve