Presentation
She presented to us with pronounced eye manifestation which on further inquiry and investigation was found to be associated with rheumatoid arthritis. There was perforation of left globe on presentation and the right one was preserved. [ncbi.nlm.nih.gov]
(Present on Admission - Yes) Total Medicare Hospitalizations - Jan 2013 to Dec 2017 (Present on Admission - Yes) Total National Projected Hospitalizations - Annualized (Present on Admission - Not Y) Total Medicare Hospitalizations - Jan 2013 to Dec 2017 [dexur.com]
Necrotizing scleritis without inflammation, also known as scleromalacia perforans, is a very rare form of scleritis presenting with no symptoms. [zgthpbcc.gq]
Entire Body System
- Asymptomatic
Treatment and prognosis The condition is usually asymptomatic. Rupture of the globe is rare and usually only seen in those with coexisting markedly elevated intraocular pressure, i.e. severe glaucoma 1. [radiopaedia.org]
Also known as necrotizing scleritis without inflammation, scleromalacia perforans is generally asymptomatic (without symptoms), but may cause painless eye irritation and redness. [verywellhealth.com]
Key Facts • Obliterative arteritis involving deep episcleral plexus • Very rare, almost always associated with rheumatoid arthritis • Typically a painless, asymptomatic process • More common in elderly women Clinical ... Marian S. [educalingo.com]
Even more alarming is the fact that necrotizing scleritis can at times present with voracious inflammation and be obvious and symptomatic, but at other times can be asymptomatic without obvious inflammation, with progression unknown to the patient until [mersi.com]
Blue sclera • asymptomatic condition • marked, generalised blue discolouration of sclera due to thinning. osteogenesis imperfecta. [slideshare.net]
Jaw & Teeth
- Oral Ulcers
Oral ulcers and xerostomia (dry mouth) were also reported by her. Joints involved were mostly the small joints of the hands and feet with morning stiffness of about 30 to 45 minutes. [journal.medizzy.com]
She also complained of dry mouth, oral ulcers, and there was a history of stillbirth in eighth month of gestational amenorrhea 1 year back. [ncbi.nlm.nih.gov]
- Xerostomia
Oral ulcers and xerostomia (dry mouth) were also reported by her. Joints involved were mostly the small joints of the hands and feet with morning stiffness of about 30 to 45 minutes. [journal.medizzy.com]
Cardiovascular
- Hypertension
Anterior Chamber Brain and Optical Pathway Choroid Congenital Syndromes, Malformations and Abnomalities Conjunctiva Cornea Glaucomas, Ocular Hypertension Hypertensive Retinopathy Iris and Ciliary Body Lacrimal System Lens Lids Neuro-Ophthalmology Optic [atlasophthalmology.net]
She developed steroid induced diabetes and hypertension in September 1993, and she was treated with oral hypoglycemic and anti hypertensive agents. Cyclophosphamide and prednisolone were tapered slowly and stopped in June 1999. [ncbi.nlm.nih.gov]
Statistical attributes of the steroid hypertensive response in the clinically normal eye. I. The demonstration of three levels of response. Invest Ophthalmol Vis Sci. 1965 Apr;4:187-97. [reviewofoptometry.com]
She had been treated for hypertension and coronary artery disease for seven years and osteoarthritis for 20 years. Her best-corrected visual acuity was 6/36 in the right eye and 6/15 in the left eye. [ijo.in]
Case Reports Case 1 A 65-year-old woman with hypertension, chronic renal disease on peritoneal hemodialysis, pulmonary fibrosis, postsurgery for pterygium in both eyes (OU) and in topical treatment for chronic glaucoma was referred to our service with [thepajo.org]
Eyes
- Scleral Disease
Scleromalacia perforans [DOID:14230] A scleral disease that is characterized by a painless severe necrotizing scleritis where the sclera is white, avascular, and thin. [diseases.jensenlab.org]
In 1952 Anderson and Margolis3reviewed the literature and reported that there were approximately 30 cases of scleral disease, either similar or related in varying degrees to this condition. [acpjournals.org]
She had however later several remissions of episcleritis and on admission to the eye department in 1953 she had developed a devastating scleral disease in the left eye. *) Received February 18th 1956. Acta Ophthalmol. [documents.tips]
Watson Medicine British medical journal 1978 Although scleral disease is uncommon, it is associated with connective-tissue disorders and one series showed that 27% of patients who develop necrotising scleritis are dead from systemic complications within [semanticscholar.org]
1 reference stated in Disease Ontology release 2019-03-01 retrieved 5 March 2019 Disease Ontology ID DOID:14230 subclass of scleral disease 1 reference stated in Disease Ontology release 2019-03-01 retrieved 5 March 2019 Disease Ontology ID DOID:14230 [wikidata.org]
- Photophobia
A tint is incorporated in the spectacle lenses to alleviate photophobia. Oral NSAIDs and topical steroids are used for bringing the ocular inflammation under control and analgesics are essential to relieve pain. [visionmagazineonline.co.za]
Ocular symptoms may include dry or red eyes, foreign-body sensation, pruritus, photophobia, pain, visual changes, and even complete loss of vision. [aafp.org]
Encyclopedia) Fluorescein angiography (Medical Encyclopedia) Fluorescein eye stain (Medical Encyclopedia) Heterochromia (Medical Encyclopedia) Ophthalmoscopy (Medical Encyclopedia) Orbit CT scan (Medical Encyclopedia) Orbital pseudotumor (Medical Encyclopedia) Photophobia [icdlist.com]
An 18-year-old man presented with painful red eye, a history of photophobia, and passing highly colored urine since childhood. Dermatological and biochemical evaluations were done. [journals.lww.com]
Also, photophobia began in her right eye 2 months back. Although she had been taking treatment from local clinics and was kept on antibiotics during this period no improvement was seen. [journal.medizzy.com]
- Prolapse
Three complications were noted: endophthalmitis, graft necrosis, and graft dehiscence with uveal prolapse. However, no regrafts were needed. [nature.com]
Small holes in the TDM that do not cause iris prolapse can be ignored, whereas larger holes or tears with iris prolapse must lead to halting the NPGS and convert it to a trabeculectomy. [medtextfree.wordpress.com]
Figure 6: Anteroposterior and lateral view: Above, prolapsed uvea. Center, dissection around the defect. Below, fascia lata restoring the ocular anatomy. (A) ciliary body and choroid. (B) Thick sclera. [thepajo.org]
On separating the lids with eye speculum, a large perforation involving the superior thinned limbal cornea with prolapse of vitreous and uveal tissue were noted. Vision in the left eye was only perception of light. [ncbi.nlm.nih.gov]
- Visual Impairment
Giant pigment epithelial tear and retinal detachment has been reported in a patient with scleritis. [19] Visual impairment is a possible complication. Cornea is affected more than 50% of time. [emedicine.medscape.com]
Musculoskeletal
- Arthritis
Rheumatoid arthritis--case report]. Gheorghe A, Pandelescu M, Muraru C Infliximab treatment in a case of rheumatoid scleromalacia perforans. [medicbind.com]
Scleromalacia perforans is a rare ocular manifestation of rheumatoid arthritis which can potentially lead to blindness and is a late consequence in the course of the disease. [ncbi.nlm.nih.gov]
Br J Ophthalmol 1976 ; 60 : 163 – 91. 2 Rare extra-articular manifestation of rheumatoid arthritis: scleromalacia perforans. Kaohsiung J Med Sci 2005 ; 21 : 233 – 5. 3 Extra-articular manifestations and complications of rheumatoid arthritis. [academic.oup.com]
It is rare in the sense that we usually see scleromalacia perforans with fixed deformities of rheumatoid arthritis in the hands or progressed systemic complications but not as a starting landmark of disease. [go.gale.com]
1 Scleromalacia perforans: a case report. ( 29866188 ) 2018 2 Scleromalacia perforans in rheumatoid arthritis. ( 30597078 ) Yangzes S...Ram J 2018 3 Scleromalacia Perforans from Long-Standing Bulbar Conjunctival Rhinosporidiosis. ( 27342330 ) Bothra N [malacards.org]
- Morning Stiffness
Joints involved were mostly the small joints of the hands and feet with morning stiffness of about 30 to 45 minutes. The examination of her joints confirmed her history when acute synovitis was noted in both hands and feet. [journal.medizzy.com]
She also complained of pain in all small joints of her hands associated with morning stiffness of 30–45 minutes. She had severe pain around 7/10 on VAPS associated with joint swelling. On examination she was vitally stable and anemic. [ncbi.nlm.nih.gov]
Workup
Diagnosis, Workup and Treatment Further investigation into the patient’s ocular history found that he had undergone the I-Brite eye-whitening procedure four years prior to presentation. [reviewofophthalmology.com]
Laboratory testing As scleritis may occur in association with many systemic diseases, laboratory workup may be extensive. [eyewiki.aao.org]
GPA is often overlooked due to its rare incidence—just three cases per 100,000 people. 3 Approximately 10% of patients with GPA develop scleritis, so ANCA testing should be included in the workup of these patients. 4 In addition to GPA, rheumatoid arthritis [reviewofoptometry.com]
Treatment
Reddy SC, Tajunisah I, Rohana T Successful treatment of severe nodular scleritis with adalimumab. Restrepo JP, Molina MP [Scleromalacia perforans. Rheumatoid arthritis--case report]. [medicbind.com]
Systemic immunosuppressive treatment was started, and glaucoma treatment was regulated. A corneal patch and multilayer amniotic membrane were placed, which necrotized after 2 months. [thepajo.org]
[…] from Long-Standing Bulbar Conjunctival Rhinosporidiosis. ( 27342330 ) Bothra N....Tripathy D. 2016 4 Bilateral scleromalacia perforans and peripheral corneal thinning in Wegener's granulomatosis. ( 22553696 ) Reddy S.C....Rohana T. 2011 5 Infliximab treatment [malacards.org]
Treatment of Scleromalacia Perforans The usual treatment of Scleromalacia Perforans includes the use of both the topical and systemic medications. [webeyeclinic.com]
Prognosis
Treatment and prognosis The condition is usually asymptomatic. Rupture of the globe is rare and usually only seen in those with coexisting markedly elevated intraocular pressure, i.e. severe glaucoma 1. [radiopaedia.org]
Prognosis As with the treatment of scleromalacia perforans, the prognosis of the disease can vary enormously, informed by everything from a person's age and general health to the multitude of risk factors that influence astigmatism, cataracts, glaucoma [verywellhealth.com]
Medical and surgical management are novel treatments with a good prognosis for the JRA. In this case, we treated the patient with medical management only, and the patient's prognosis was good. [cureus.com]
Prognosis and Outcomes Scleromalacia Perforans has a good outcome provided the condition is diagnosed at an early stage and treatment started before any complication arises. [webeyeclinic.com]
Prognosis The prognosis varies depending on the disease classification. It may range from a self-limited episode of inflammation to a severe necrotizing process with vision-threatening complications. [emedicine.medscape.com]
Etiology
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]
The etiology is obscure.>An area in the sclera first undergoes a simple necrosis. This area is surrended by epitheloid cells. [myslide.es]
Manifestation code identifies conditions for which it is important to record both the etiology and the symptom of the disease. [books.google.com]
The etiology is commonly idiopathic or autoimmune but some cases are associated with systemic infection such as tuberculosis. [bmcresnotes.biomedcentral.com]
Although the etiology and pathogenesis of scleromalacia perforans are diverse and they are not completely understood, ophthalmic surgery is one of the well known causes of scleromalacia perforans. [jrd.or.kr]
Epidemiology
Epidemiology of scleromalacia perforans The disease is of autoimmune origin causing chronic inflammation. The disease affects the middle-aged to the elderly, particularly people over 40 years of age. [visionmagazineonline.co.za]
Keywords: Scleromalacia perforans; Rheumatoid nodules; Extraarticular arthritis; Immunosuppressive therapy Classification, Epidemiology, Clinical Manifestations The sclera is a dense connective tissue covering about five sixths of the eye [1,2]. [longdom.org]
Hodson K, Galor A, Karp K, et al., Epidemiology and visual outcomes in patients with infectious scleritis, Cornea, 2013;32:466–72. 6. [touchophthalmology.com]
Tuberculosis Gout Lyme disease Foreign body Hypertension Surgically-induced scleritis is a rare complication following ophthalmologic procedures such as the following: Cataract surgery Pterygium excision Strabismus surgery Retinal detachment repair.ref16} Epidemiology [emedicine.medscape.com]
Pathophysiology
Pathophysiology Young and Watson suggest three determinants of scleral destruction: activation of scleral fibrocytes and resorption of pericellular matrix, infiltration of the scleral stroma by inflammatory cells, prolonged local vaso-occlusion. [eyewiki.aao.org]
Prevention
Glaucoma implant surgery using autogenous fascia lata to form a healthy ground and a patch graft to prevent tube erosion seems to be a safe and effective method when normal sclera is lacking, such as in scleromalacia perforans. [ncbi.nlm.nih.gov]
M.D.C MDC Category Not Assigned For This ICD Code H15.05 Medicare Severity-Diagnosis Related Groups MS-DRG MS-DRG Category Not Assigned For This ICD Code H15.05 Clinical Classifications Software CCS CCS Category Not Assigned For This ICD Code H15.05 Prevention [medicbind.com]