Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0851184 (thinning)
 11,252 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Although systemic immunosuppressive chemotherapy is effective in halting progressive necrotizing scleritis, the onset of its action may be too slow to prevent profound scleral thinning and/or traumatic or spontaneous perforation. Scleral homografts may be used to maintain the integrity of the globe until immunosuppressive drugs can take effect. The authors reviewed their experience with scleral homografts in 12 patients with progressive necrotizing scleritis; eight (all with autoimmune disease) had concomitant chemotherapy and four (two with autoimmune disease) did not. Grafts remained stable in patients receiving both surgical and drug therapy over a mean follow-up of 12 months. One graft melted after discontinuation of chemotherapy, but regrafting and renewed immunosuppression salvaged the eye. Grafts in two of the patients not initially given chemotherapy melted rapidly (within 14 and 45 days, respectively). Both eyes were salvaged by regrafting and/or addition of chemotherapy. Though rarely successful by itself against necrotizing scleritis, scleral grafting is a useful adjunct to chemotherapy.
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PMID:Scleral grafting for necrotizing scleritis. 271 May 20

The defining ultrastructural features of hereditary nephritis are "basket weave" lamellation or thinning of glomerular basement membranes. Electron-dense deposits are not seen and immunofluorescence (IF) is generally negative. In this study, we report 5 cases of hereditary nephritis in which substantial amounts of glomerular electron-dense deposits were identified on electron microscopy, with corresponding positive IF staining in 4 cases, suggesting immune complex-mediated glomerulonephritis. However, no case had histological evidence of glomerular endocapillary or extracapillary proliferation or leukocyte infiltration typical of active glomerulonephritis. Four cases were diagnosed at outside institutions simply as forms of glomerulonephritis without considering the possibility of hereditary nephritis and were sent for consultation in contemplation of possible immunosuppressive therapy. All patients had negative serologies and no known underlying infectious or autoimmune disease; 4 patients had family history of hematuria or renal disease. The glomerular electron-dense deposits were predominantly mesangial (4 cases) and intramembranous (4 cases), as well as subepithelial (2 cases) or subendothelial (1 case). Corresponding IF positivity for immune reactants was identified in 4 cases, and IgG was the predominant immunoglobulin deposited. A characteristic feature was the tendency for deposits to form between the complex layers of glomerular basement membrane material, favoring a process of nonspecific entrapment of immune reactants within the thickened, lamellated basement membrane. In all cases, a diagnosis of hereditary nephritis was confirmed by demonstration of the characteristic loss of immunoreactivity for the alpha5 subunit of collagen IV (4 cases) or Goodpasture's antigen (1 case) in renal or epidermal basement membranes. These cases expand the spectrum of unusual pathological findings in hereditary nephritis and emphasize the potential for hereditary nephritis to mimic immune complex glomerulonephritis.
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PMID:Hereditary nephritis mimicking immune complex-mediated glomerulonephritis. 1664 52

Relapsing polychondritis (RP) is a rare systemic autoimmune disease characterized by episodic inflammation of cartilaginous tissues throughout the body. It is distinguished by recurrent bouts of inflammation, which lead to the permanent destruction of the involved structures. It can be a life-threatening, debilitating, and frightening disease and is often difficult to diagnose in its early stages. Ocular manifestations have been reported to occur in up to 65% of cases and include proptosis, eyelid edema, extraocular muscles palsy, episcleritis, scleritis, conjunctivitis, corneal infiltrate, peripheral ulcerative keratitis, corneal thinning or perforation, iridocyclitis, cataract, retinopathy, exudative retinal detachment, and optic neuritis. Corticosteroids remain the mainstay of treatment for RP; however, other treatment modalities include nonsteroidal anti-inflammatory drugs, colchicine, dapsone, and immunomodulatory drugs. This article reviews the literature and summarizes the epidemiology, pathogenesis, clinical features, treatment, and prognosis of the systemic and ocular manifestations of RP.
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PMID:Relapsing polychondritis: systemic and ocular manifestations, differential diagnosis, management, and prognosis. 2195 72

Neuromyelitis optica (NMO) is an inflammatory autoimmune disease of the central nervous system that preferentially targets the optic nerves and spinal cord. The clinical presentation may suggest multiple sclerosis (MS), but a highly specific serum autoantibody against the astrocytic water channel aquaporin-4 present in up to 80% of NMO patients enables distinction from MS. Optic neuritis may occur in either condition resulting in neuro-anatomical retinal changes. Optical coherence tomography (OCT) has become a useful tool for analyzing retinal damage both in MS and NMO. Numerous studies showed that optic neuritis in NMO typically results in more severe retinal nerve fiber layer (RNFL) and ganglion cell layer thinning and more frequent development of microcystic macular edema than in MS. Furthermore, while patients' RNFL thinning also occurs in the absence of optic neuritis in MS, subclinical damage seems to be rare in NMO. Thus, OCT might be useful in differentiating NMO from MS and serve as an outcome parameter in clinical studies.
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PMID:Neuromyelitis optica and multiple sclerosis: Seeing differences through optical coherence tomography. 2566 42

Autoimmune diseases such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and systemic sclerosis (scleroderma) preferentially affect women, and are characterized by systemic inflammation leading to target organ dysfunction. The public health burden of autoimmune diseases, which collectively represent a leading cause of morbidity and mortality among women throughout adulthood, is substantial. While some features of these diseases have been observed to improve over the menopausal transition, such as disease flare rate in SLE and skin softening and thinning in scleroderma, others, such as swollen and tender joints and radiographically confirmed damage in RA may worsen. The general trends, however, are not consistent or conclusive for all disease-related manifestations. Of great importance is the recognition that comorbid diseases, including osteoporosis and accelerated cardiovascular disease, contribute excess morbidity and mortality that becomes increasingly apparent as women with autoimmune diseases undergo the menopausal transition.
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PMID:Rheumatic autoimmune diseases in women and midlife health. 2855 45

We report an unusual presentation in a 9-year-old girl with unilateral circumferential thinning of the entire right upper limb without any other neurological deficit, with normal nerve conduction and electromyography initially thought of as a neurodegenerative disorder based on clinical presentation. Magnetic resonance imaging of the upper limb showed partial lipoatrophy with normal glucose metabolism and lipid profile and negativity for HIV and autoimmune disease (panniculitis) with no family history of similar disorder. Remember to think out of box before labeling neurodegenerative disease.
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PMID:Unilateral Limb Thinning -Thinking Out of the Box. 2972 Aug 3

Peripheral ulcerative keratitis (PUK) is a group of corneal disorders that cause peripheral corneal thinning, threatening globe integrity in advance stages. It is usually associated with systemic autoimmune diseases and management is based on local and systemic approaches. We present the case of a 47-year-old man with a previous history of bacterial keratitis in his left eye presenting with 1 month of bilateral ocular pain and redness. At examination, diffuse bilateral globe inflammation with paracentral corneal thinning in his left eye was observed. He was diagnosed with bilateral scleritis and PUK in his left eye. Workup for associated systemic autoimmune disease yielded negative results. The patient was started on pulses of intravenous methylprednisolone followed by oral prednisone failing to achieve sufficient control of the inflammatory syndrome. Subsequently, periodic intravenous cyclophosphamide was administered with a favorable response. A multilayer amniotic membrane graft was applied, but there was rapid melting with reabsorption of the tissue, resulting in extreme corneal thinning at the inferior paracentral cornea. A decentered 8.5-mm superficial anterior lamellar keratoplasty (SALK) was then performed obtaining the donor graft with a femtosecond laser but performing manual trepanation in the recipient. At 12 months, visual and biomicroscopic measures do not show deterioration and inflammation remains under control with oral azathioprine as maintenance regime. The management of PUK includes both systemic immunosuppression and tectonic procedures to preserve the globe integrity. Diverse surgical techniques have been attempted, but no definitive guidelines are available. Decentered large SALK is a simple technique that can yield acceptable visual results.
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PMID:Peripheral Ulcerative Keratitis in a Patient with Bilateral Scleritis: Medical and Surgical Management. 3317 97