• These disorders include a chronic inflammatory disease called Aicardi-Goutieres syndrome, as well as systemic lupus erythematosus (SLE), including a rare form of SLE called chilblain lupus that mainly affects the skin. (wikipedia.org)
  • Systemic Lupus Erythematosus (SLE) Systemic lupus erythematosus is a chronic, multisystem, inflammatory disorder of autoimmune etiology, occurring predominantly in young women. (msdmanuals.com)
  • Systemic lupus erythematosus (SLE) has been the most commonly reported rheumatologic cause of ESRD. (medscape.com)
  • This chapter emphasizes congenital and genetic disorders, but we also discuss the pathophysiology and manifestation of various acquired neurocutaneous disorders with an emphasis Behcet's disease, dermatomyositis, Sjögren's syndrome, systemic lupus erythematosus, scleroderma, Parry-Romberg syndrome and Degos disease. (degosdisease.org)
  • Granulomatosis with polyangiitis (formerly known as Wegner's granulomatosis). (clevelandclinic.org)
  • Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss vasculitis). (clevelandclinic.org)
  • Polyarteritis nodosa, granulomatosis with polyangiitis, Henoch-Schönlein purpura, scleroderma, and otherwise nonspecified vasculitides also were reported to have caused ESRD during this period. (medscape.com)
  • With systemic and congenital disorders, extraocular manifestations are observed. (lecturio.com)
  • Sarcoidosis is a non-caseating granulomatous multisystem disease with a wide range of clinical and radiographic manifestations. (radiopaedia.org)
  • 2. Autoimmune disorders (14.00D) . Autoimmune disorders are caused by dysfunctional immune responses directed against the body's own tissues, resulting in chronic, multisystem impairments that differ in clinical manifestations, course, and outcome. (ssa.gov)
  • These systemic disorders and the associated renal diseases and cutaneous manifestations are tabulated in Table 1, below. (medscape.com)
  • This article does not discuss systemic disorders, because most of them are discussed in other articles in Medscape Reference . (medscape.com)
  • Various conditions involving the central nervous system (CNS) can emerge as RPD, including Creutzfeld-Jakob disease (CJD) and other spongiform encephalopathies, vascular disorders, autoimmune and paraneoplastic encephalopathies, subacute infections, metabolic and toxic disorders and systemic diseases (see Table 1 ). (touchneurology.com)
  • Sarcoidosis is a multisystem disorder of unknown etiology characterized by the formation of inflammatory non-caseating granulomas within affected tissues. (radiopaedia.org)
  • Nephrology: nephritis, nephritic proteinuria associated with systemic autoimmune disease. (clevelandclinic.org)
  • The initial clinical course and the extent of systemic vasculitic lesions may influence the long-term functional prognosis. (lookformedical.com)
  • AGN can be due to a primary renal disease or to a systemic disease. (medscape.com)
  • Most patients undergo spontaneous resolution or present a good response to systemic treatment, but a small percentage of patients develop a chronic, progressive disease, which can be refractory to multiple lines of treatment [ 1 ]. (biomedcentral.com)
  • We believe that cysteinyl leukotriene type 1 receptor antagonists are safe and effective drugs for most patients with asthma but caution is needed for those with more severe disease who require systemic corticosteroids, especially if they show characteristics of the atypical allergic diathesis seen in the prodromal phase of Churg-Strauss syndrome. (lookformedical.com)
  • The majority of patients (77.1%) were treated with oral prednisolone, either alone (11.5%) or in combination (65.6%) with other topical and systemic agents. (bvsalud.org)
  • lupus anticoagulants, recurrent venous thrombosis, stroke, spontaneous abortions, livedo reticularis) also can lead to sterile endocardial vegetations and systemic emboli. (msdmanuals.com)
  • The decreased GFR and avid distal nephron salt and water retention result in expansion of intravascular volume, edema, and, frequently, systemic hypertension. (medscape.com)
  • Progressive neurologic deterioration unresponsive to systemic immunosuppression including corticosteroid therapy and chemotherapeutic agents. (wikipedia.org)
  • Systemic autoimmune conditions need a systemic corticosteroid as a fast-acting agent to halt the inflammatory process while cytotoxic therapy maintains long term disease control. (avehjournal.org)
  • Progressive neurologic deterioration unresponsive to systemic immunosuppression including corticosteroid therapy and chemotherapeutic agents. (wikipedia.org)
  • The study team has found that topical photodynamic therapy (PDT) is a very potent inducer of PAF in murine skin and induces systemic immunosuppression only in mice expressing PAF receptors. (nih.gov)
  • Peripheral ulcerative keratitis is an important condition as the presence of comorbid systemic disease is associated with significant morbidity and increased mortality. (avehjournal.org)
  • Sarcoidosis is an inflammatory, multisystem, granulomatous disease of unknown etiology, and although it predominantly affects the lungs, sarcoidosis often has ophthalmologic manifestations. (medscape.com)
  • AGN can be due to a primary renal disease or to a systemic disease. (medscape.com)
  • Malignant atrophic papulosis is a multisystem disorder involving small-caliber blood vessels. (medscape.com)
  • Which is a specific familial systemic granulomatosis, which is akin to sarcoid, which is a close relative. (cybersight.org)
  • Only 4 of the 30 cases (13%) had systemic involvement. (medscape.com)
  • Patients with multisystem involvement have a poor prognosis, with a mean survival of approximately 2 years. (medscape.com)
  • Most patients with ophthalmic sarcoidosis have evidence of systemic involvement at the time of the initial examination. (medscape.com)
  • With systemic disease, the reported mean survival is approximately 2 years, but there is a wide variation, from less than 1 year to more than 12 years. (medscape.com)
  • In which case, the systemic manifestation of the disease may be delayed or completely obliterated. (cybersight.org)
  • So sometimes patients never develop the systemic manifestations, because of the concurrent systemic treatment that is given for the ocular disease. (cybersight.org)
  • In a series of patients with orbital and adnexal sarcoidosis alone at presentation, systemic disease developed in 8% by 5 years. (medscape.com)
  • Neuro-ophthalmic findings may present without systemic evidence of disease and elude diagnosis until a biopsy is performed. (medscape.com)
  • As a multisystem disease, GPA often presents with nonspecific symptoms and can be difficult to recognise in primary care. (patient.info)
  • vasculitides should be considered in patients presenting with constitutional symptoms and signs of multisystem disease (e.g., palpable purpura , pulmonary infiltrates, unexplained ischemic events). (amboss.com)
  • 2 It may be the first presentation of systemic disease, and early diagnosis and management has the potential to not only preserve vision but also reduce mortality. (avehjournal.org)
  • [ 9 , 10 ] The benign and malignant variants are clinically indistinguishable initially but become distinct once systemic complications arise. (medscape.com)
  • CSS has also been shown to be a pathological syndrome of angiitis and allergic granulomatosis [2]. (who.int)