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754          Part SIX  Systemic Immune Diseases



         TABLE 55.6  Differential Diagnosis of                 REFERENCES
         Systemic Sclerosis (SSc) and Scleroderma
                                                                1.  Maricq HR, Weinrich MC, Keil JE, et al. Prevalence of scleroderma
          Disorders Characterized by Similar Presentations        spectrum disorders in the general population of South Carolina. Arthritis
          •  Systemic lupus erythematosus                         Rheum 1989;32:998–1006.
          •  Sjögren syndrome                                   2.  Mayes MD, Lacey JV Jr, Beebe-Dimmer J, et al. Prevalence, incidence,
          •  Rheumatoid arthritis                                 survival, and disease characteristics of systemic sclerosis in a large US
          •  Polymyositis/dermatomyositis                         population. Arthritis Rheum 2003;48:2246–55.
          •  Primary Raynaud phenomenon                         3.  Bossini-Castillo L, López-Isac E, Martín J. Immunogenetics of systemic
                                                                  sclerosis: defining heritability, functional variants and
          Disorders Characterized by Similar Visceral Features    shared-autoimmunity pathways. J Autoimmun 2015;64:53–65.
          •  Primary pulmonary hypertension                     4.  Hummers LK. The importance of recognizing scleroderma-type disorders
          •  Primary biliary cirrhosis                            in clinical practice. Nat Clin Pract Rheumatol 2008;4:638–40.
          •  Idiopathic intestinal hypomotility                 5.  Janowsky EC, Kupper LL, Hulka BS. Meta-analyses of the relation
          •  Idiopathic pulmonary fibrosis                        between silicone breast implants and the risk of connective-tissue
          •  Malignant hypertension                               diseases. N Engl J Med 2000;342:781–90.
                                                                6.  Bhattacharyya S, Wei J, Varga J. Understanding fibrosis in systemic
          Disorders Characterized by Skin thickening              sclerosis: shifting paradigms, emerging opportunities. Nat Rev Rheumatol
          •  Scleromyxedema                                       2011;8:42–54.
          •  Scleredema (of Buschke), diabetic scleredema       7.  Kuwana M, Okazaki Y, Yasuoka H, et al. Defective vasculogenesis in
          •  Nephrogenic fibrosing dermatopathy                   systemic sclerosis. Lancet 2004;364:603–10.
          •  Eosinophilic fasciitis/diffuse fasciitis with eosinophilia  8.  Sato S, Fujimoto M, Hasegawa M, et al. Altered blood B lymphocyte
          •  Eosinophilia–myalgia syndrome                        homeostasis in systemic sclerosis: expanded naive B cells and diminished
          •  Generalized morphea                                  but activated memory B cells. Arthritis Rheum 2004;50:1918–27.
          •  Chronic graft versus host disease                  9.  Harris ML, Rosen A. Autoimmunity in scleroderma: the origin,
          •  POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy,
           monoclonal protein, skin changes)                      pathogenetic role, and clinical significance of autoantibodies. Curr Opin
          •  Amyloidosis                                          Rheumatol 2003;15:778–84.
          •  Carcinoid syndrome                                10.  Mori Y, Chen SJ, Varga J. Expression and regulation of intracellular
          •  Pentazocine-induced scleroderma                      SMAD signaling in scleroderma skin fibroblasts. Arthritis Rheum
          •  Diabetic digital sclerosis                           2003;48:1964–78.
          •  Vinyl chloride disease                            11.  Domsic RT, Rodriguez-Reyna T, Lucas M, et al. Skin thickness
          •  Toxic oil syndrome                                   progression rate: a predictor of mortality and early internal organ
          •  Bleomycin exposure                                   involvement in diffuse scleroderma. Ann Rheum Dis 2011;70:104–9.
          •  Werner syndrome                                   12.  Mendoza F, Derk CT. Systemic sclerosis mortality in the United States:
          •  Phenylketonuria                                      1999-2002 implications for patient care. J Clin Rheumatol
          •  Porphyria cutanea tarda                              2007;13:187–92.
          •  Vibration white finger syndrome                   13.  LeRoy EC, Medsger TA Jr. Criteria for the classification of early systemic
          •  Chronic reflex sympathetic dystrophy                 sclerosis. J Rheumatol 2001;28:1573–6.
                                                               14.  Chung L, Domsic RT, Lingala B, et al. Survival and predictors of
                                                                  mortality in systemic sclerosis-associated pulmonary arterial
                                                                  hypertension: outcomes from the pulmonary hypertension assessment
                                                                  and recognition of outcomes in scleroderma registry. Arthritis Care Res
                                                                  2014;66:489–95.
        histological evidence of an inflammatory process in the fasciae   15.  Flavahan NA. A vascular mechanistic approach to understanding
        is present. Nephrogenic systemic fibrosis is described in patients   Raynaud phenomenon. Nature 2015;11:146–58.
        with end-stage renal disease who were exposed to gadolinium-  16.  Cappelli L, Wigley FM. Management of Raynaud phenomenon and
                         35
        based contrast agents.  Scleromyxedema, a condition of mucinous   digital ulcers in scleroderma. Rheum Dis Clin North Am 2015;41:
        deposition in skin, is associated with the presence of monoclonal   419–38.
        gammopathy that is characterized by a diffuse, papular skin   17.  Korn JH, Mayes M, Matucci Cerinic M, et al. Digital ulcers in systemic
        eruption. Scleredema occurs with deposition of collagen and   sclerosis: prevention by treatment with bosentan, an oral endothelin
                                                                  receptor antagonist. Arthritis Rheum 2004;50:3985–93.
        mucin in the dermis and primarily involves the upper back,   18.  Roberts CG, Hummers LK, Ravich WJ, et al. A case-controlled study of
        neck, and face. It occurs in association with diabetes, a monoclonal   the pathology of oesophageal disease in systemic sclerosis (scleroderma).
        gammopathy, or postinfection pharyngitis, particularly streptococ-  Gut 2006;55:1697–703.
        cal pharyngitis. GvHD occurs after bone marrow or stem cell   19.  Tyndall AJ, Bannert B, Vonk M, et al. Causes and risk factors for death in
        transplantation and is characterized by patchy areas of inflam-  systemic sclerosis: a study from the EULAR Scleroderma Trials and
        matory and thickened skin with liver and GI involvement. Toxin   Research (EUSTAR) database. Ann Rheum Dis 2010;69:1809–15.
        exposure was linked to the toxic oil syndrome (adulterated   20.  Goh NS, Desai SR, Veeraraghavan S, et al. Interstitial lung disease in
        rapeseed oil) and eosinophilia–myalgia syndrome (related to   systemic sclerosis: a simple staging system. Am J Respir Crit Care Med
        the presence of an impurity in the production process for   2008;177:1248–54.
        L-tryptophan, a sleep aid). These syndromes have largely subsided   21.  Tashkin DP, Elashoff R, Clements PJ, et al. Scleroderma Lung Study
        by banning the agents involved, but sporadic cases of eosinophilia–  Research Group. Cyclophosphamide versus placebo in scleroderma lung
                                                                  disease. N Engl J Med 2006;354:2655–66.
        myalgia syndrome continue to occur. 36                 22.  Clements PJ, Tashkin D, Roth M, et al. The Scleroderma Lung Study II
                                                                  (SLS II) shows that both oral cyclophosphamide (CYC) and
        Please check your eBook at https://expertconsult.inkling.com/   mycophenolate mofetil (MMF) are efficacious in treating progressive
        for self-assessment questions. See inside cover for registration   interstitial lung disease (ILD) in patients with systemic sclerosis (SSc).
        details.                                                  Arthritis Rheumatol 2015;67(Suppl. 10). http://acrabstracts.org/abstract/
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