Bilateral parotid gland enlargement is seen in all except
Ref Robbins 9/e p225_227 Clinical Manifestations SLE is a multisystem disease that is highly variable in clini- cal presentation. Typically, the patient is a young woman with some, but rarely all, of the following features: a but- terfly rash over the face, fever, pain and swelling in one or more peripheral joints (hands and wrists, knees, feet, ankles, elbows, shoulders), pleuritic chest pain, and photo- sensitivity. In many patients, however, the presentation of SLE is subtle and puzzling, taking forms such as a febrile illness of unknown origin, abnormal urinary findings, or joint disease masquerading as RA or rheumatic fever. ANAs are found in viually 100% of patients, but an impoant point is that ANAs are not specific (Table 4-10). A variety of clinical findings may point toward renal involvement, including hematuria, red cell casts, protein- uria, and in some cases the classic nephrotic syndrome (Chapter 13). Laboratory evidence of some hematologic derangement is common, and in some patients anemia or thrombocytopenia may be the presenting manifestation as well as the dominant clinical problem. In still others, neu- ropsychiatric manifestations, including psychosis or con- vulsions, or coronary aery disease may be prominent clinical problems. Patients with SLE are also prone to infections, presumably because of their underlying immune dysfunction and treatment with immunosuppressive drugs. Recent strategies include B cell depletion with anti- CD20 antibody (Rituximab) and by blocking growth factors. The course of the disease is variable and unpredict- able. Rare acute cases progress to death within weeks to months. More often, with appropriate therapy, the disease is characterized by flareups and remissions spanning a period of years or even decades. During acute flareups, increased deposition of immune complexes and the accom- panying complement activation are thought to result in hypocomplementemia. Disease exacerbations usually are treated with coicosteroids or other immunosuppressive drugs. Even without therapy, in some patients the disease may run a benign course with only skin manifestations and mild hematuria for years. The outcome has improved sig- nificantly, and a 5-year survival can be expected in approx- imately 95% of patients. The most common causes of death are renal failure, intercurrent infections, and cardiovascular disease. The incidence of cancer also is increased, paicularly B cell lymphomas, an association common to diseases marked by B cell hyperstimulation (e.g., Sjogren syndrome, discussed below). Patients treated with steroids and immunosup- pressive drugs incur the usual risks associated with such therapy
Sarcoidosis
Sjogern's syndrome
SLE
Viral infection
General anatomy