January 10, 2005
In depth explanation of some of the symptoms involved with Lupus
Systemic Effects
Constitutional
90% of patients with SLE experience fatigue. Arthralgia and myalgia often accompany complaints of malaise. A less common but more serious constitutional feature of SLE is persistent fever and weight loss.
Musculoskeletal
Approximately 90% of patients with SLE have musculoskeletal symptoms. The typical clinical manifestation is arthralgia. The joints most commonly involved are the proximal interphalangeal, metacarpophalangeal, wrist, and knees. In contrast to rheumatoid arthritis, however, lupus is rarely accompanied by frank articular erosions. When arthritis occurs in SLE it usually is the consequence of periarticular inflammation with involvement of tendons. This can lead to Jaccoud's arthropathy which is notable for reducible deformities. Myalgias are another common feature of SLE. Less common is frank inflammatory myositis which occurs occasionally during the course of SLE. Steroid induced myopathy is a potential source of confusion. However, with inflammatory muscle disease, there is usually an elevation of the muscle enzymes, such as creatine phosphokinase, lactate dehydrogenase or aldolase.
Mucocutaneous
Mucosal ulcers are not an infrequent complication of lupus, occurring in 30% of patients. They most often occur on the hard or soft palate but also may be found on the nasal septum. The ulcers are usually painless and undetected by the patient but may be painful when there is a secondary infection, such as oral candidiasis. It is controversial whether the ulcers represent a simple inflammatory mucositis or a frank vasculitis of the mucous membranes.
Approximately 80% of patients with SLE have dermatological manifestations during the course of their illness. The acute cutaneous eruption is manifest as a photosensitive rash which often has a butterfly appearance by virtue of involving the bridge of the nose and malar areas of the face. A characteristic feature of this rash is sparing of the nasolabial folds. Photosensitivity is less common in patients of color but occurs in 50% of all patients with SLE. The rash of subacute cutaneous lupus is observed in anti-Ro positive patients. This eruption is intermediately photosensitive and can either have an annular, polycyclic appearance or a more papulosquamous, pityriasiform, or psoriasiform appearance. 25% of patients with SLE have discoid skin lesions. These lesions are often on the face with a predilection for the inner pinna of the ear but are not photosensitive. These lesions are characterized clinically by follicular plugging, skin atrophy, scaling, telangiectasia and skin erythema.
Alopecia occurs in 50% of patients. Typically this is manifest as reversible hair thinning during periods of disease activity. This is demonstrated by the ease with which hair can be plucked from the scalp and the development of "lupus hairs" (i.e. short strands at the scalp line). Following an acute, usually febrile, exacerbation of SLE patients may experience precipitous generalized hair loss as part of a telogen effluvium. This results from a period of arrested hair growth during the acute episode. Discoid lesions involving the scalp leads to scarring alopecia.
Unusual cutaneous manifestations of lupus include urticaria, angioedema, bullae and panniculitis known as lupus profundus.
Raynaudus phenomenon is observed in 30% of patients. Livedo reticularis occurs with increased incidence in patients with SLE. Livedo also may be a marker for patients with SLE and the secondary antiphospholipid antibody syndrome. Digital purpura is another manifestation of SLE and may occur as the consequence of vasculitis. Palpable purpura with histologic evidence of leukocytoclastic vasculitis is an occasional feature of SLE.
Serositis
Inflammatory serositis of the pleura, pericardium and peritoneum occurs in 50% of patients with SLE. This may produce pleuritis, pericarditis and medical peritonitis. These may occur in the absence of any significant effusion and represent a non-effusive serositis. Alternatively, patients can develop large pleural effusions, pericardial effusions or ascites. These effusions are typically inflammatory and exudative. Frank cardiac tamponade has been reported, albeit, on rare occasions.
Hematological
Anemia of chronic inflammation is a common feature of exacerbated SLE. Coombs positive hemolytic anemia with an acute declining hematocrit and reticulocytosis is a characteristic but not especially common occurrence in SLE, appearing in 10% of patients. Autoimmune thrombocytopenia purpura can be a presenting feature of SLE or occur at any time in the course of the illness. Thrombocytopenia as a consequence of the antiphopholipid antibody syndrome has also been described in SLE. Leukopenia with lymphopenia is also a characteristic feature of SLE. Interestingly, when this occurs in the absence of cytotoxic drug therapy of the illness, it is not a significant risk for infection.
Renal
Although pathologically the majority of patients with SLE may have glomeruplopathy clinically relevant kidney disease occurs in about 50% of patients. This is usually the consequence of the deposition of immune complexes containing anti-DNA in the kidney. Serum antibodies to anti-DNA are a marker for the development of renal disease. Hypocomplementemia is often a harbinger of active renal disease. Mesangial lupus nephropathy is generally associated with an excellent prognosis. Proliferative lupus nephropathy, especially diffuse proliferative, often has a nephritic picture with hypertension, urinary red cell casts and can be accompanied by significant deterioration in renal function. Nephrotic syndrome in the absence of hypertension, active urinary sediment, or significant hypocomplementemia suggests membranous lupus nephropathy.
Central Nervous System
Neuropsychiatric complications occur in 50% of SLE patients and include acute and chronic, as well as focal and diffuse manifestations. Cerebral vascular accidents are the consequence of either inflammatory or non-inflammatory, thrombotic vasculopathy in the central nervous system. Seizures complicate the course in 25% of patients with lupus. Diffuse cerebral dysfunction is manifest as an organic effective disorders, personality disorder, psychosis, or coma. Vascular or migraine headaches occur in 10% of lupus patients. Recurrent involvement of the central nervous system may result in an organic brain syndrome and dementia.
Secondary Antiphospholipid Antibody Syndrome
Patients with SLE have an increased incidence of the antiphopholipid antibody syndrome. This syndrome is defined by the co-occurrence of thrombotic events and the presence of autoantibodies against negatively charged phospholipid, such as a biological false-positive VDRL, lupus anticoagulant, or anti-cardiolipin antibody. This syndrome occurs most frequently in patients with high titer IgG anti-cardiolipin antibodies or lupus anticoagulant. Patients with this disorders are at risk for recurrent arterial and venous thrombosis, thrombocytopenia, and fetal wastage. The mechanisms of this prothrombotic diathesis are uncertain, but these autoantibodies, perhaps interacting with co-factors, bind to target antigens on endothelial cells, platelets or coagulation factors producing a hypercoaguable state.
Ocular
Patients with lupus may develop anterior uveitis or iridocyclitis. Frank retinal vasculitis has been described, as well as central retinal artery occlusion, central retinal vein occlusion and ischemic optic neuropathy. Xerostomia with keratoconjunctivitis sicca is seen in 10% of patients.
Lung
As mentioned, the most common involvement of the lung is inflammatory serositis producing pleuritis. However, patients with lupus can also develop transient hypoxia on the basis of pulmonary leukosequestration, inflammatory pneumonitis, interstitial pulmonary fibrosis, pulmonary hypertension, diaphragmatic dysfunction, and phrenic nerve palsy.
Cardiac
The most common cardiac manifestation is pericarditis with or without effusion. Additionally, patients with lupus can develop myocarditis. Nonbacterial verrucous endocarditis or Libman-Sacks endocarditis produces millimeter vegetation on the mitral and aortic valve. These are usually asymptomatic and an incidental pathologic finding at autopsy. Rarely, they can be a cause of cerebral or coronary artery embolization. Thrombotic valvulitis and thrombosis of cardiac chambers have been described in patients with the antiphospholipid antibody syndrome. Active SLE can be accompanied by coronary artery vasculitis and, on rare occasion, this has produced myocardial infarction.
There is an increased incidence of atherosclerotic heart disease in SLE, including in premenopausal women. This may be related to coronary artery pathology initiated by immune complex deposition, but certainly is aggravated by chronic steroid therapy which can produce hyperlipidemia and hyperglycemia. Additionally, the hyperlipidemia of the nephrotic syndrome that can accompany lupus nephritis promotes atherosclerosis. SLE patients with the secondary antiphospholipid antibody syndrome also develop myocardial infractions but on the basis of bland coronary artery thrombosis.
Gastrointestinal
Medical peritonitis with or without ascites is a manifestation of lupus serositis involving the peritoneum. Less common manifestations of lupus involving the gastrointestinal tract include mesenteric ischemia from mesenteric vasculitis and pancreatitis. The latter can be a manifestation of disease activity, or less commonly, a consequence of disease treatment as with steroids. Nonspecific inflammatory liver disease has been described in lupus. Liver function abnormalities in lupus, however, are most commonly explained by idiosyncratic reactions to aspirin, anti-inflammatory drugs, hydroxychloroquine, or azathioprine. Progression to cirrhosis as a consequence of inflammatory liver disease in SLE is rare.
Posted by Lisa on January 10, 2005 04:40 PM
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