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Contributor: Laura Tarter, MD
A 25 year old female presents with fatigue, arthralgias, and a rash. She reports that she has been having joint pain with worsened stiffness and swelling in the mornings for the past three months. She has also noted rashes, particularly on her face and neck, over this same time period. On exam, several MCPs are tender to palpation with trace synovitis, and she has multiple well-demarcated erythematous plaques on her face and neck with fine overlying scale. Basic labs are notable for a total lymphocyte count of 500, a creatinine of 1.6, and red blood cell casts and 3+ proteinuria on urinalysis. A 24-hour urine protein returns at 4.2 grams/day. Autoantibody testing is notable for a positive ANA at a titer of 1:640 with a diffuse homogeneous staining pattern, the presence of high titer double stranded DNA antibodies, the presence of anti-Smith antibodies, and hypocomplementemia.
What is the significance of the tests ordered in making the diagnosis of SLE?
What are the typical features seen in this patient that are helpful in making the diagnosis of SLE?
What are the next steps in management of this patient?
A 32 year old female with a 7 year history of SLE (manifestations: +ANA, +Smith, +SSA, malar rash, pleuritis, arthritis) comes to your primary care clinic for a routine visit. She has been doing well. Her medications include Hydroxychloroquine 400mg daily and a multivitamin.
From a primary care perspective, what health care maintenance should be addressed with this patient?
(Answer questions 1 – 5 on a piece a paper. Find Answer Key at the bottom on the page.)
A 28 year-old woman presents with fatigue, arthralgias, intermittent headaches, and easy bruising. Basic laboratory testing is notable for a white blood cell count of 3.2, absolute lymphocyte count of 0.8, and platelets of 15. On chart review, a complete blood count 2 years prior was notable for a white blood cell count of 3.0, and an absolute lymphocyte count of 0.6. Creatinine, liver function tests, albumin, urinalysis, and coagulation testing are all normal. Autoantibody testing is notable for a positive ANA at a titer of 1:1280 with a diffuse homogeneous staining pattern, the presence of double stranded DNA antibodies at a titer of 1:640, and hypocomplementemia. Exam is notable for malar erythema, multiple painless oral ulcers, and ecchymoses predominantly over the extensor surfaces of her lower extremities. No splenomegaly was noted on exam.
1) Does this patient meet classification criteria for SLE?
2) Which of the following etiologies should be considered in the differential diagnosis of her thrombocytopenia?
A 32 year-old male with known SLE (+ANA, +Smith, hypocomplementemia, discoid lupus erythematosus (DLE), prior class III/V lupus nephritis, prior neuropsychiatric SLE) is admitted with chest pain. He describes the pain as pleuritic in nature, worse with lying down, and improved with leaning forward. He has had mild upper respiratory symptoms for 1 week, low-grade fevers at home, and does note a non-productive cough. He has been on a stable dose of Plaquenil 400mg daily, Mycophenolate mofetil 1000mg daily, and Prednisone 5mg daily. No recent travel.
Vital signs in the emergency room are notable for a temperature of 100 F and heart rate of 109. His lung exam is clear to auscultation bilaterally. The remainder of his exam is notable for hypopigmented peri-auricular lesions consistent with inactive DLE.
3) Which of the following is the least likely explanation of his symptoms?
The patient has classic symptoms for pleuropericarditis (pleuritic chest pain worsened with lying flat and relieved with leaning forward). However, particularly in patients with SLE, the differential for cardiopulmonary processes is broad, and it is critical that multiple potential etiologies be considered. There is a markedly increased risk of cardiovascular disease (4-fold increased risk). Pulmonary embolism is also more common in patients with SLE (often in the presence of antiphospholipid antibodies), as are infections, particularly in patients on immunosuppressive therapy.
Basic labs are notable for a white blood cell count of 3.2 and otherwise normal CBC, normal liver function tests, normal creatinine, and urinalysis without blood or protein. A chest x-ray is done and shows a normal cardiac silhouette and very slight blunting of the costophrenic angles. Apart from HR of 110, ECG is unremarkable. Initial troponin is negative.
4) What further workup is indicated?
5) At this point, what medications changes should be made to treat his symptoms?
1) The correct answer is A.
The patient meets both the ACR classification criteria for SLE (1997 update of 1982 criteria) and the 2012 SLICC classification criteria for SLE.
She fulfills 4 of 11 ACR classification criteria for SLE (> 4 needed to meet criteria): +ANA, immunologic disorder (+dsDNA, hypocomplementemia), hematologic disorder (thrombocytopenia, leukopenia on > 2 occasions, lymphopenia on > 2 occasions), malar rash, and oral ulcers.
She also fulfills 6 of 17 SLICC classification criteria for SLE (> 4 needed with at least 1 immunologic and 1 clinical criteria met): +ANA, +dsDNA, hypocomplementemia, leukopenia/lymphopenia, thrombocytopenia, acute cutaneous lupus (malar rash), oral ulcers.
2) The correct answers are: A and B.
Thrombocytopenia in SLE has multiple possible etiologies, including ITP, TTP, the antiphospholipid antibody syndrome, as well as medication toxicity, viral infection, and malignancy. Liver disease, including AIH, can lead to hypersplenism, splenic sequestration of platelets, and consequently thrombocytopenia. However, she has normal liver function tests and normal synthetic function on laboratory analysis, no splenomegaly on exam, and no stigmata of chronic liver disease, making AIH extremely unlikely.
3) The correct answer is A.
All of the above need to be addressed with further evaluation.
The patient has classic symptoms for pleuropericarditis (pleuritic chest pain worsened with lying flat and relieved with leaning forward). However, particularly in patients with SLE, the differential for cardiopulmonary processes is broad, and it is critical that multiple potential etiologies be considered. There is a markedly increased risk of cardiovascular disease (4-fold increased risk). Pulmonary embolism is also more common in patients
4) The correct answers are A and B.
Given the lack of pulmonary infiltrates on chest x-ray, bacterial pneumonia is less likely. Despite the normal cardiac silhouette and lack of ECG changes, pericarditis remains possible and an echocardiogram is indicated. A pulmonary embolism also still needs to be considered.
CT angiogram of the chest is negative for pulmonary embolism. Echocardiogram shows a small pericardial effusion and is otherwise unremarkable
5) The correct answers are A and B.
The patient has classic symptoms for pericarditis and a small effusion on echocardiogram supporting this diagnosis. Therapeutic approaches include: increasing corticosteroid dose (PO or IV if necessary), colchicine, and consideration of NSAID therapy (to be employed with caution in this patient with a history of lupus nephritis). In the outpatient setting, depending on his response, a change to his baseline immunosuppressive regimen may be indicated. However, there is not an indication for use of rituximab in this setting.
Last updated February 2015