WCIM 2014 SEOUL KOREA 235
Poster Session
The Korean Journal of Internal Medicine Vol. 29, No. 5 (Suppl. 1)
PS 0722 Rheumatology
SystemIc Lupus Erythematosus and Castleman Disease Co-Existence in a Patient with Weight Loss and Lym- phadenopathy
Yusuf HANCERLI1, Veysel OZALPER1, Abdullah HAHOLU2, Ramazan ARIKAN1, Emrullah SOLMAZGUL1
Department of Internal Medicine, GATA Haydarpasa Teaching Hospital, Turkey1, Department of Patholo- gy, GATA Haydarpasa Teaching Hospital, Turkey2
Objective: Castleman’s disease (CD) is quite rare and its etiology is not clearly unrav- elled yet. It is a lymphoproliferative disease with giant lymph node hyperplasia. This report presents newly diagnosed SLE and CD association on a 21 year old male who was transferred to our hospital with weight loss, fatigue and multiple lymphadenopa- thy (LAP) complaints along with the pre-diagnosis of lymphoma.
Case Report: In September 2013, the patient was admitted to our Hematology Clinic with the complaints of weakness, neck and arm pit swelling and weight loss. The patient was at 68 kg in November 2012, while admitted to the hospital in September 2013 he was at 47 kg. On physical examination, bilateral cervical, supraclavicular, inguinal and axillary LAP which reached 3-4 cm in size and hepatomegaly was iden- tifi ed. He had pain in both hand’s mid-interfalengeal joints and was unable to move comfortably. Labarotary fi ndings were hemoglobin:10.5 g/dl, direct coombs +2 posi- tive, platelet 79100 u/l, sedim 96, C-reactive protein 26.6, ANA: 2.672 (cut off<0,896), AntidsDNA:2.517 (cut off<0,947), nRNP/Sm positive, C3: 0.675 g/dl, C4 :0.0871 g/
dl,24- hour urine protein 1481.24 mg. The patient was diagnosed as SLE with the present fi ndings. The excisional biopsy from the right axillary lymph node area which showed the highest activity in PET CT ( SUVmax : 6.4) was considered signifi cant in the terms of CD.
Conclusion: Altough LAP ‘s most common causes are malignancy, autoimmune dis- eases and infections, it would be appropriate to take into consideration lymphoprolif- erative diseases such as CD in the stage of diagnosis evaluation in spite of very rare occurence.
PS 0723 Rheumatology
Splenic Infarcts in a Patient with Systemic Lupus Ery- thematosus and Autoimmune Phospholipid Syndrome:
A Case Report
Aissa Althea FLAVIER-HUNDAL1, Ashish ANIL SULE1, Annamarie BORJA1 Tan Tock Seng Hospital, Singapore1
Background: Systemic lupus with antiphospholipid syndrome is associated with thrombotic manifestations. There is little literature on lupus with anitiphospholipid syndrome patients presenting with splenic infarctions.
Case Presentation: This is a case of a 28-year-old Chinese Female who presented with fever, night sweats and splenomegaly and lymphadenopathies. Investigations showed Hemoglobin of 6.1g/dL, total white cell 8.1x109/L, Platelets 300x109/L, Abso- lute Reticulocyte count 182.5, Direct coombs positive, Bilirubin 12, Lactate Dehydro- genase 1264, Haptoglobin 15, ANA-IFA 640, homogenous pattern, Anti-ds-DNA >200, Lupus anti -coagulant and anti-cardiolipin positive. CT abdomen and pelvis showed splenomegaly with multiple hypodense lesions as well as abdominal and retroperito- neal adenopathy. CT neck was also done which showed polypoidal soft tissue in the nasopharynx. She underwent post-nasal space biopsy, which was negative of malig- nancy. She had bone marrow aspiration, which revealed hypercellular marrow with increase erythropoesis consistent with peripheral destruction. She also went for biopsy of the spleen, which was negative for tuberculosis and malignancy or lymphoma. Pa- tient was then started on steroids for treatment of systemic lupus erythematosus with autoimmune phospholipid syndrome and showed clinical improvement.
Discussion: Splenic hypodensities are usually benign and differential diagnoses include cysts, infarction, metastases, lymphomas, hamartomas, haemangiomas and tuberculo- sis. In a large, multicenter study, involving 1,000 adult patients with antiphospholipid syndrome, spleen infarct was present in only 1.1% of patients. Given the history of fever, splenomegaly and elevated serum lactate dehydrogenase, this case illustrates a possibility of splenic infarctions on the background of a hypercoagulable state, specifi - cally antiphospholipid syndrome.
Conclusion: Hypodensities in the spleen in a patient with lupus can be associated with antiphospholipid syndrome with splenic infarctions. It would be necessary to rule out metastases, tuberculosis and lymphoma as differential diagnoses in such cases.
PS 0724 Rheumatology
A Case Report on Bullous Systemic Lupus Erythemato- sus
Abagail Celfred Maxilom POTENCIOSO1, Joseph ANTIGUA2
Internal Medicine, Visayas Community Medical Center, Philippines1, Internal Medicine – Rheumatology, Visayas Community Medical Center, Philippines2
Bullous systemic lupus erythematosus is a generalized blistering skin eruption that uncommonly occurs in patients with established SLE and heals without scar forma- tion. It is a rare subtype of SLE with an incidence of 0.5% per million in a year and a prevalence of less than 1 per 1 million population. The clinical features of patients with SLE show a great variation, and for this reason it is diffi cult to develop a unifying concept of this disease. It is evaluated by constellations of clinical features, histolog- ical changes as well as laboratory abnormalities to exclude other causes. There is no absolute drug of choice in treating bullous SLE. It is treated medically with dapsone, antimalarial drugs and immunosuppressant agents. We report a case of a 33 year old female, who presented with multiple bullous lesions, lupus nephritis and clinical fi ndings of SLE. Appropriate laboratory tests were done to support the diagnosis of SLE. Her skin biopsy was suggestive of subepidermal blistering disease consistent with bullous SLE. Direct immunofl uorescence showed linear deposition of IgA (+2) at the basement membrane zone and Linear IgA Bullous Disease (LABD) was considered. She was treated with Hydroxychloroquine and Prednisone but due to another recurrence of skin eruption which heralds another episode of lupus nephritis, she was eventually managed as a case of Bullous SLE and was treated with Methotrexate and Prednisone.
There was a noticeable healing of her skin lesions without scar formation and im- provement of other symptoms within a few days to weeks later. With further under- standing of the evolving pathogenesis towards bullous SLE, we therefore propose that a patient diagnosed with LABD that consistently shows signs of SLE whenever the skin lesions recurs be treated and managed under the case of nonspecifi c bullous lesions of SLE.
236 32nd World Congress of Internal Medicine (October 24-28, 2014) WCIM 2014
PS 0725 Rheumatology
Case Report: Elderly-Onset Systemic Lupus Erythema- tosus
Ruben GOMEZ1, Ericka CARRILLO1, Filiberto GOMEZ1 Instituto Mexicano Del Seguro Social, Mexico
Elderly onset SLE contributes to 10% of all SLE and is considered a rare disease, the signs and symptoms are common to other more frequent diseases in this age so the differential diagnosis is wide and could represent a great clinical challenge. We report two cases, one patient presented with data of serositis, acute renal failure and glo- merulonephritis; while the other had evidence of cerebral vasculitis.
Case 1: 60 year old male with 6 months of asthenia, malaise and early-morning bilat- eral limb edema, received treatment with diuretics without improvement, presenting arterial hypertension. He was admitted with IV NYHA class Congestive HF and left pleural effusion. Laboratory reported Hb 14.3 g/dl, leucocytes 5750 (normal differential count), ESR of 50 mm/h, creatinine 2.1 mg/dl, urea 60 mg/dl, urinalysis was positive for albumin, hemoglobin and granular casts; GFR 35 ml/min, and 24-hour albuminuria 3.8 g. ECHO showed a 68% LVEF and 150 ml pericardial effusion. Immunologic profi le showed positive ANA (1:140), positive anti-dsDNA (1:60), and positive anti-SM (1:65).
Kidney biopsy showed focal-segmental glomerulonephritis.
Case 2: 68 year-old female positive for Diabetes Mellitus and Arterial Hypertension treated with glibenclamide and captopril. She presented at ER with diplopia, bilateral internuclear ophthalmoplegia and bilateral limb paraparesis, initial head CT-scan was normal, so she was admitted to the hospital. A second simple and contrasted head CT-scan was also normal, then the head MRI showed vasculitis and the brain SPECT revealed hypocaptating areas in midbrain, frontal and parietal cortex. Laboratory reported Hb 13.3 g/dl, leucocytes 2578, glycose 145 mg/dl, normal creatinine, hepatic profi le and urinalysis. Immunologic profi le reported negative ANA, positive anti-dsDNA (1:95) and positive anti-SM (1:120). Both patients received treatment with Cyclophos- phamide and Methylprednisolone with great response.
PS 0726 Rheumatology
A Diagnostic Problem of Mixed Connective Tissue Dis- ease Patient with Dyspnea and Paraplegia
Awalia AWALIA1 Airlangga University, Indonesia1
Background: Patients with mixed connective tissue disease (MCTD) show combina- tion of clinical features of SLE, systemic sclerosis (SSc), rheumatoid arthritis (RA), or polymyositis. Pleuropulmonary manifestations are common in MCTD (20-85%) and follow those seen in SLE, SSc, RA, or polymyositis. Interstitial lung disease (ILD) are the major manifestations. Neurological involvement occurs in 10% of MCTD patients, but acute transverse myelitis (ATM) is rare.
Case: A 63 year-old woman admitted to the hospital due to dyspnea and fever. She had MCTD (SLE and SSc) since 6 years ago, often had dyspnea and backache. She was on azathioprine and NSAID. She got methotrexate before (stopped due to ILD), got cyclophosphamide i.v. once, but had allergic reaction. She was assessed MCTD with ILD, pneumonia (suspect lung TB), and got antibiotic. After a week, suddenly she had paraplegia. We thought of spondylitis TB with differential diagnosis ATM. MRI could not be done due to metal hip arthroplasty. Blood culture were positive, sputum Acid Fast Bacili were negative. Chest CT-scan showed lung mass with necrotic area at posterior segment, superior lobe of right lung, enlargement of peribronchial and subcarina lymph nodes. There were reticulonodular pattern in both lung, and right pleural effusion. There was litic lesion at VTh4 with compression at VTh5. There was soft tissue enhancement at the posterior, suspect metastatic process. We planned to do trans-thoracal lung biopsy and spine core biopsy. After 2 weeks, the dyspnea got worse and she suddenly had diffi culty in swallowing. There were rales all over the lung, blood test showed anemia and trombocytopenia. We assessed the patient with MCTD with SLE fl are, ILD, suspect lung cancer with spine metastasis. She was given pulse dose steroid for 3 days but at 2nd day she died.
