UNUSUAL PRESENTATIONS OF SYSTEMIC LUPUS ERYTHEMATOSUS

Transcription

1 UNUSUAL PRESENTATIONS OF SYSTEMIC LUPUS ERYTHEMATOSUS Presenter- Dr. Meghana B S Prof Dr. NAGARAJA B S Prof Dr. NIRMALA A C Dr. SIVARANJANI H Dr. B C PRAKASH Dr. MUMTAZ ALI KHAN

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3 A 60 year old lady, k/c/o Hypertension, Hypothyroidism and Coronary artery disease presented with easy fatiguability - 1 week yellowish discoloration of the eyes-1 week No evidence of bleeding manifestation

4 Treatment history: Tab. Ecospirin 75mg Tab. Atoravstatin 20mg Tab. Metoprolol 50mg Tab. Thyronorm 100mcg Anti VEGF injections one dose for age related macular degeneration Vitamin and minerals supplements

5 ON EXAMINATION Pallor ++ Icterus + Bilateral pitting pedal edema + Jugular venous pressure raised

6 Abdomen: Mild splenomegaly + Respiratory system: B/L basal crepitations present CNS and Cardiovascular System: Normal

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8 Labarotory findings Complete hemogram: Hb 6.3gm% MCV 136 fl Total count 7300cells/cumm Platelet count 2.32L cells/cumm Peripheral smear- dimorphic anemia, anisocytosis, target cells, polychromatophils, nrbcs and spherocytes (50%)

9 Corrected reticulocyte count 7.2% LDH- 809 IU/L LFT: TB- 8.2mg/dl IB-7.1mg/dl DB-1.1mg/dl T.P-9.0g/dl Albumin- 3.6g/dl SGOT/SGPT- 41/07

10 RFT S. urea-6.97 mg/dl S. Creatinine-0.5 mg/dl RBS- 115mg/dl VITAMIN B pg/ml FOLIC ACID ng/ml TSH μIU/mL Iron profile- Normal

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12 Direct COOMB S test Positive ANA screening Positive ANA Profile- Ab to SS-A 3+ Ab to SS-B 3+ Ab to dsdna 3+ Ab to Ro Ab to Sm negative

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14 Urine routine Protein 1+ RBCs- 4RBC s/hpf WBC- 3Cells /HPF Epithelial Cells- 2/HPF Casts- NIL Renal biopsy was advised, suspecting lupus nephritis

15 Renal biopsy Class III. Segmental endocapillary proliferation with karryorhextic debri and leukocyte infiltration

16 Patient was started on tab. Wysolone 60mg on tapering doses and tab. Azathioprine 50mg OD. On subsequent follow up after 1 month Patient s clinical and biochemical parameters improved.

17 Hb-12.3 TC-7800 Platelets-2L PS- NORMOCYTIC NORMOCHROMIC BLOOD PICTURE LFT- Normal RFT- 20/0.8 TSH- 0.47

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19 38 year old lady k/c/o hypothyroidism on treatment Presented with menorrhagia, easy fatiguability and swelling of both lower limbs since 7days Past history: 4 admissions for blood transfusion

20 ON EXAMINATION Pallor ++ Icterus + Hyperpigmented rashes over both cheeks B/L pitting pedal edema present Jvp elevated

21 CVS: S1, S2 normal, hemic murmurs present Abdomen: Moderate hepatosplenomegaly present CNS and Respiratory system: Normal

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23 INVESTIGATIONS Hb- 5.3gm/dl Total count 11,000 Platelets cells/cumm Peripheral smear- Dimorphic anemia with thrombocytopenia Corrected reticulocyte count 2.47% LFT; TB:3.9mg/dl IB:2.9mg/dl DB:1.0mg/dl Urine routine- Normal TSH μIU/ml

24 Iron profile normal Vitamin B12 307pg/ml USG abdomen moderate hepatosplenomegaly

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26 Direct coombs test positive. ANA positive ANA profile anti smith antibody positive.

27 Patient was started on Tab. Wysolone 60mg per day On follow up after one month, patient still had symptoms Lab findings showed- Hb- 7.6g/dl Peripheral smear- Microcytic hypochromic anemia LFT- TB: 2.1mg/dl IB: 1.9mg/dl

28 She was further evaluated Iron profile- normal Bone marrow biopsy hypercellular marrow with erythroid hyperplasia Haemoglobin electrophoresis showed beta thalassemia trait

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30 Blood transfusion was done. Her Haemoglobin and MCV was maintained Patient is symptomatically better on subsequent follow up.

31 CASE 1 CASE 2 Age /Sex 60yr female 38yr female Comorbidities k/c/o hypothyroidism k/c/o hypothyroidism E/o Bleeding manifestation Past history of blood transfusion Features of anemia in failure Absent Absent Present Present Present Present On evaluation Hemolytic blood picture Hemolytic blood picture COOMBS test Positive Positive ANA Anti dsdna, antiro, anti LA, anti RO52 Anti Sm antibody Treatment Tab. Wysolone 60mg Tab. Wysolone 60mg On Follow up Patient improved Symptoms persisted Additional features Lupus nephritis Beta thalassemia trait

32 DISCUSSION Systemic lupus erythematosus is an autoimmune disease Affects multiple organs through autoantibodies and immune complexes. Hematologic manifestations are commonly identified in SLE

33 However SLE presenting as hemolytic anemia is quite rare Gomenzo et al., described Autoimmune hemolytic anemia (AIHA) in only 4% of adultonset SLE cases.

34 AIHA may be the sole presenting sign of the disease in many until several years. Patients with AIHA and anti-dsdna antibodies exhibit an increased risk of renal disease

35 Also coexistence of SLE with other haemolytic anaemia has been reported rarely. Castellino et al, 17 subjects out of 177 SLE subjects had beta thalassaemia (all female, mean age 53 years, range 20 88).

36 He concluded that beta thalassemia is less common in patients with SLE but the symptoms of SLE were more severe if they coexist Whenever the transfusion requirement increases in a patient with thalassemia trait, it warrants to search for an associated condition.

37 Recurrences during the course of SLE are rare, with an estimated recurrence rate of 3% to 5% per year. The prognosis of hemolytic anemia is uncertain as only two studies, Ward et al., and Drenkard et al., reported that hemolytic anemia is independently not considered a predictor of poor prognosis.

38 TAKE HOME MESSAGE SLE may progress rapidly and requires prompt early diagnosis and treatment Testing for antinuclear antibodies is warranted in patients with unexplained autoimmune hemolytic anemia. Identification of other causes of non responders before labelling as refractory AIHA. There are no high-quality data to guide second-line treatment

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