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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 1994  |  Volume : 5  |  Issue : 1  |  Page : 33-36
Interrelation between Systemic Lupus Erythematosus and Thrombotic Thrombocytopenic Purpura

1 Renal Unit, Dubai Hospital, Dubai, United Arab Emirates
2 Hematology Unit, Rashid Hospital, Dubai, United Arab Emirates

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Features suggestive of thrombotic thrombocytopenic purpura (TTP) are known to occur in patients with systemic lupus erythematosus (SLE). We report a patient who had TTP which resolved with plasma exchange and immunosuppression, but presented three years later with features of SLE. The diagnosis satisfied all the required criteria in both instances. The interrelationship between the two conditions is discussed.

Keywords: SLE, TTP, Plasma exchange, Immunosuppression, Pregnancy.

How to cite this article:
Suleiman ME, Al-Rukhaimi MA, Railey MH, Raizada SN, Fernandes HA, Marashi MM. Interrelation between Systemic Lupus Erythematosus and Thrombotic Thrombocytopenic Purpura. Saudi J Kidney Dis Transpl 1994;5:33-6

How to cite this URL:
Suleiman ME, Al-Rukhaimi MA, Railey MH, Raizada SN, Fernandes HA, Marashi MM. Interrelation between Systemic Lupus Erythematosus and Thrombotic Thrombocytopenic Purpura. Saudi J Kidney Dis Transpl [serial online] 1994 [cited 2022 Oct 3];5:33-6. Available from: https://www.sjkdt.org/text.asp?1994/5/1/33/41359

   Introduction Top

Several cases with unequivocal interrelationship between Systemic Lupus Erythematosus (SLE) and Thrombotic Thrombocytopenic Purpura (TTP) have been reported and in most cases SLE has preceded or occurred concurrently with TTP [1],[2],[3],[4],[5],[6] . In this report we describe a patient who was diagnosed to have TTP in 1988 and presented with SLE three years later. In both instances, the diagnosis was unquestionable and satisfied all the required criteria. To the best of our knowledge this is the second reported case in which TTP preceded SLE.

   Case Summary Top

On 11th June, 1988 an unmarried, 18 year old female university student was referred to our hospital from a nearby maternity hospital where she was admitted a day earlier with a five day history of giddiness, abdominal pain and excessive vaginal bleeding. She was found to be anemic with hemoglobin (Hb) of 5.4 gm/dl and had been given three units of blood transfusion. The pregnancy test was negative. In addition, abdominal sonography demonstrated fluid in the peritoneal space and on the suspicion of hemoperitoneum, she had undergone exploratory laparotomy. About 500 ml of sero-sanguinous fluid was removed from the peritoneal space. The right ovary was found to be multicystic and congested and there were petechial hemorrhages on the mesentery and bowel walls. The liver, spleen and appendix were found to be normal. At this time, a review of her investigations revealed that platelets were 12 x 109 /I and fibrin degradation products (FDP) were raised in the serum (40 mg/1). Serum fibrinogen was 2.17 g/1. A tentative diagnosis of a coagulopathy was made and the patient was referred to Dubai Hospital.

The patient had no history of bruises, fever or sore throat in the recent past. There was no history of any drug ingestion during the preceding four weeks. Her menstrual cycles had been normal. Clinical examination revealed a well looking, afebrile patient with mild pallor. There was no jaundice. There were purpuric spots on the limbs, but no bruises. The throat and buccal cavity showed no bleeding or purpuric spots. Cardiovascular and respiratory systems were normal. The abdomen was distended and had generalized tenderness. It was not possible to feel the liver or spleen. Bowel sounds were absent. Central Nervous System (CNS) examination was normal. Fundi showed no hemorrhages. There was still moderate vaginal bleeding and about 300 ml of sero-sanguinous fluid had drained from the peritoneal space over the preceeding 12 hours.

Investigations revealed normal prothrombin time (PT) and partial thromboplastin time (PTT), serum fibrinogen of 2.6 gm/L and serum FDP of 40 mg/L. The platelet count had risen to 42 x 109/l. The peripheral blood film showed schistocytes, a few microcytes and nucleated red blood cells. Bone marrow aspirate revealed an active marrow with normal megakaryocytes. At this juncture a diagnosis of microangiopathic hemolytic anemia was entertained with TTP and disseminated intravascular coagulation (DIC) as the differential diagnoses. As the patient started to run a low grade pyrexia, antibiotics were commenced empirically after appropriate specimens were sent for culture. On the next day, crackles were noted over the right lower chest and chest x-ray revealed a right sided pleural effusion. By evening, the patient developed transient weakness of the right side of the body which lasted for about 10 minutes. Coagulation profile at this time showed normal PT and PTT, serum fibrinogen of 2.6 gm/1 and serum FDP of more than 40 mg/1 but less than 80 mg/L. The clinical picture was then clearly that of TTP and treatment with aspirin, dipyridamole, steroids and plasma exchange was instituted. She developed two further episodes of transient weakness on the right side of the body. These episodes were associated with right facial palsy. The platelet count which had dropped once more to 12 x 109/l prior to instituting plasma exchange, showed improvement within 24 hours and rose to 51 x 109/L. During this period she had maintained a reasonable urine output and the blood urea ranged between 14.3 and 21.5 mmol/L.

However, over the next two days, the patient's clinical condition deteriorated and she became anuric. She developed pedal edema and jugular venous pressure was elevated. She also had tachycardia with pulse rate of 110/minute and dyspnea with a respiratory rate of 48/minute. Chest x-ray showed features of pulmonary edema. There were however no neurological deficits and no further episodes of weakness. She developed pain in the elbows which was diagnosed to be due to hemoarthrosis. It was concluded that she had non-responsive TTP affecting the kidneys, the CNS, the heart and lungs. At this juncture, the dose of steroids was increased and sulphinpyrazone was added to the antiplatelet regimen. Over the next 36 hours she showed marked improvement both clinically and hematologically. However, acute renal failure persisted and she was managed by haemodialysis till her urine output improved. Plasma exchange was subsequently phased out and finally discontinued on 26th June 1988.

Investigations performed subsequently disclosed normal sized kidneys with no focal pathology by renal ultrasonography. Platelet antibodies, rheumatoid arthritis (RA) factor, lupus erythematosus (LE) cells, anti-nuclear factor (ANF) and antibody to double stranded DNA (anti-dsDNA) were found to be negative. Immunoglobulins were normal. She was discharged well from the hospital on 20th July, 1988, and was thereafter seen as an out patient 10 days later. She looked well and had gained one and a half kilogram of weight. Blood urea had dropped to 1.79 mmol/L and the electrolyte profile was recorded as normal. Full blood count showed hemoglobin 9.4 gm/dl, platelet count 282 x 109/l, WBC 5.4 x 109/l with a normal differential count and peripheral blood smear showed only occasional schistocytes. She was followed up regularly as an outpatient with close hematological and biochemical monitoring. Dipyridamole and aspirin in a dose of 75 mg and 100 mg once daily respectively were continued. All parameters remained normal. In April, 1989, she got married. Her visits to our clirlic became more spaced. In October, 1990, she conceived but had an abortion at six weeks of gestation.

During a routine visit in June 1991, three years after the initial presentation with TTP, her hemoglobin was found to be 8.8 gm/dl. The anemia was normochromic in nature with low serum iron and transferrin and high ferritin levels. ESR was 140 mm in the first hour. Serum urea and creatinine levels were 8.83 mmol/L and 97.2 u.mol/L respectively while the creatinine clearance was found to be 54 ml/minute. Many LE cells were seen on the peripheral film and RA factor, C-reactive protein and ANF were all positive. Anti-dsDNA antibodies became very strongly positive at a titre of 1:1000 a few days later. A diagnosis of SLE was then made. Further laboratory reports disclosed a high globulin of 58 gm/1 and low albumin of 29 gm/L, total bilirubin 35 umol/L, alkaline phosphatase 169 IU/L, AST 33 U/L, ALT 25 U/L, GGT 23 U/L, amylase 54 U/L, phosphate 1.32 mmo/L, calcium 2.02 mmol/L, creatinine 105 μmol/L and uric acid 510 mmol/1. Immunoglobulin studies showed high IgG (40.2 gm/L) but IgA and IgM were normal, 2.89 gm/L and2.04 gm/1 respectively. Both C3 and C4 were low at 0.35 gm/1 and 0.67 gm/L respectively. Haptoglobin was normal at 1.23 gm/L. A percutaneous kidney biopsy was performed under local anesthesia. Light microscopy showed an adequate biopsy with 24 glomeruli showing global mesangial matrix increase and cell proliferation as well as necrosis. Wire loop lesions were also seen. Four glomeruli were completely hyalinised. The interstitium and tubules showed patchy chronic inflammatory cellular infiltrates, fibrosis and atrophy. The overall picture was that of diffuse proliferative GN ranking Class IV by WHO classification of lupus nephritis.

Treatment was initiated with prednisolone and cyclosporine. She was commenced on 30 mg prednisolone orally daily which had to be reduced to 20 mg because of poor tolerance to the drug. Cyclosporine was administered orally, in a dose of 100 mg once daily. A couple of days later she felt better and pain, fever and swelling subsided. However, the liver function tests showed slight derangement. Levels of AST and ALT had risen to 53 and 108 U/L respectively and GGT went up to, 136 U/L. Serum albumin was 31 gm/L.

While on treatment, a dramatic improvement in kidney function occurred and proteinuria disappeared completely. The blood pressure became normal needing no pharmacological control. She became asymptomatic, but cyclosporine and prednisolone were maintained until the creatinine clearance returned to normal. They were then tapered and discontinued after a period of one month. Aspirin was however continued.

She remained asymptomatic until a few months later when she presented with features consistent with active lupus characterized by impaired renal function and positive anticardiolipin antibodies. It was decided to commence her on pulse cyclophosphamide/ prednisolone regimen. Six injections were given of the former, the first three spaced on monthly basis while the last three were administered every 6 weeks. The whole period was covered with low dose daily prednisolone. Following this, her creatinine clearance returned to normal, the hematological indices were maintained and she was asymptomatic. As she was very anxious to become pregnant she was permitted to do so, six months after the last cyclophosphamide injection. She had an uneventful antenatal period and had full term normal delivery of a normal baby followed by an uneventful postnatal course. A short course of prednisolone was given for a few days starting on the day of delivery. She was all the time maintained on aspirin at a dose of 100 mg once daily. Her biochemical and hematological parameters remained normal. Blood pressure also remained normal.

   Discussion Top

TTP is an uncommon clinical situation which is characterized by the pentad of fever, thrombocytopenia, renal involvement, hemolytic anemia and fluctuating neurological deficits. The major criteria are microangiopathic anemia, thrombocytopenia and neurological deficits while the minor are fever, renal involvement and histological findings of thrombosis. Some authorities demand the presence of at least two of the major criteria or three minor criteria for a convincing diagnosis [6] . This syndrome is believed to be initiated by endothelial cell insult, mechanism of which is yet unknown [7] . Prostacyline production and/or release is affected negatively and prostacycline stabilizing factor as well as platelet aggregating factor inhibitor are reduced as well. Those lead to platelet aggregation and thrombus formation [7] .

The association between SLE and TTP cannot be fortuitous. It is possible that, in both, there are antibodies directed against prostacycline, prostacycline stabilizing factor, platelet aggregating factor inhibitor and capsular endothelial cells [8],[9] . In our patient, one may argue that it was possible that the diagnosis of SLE was delayed and that the previous episodes of anemia were pointers towards that diagnosis. However, at the time of presentation with severe TTP, the criteria for the diagnosis of SLE were not satisfied.

   References Top

1.Becker RC, Guilian M, Weik JK. Thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus. Cleve Clin Q 1985;52:409-15.  Back to cited text no. 1    
2.Fox DA, Faix JD, Coblyn J, Fraser P, Smith B, Weinblatt ME. Thrombotic thrombocytopenic purpura and systemic lupus erythematosus. Ann Rheum Dis 1986;45:319-22.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Levine S, Shearn MA. Thrombotic thrombocytopenic purpura and systemic lupus erythematosus. Arch Intern Med 1964;113:826-36.  Back to cited text no. 3  [PUBMED]  
4.Siegel BM, Friedman IA, Kessler S, Schwartz SO. Thrombotic thrombocytopenic purpura and lupus erythematosus. Ann Intern Med 1957;47: 1022-9.  Back to cited text no. 4  [PUBMED]  
5.Ramkisson RA. Thrombotic thrombocytopenic purpura and systemic lupus erythematosus. Calif Med 1966;104:212-4.  Back to cited text no. 5    
6.Fahad Al-Mohareb, Harakati M, Al- Momen A, et al. Thrombotic thrombocytopenic purpura: experience at King Khalid University Hospital. Saudi Kidney Dis Transplant Bull 1992;3:14-22.  Back to cited text no. 6    
7.Jaffe EA, Nachman RL, Merskey C. Thrombotic thrombocytopenic purpura. A coagulation parameter in twelve patients. J Hemat 1973;42(4): 499-509.  Back to cited text no. 7    
8.Amorosi EL, Ultmann JE. Thrombotic thrombocytopenic purpura: report of 16 cases and review of the literature. Medicine 1966;45:139-59.  Back to cited text no. 8    
9.Umlas J, Kaiser J. Thrombotic thrombocytopenic purpura (TTP). A disease or a syndrome? Am J Med 1970;49:723-8.  Back to cited text no. 9    

Correspondence Address:
Mustafa Nur El Huda Suleiman
Renal Unit, Dubai Hospital, Dubai
United Arab Emirates
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Source of Support: None, Conflict of Interest: None

PMID: 18583760

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