Manohar Lal S. Dawani ( Department of Nephrourology, Jinnab Postgraduate Medical Centre, Karachi. )
Muzamil H. Osmani ( Department of Nephrourology, Jinnab Postgraduate Medical Centre, Karachi. )
Masood A. Shaikh ( Department of Nephrourology, Jinnab Postgraduate Medical Centre, Karachi. )
S. Ali Jaffar Naqvi ( Department of Nephrourology, Jinnab Postgraduate Medical Centre, Karachi. )
Twenty-seven patients (24 females and 3 males ) met four or more of the clinical criteria of Cohen et al.5 Their ages at presentation ranged from 10 to 54 years with a mean age of 24.4 years (Fig).
Nine patients presented with nephrotic syndrome, 7 had proteinuria, 6 with acute nephritis and 5 with chronic renal failure.
The main clinical features: oedema, arthralgia, haematuria and anaemia were seen in more than 50% of patients (Table I).
Ninety-two percent had proteinuria, 89% positive ANA, 74% positive LE cell and anti DNA antibodies were raised in all the patients (15/15). Forty-eight percent had azotaemia (Table II).
Renal biopsy was done in 20 patients; in others it was not done either because of raised BUN or refusal of the patients. In one case biopsy could not be done because of pregnancy. The histological findings are shown in Table III.
Patients have been followed from one month to six years. Twenty were followed from 1 to 9 months and 7 from 15 months to 6 years. More than one-third of the patients (11/27) died. Two died of chronic renal failure (CRF), 5 of septicaemia, 2 of hepatic coma and one each due to pulmonary oedema and neurological involvement.
The patients in remission (4) on biopsy showed either focal proliferative, focal segmental glomerulitis or membranous nephropathy. Four patients had active disease i.e. positive ANA, LE cell or raised titre or anti DNA antibodies. One had chronic nephritis, one showed minimal change and in two others biopsy could not be done one due to pregnancy and the other refused. Two patients relapsed, one with focal nephritis after 3 years and the other with minimal change after 4 years.
Six patients were lost to follow-up. One was from Saudi Arabia and had chronic nephritis and an other from Sri Lanka, who had membranous nephropathy. One was a docter who didn’t believe that she has SLE. She had normal renal functions and did not agree for renal biopsy, in one it was not done because of a high BUN. One patient with chronic nephritis was lost to follow-up after one year and one with chronic nephritis never returned after biopsy.
Most of the patients (18) were treated with combined prednisolone and cyclophospha mide. Seven patients were given prednisolone (40-60mg) alone and only one patient was treated with cyclophosphamide only. One patient was on haemodialysis also.
Side effects were those usually observed with corticosteroids or immunosupressive drugs. Patients treated with steroids were temporarily cushingoid. One patient developed avascular necrosis of hip and another collapse of a lumbar vertebra. Few patients developed hypertension and psychosis developed in two cases. One patient suffered an attack of Herpez zoster during treatment with cyclophospharnide. Cyclophosphamide also caused alopecia, marked blackening of knuckles, nails and dark complexion generally. In few cases transient leukopaenia was also observed.
Lupus nephritis in the series was more common in females below the age of 30 years and the clinical features were similar to those of lupus asawhole.11,12
Most of the patients presented with nephrotic syndrome or proteinuria. The frequency was less than that reported by cameron et al.13 but similar to other reported series.14,15
A correlation between the presence of both complement fixing antibodies to DNA, low levels of serum compliment (C3) and active renal disease has also been observed16 in this series C3 levels returned to normal as the disease activity subsided.
The treatment of SLE remains one of the controversial topics17,22 W Most of the patients were given combined prednisolone and cyclophosphamide and only 7 patients were treated with large dose of prednisolone. One patient was given cyclophosphamide only. It cannot be said that combination is better than prednisolone alone or vice versa, as two of each group were in remission and also one each of the two with relapse were on prednisolone or combination therapy.
No serious side effects of cyclophosphamide i.e. haemorrhagic cystitis or development of malignancy were seen.
The pattern of death in this series was quite different.13-23 The causes of death being renal failure/septicaemia, and myocardial infarction, whereas, in this series sepsis was the most common cause. It is not clear whether this relates to the disease itself, or the use of steroids and immunosupressive drugs. Only one patient took a fulminant course and died within one month and others from 6 months to 4 years after diagnosis. Renal failure in an infrequent cause of death in SLE11,24,26. Only two patients in the series died of renal failure.
One patient was pregnant at presentation, who delivered a live baby at full term though an increased foetal loss occurs in lupus27 Patients may have an exacerbation of the disease, during pregnancy, a spontaneous abortion or may suffer from pre-eclampsia.
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