There are several explanations for the origin of term lupus erythematosus. “Lupus” is Latin for wolf, and "erythro" is derived from Greek for "red". All explanations originate with the reddish, butterfly-shaped malar rash that the disease classically exhibits across the nose and cheeks[1]. The term lupus has also been mentioned by Rogerius Frugardi (1230 AD) to describe the erosive facial lesions and by Giovanni Manardi (1530 AD) to denote boils and ulceration of the lower extremity [2].

Systemic lupus erythematosis (SLE) is a multisystemic chronic autoimmune disorder predominantly affecting women of child bearing age. The diverse clinical course and a high prevalence of SLE make it a major cause of morbidity and mortality especially in the younger population. The prevalence of SLE has a vast demographic variation, with a higher incidece in the Africans and Hispanics. Although the incidence of SLE has drastically increased in the past decade due to the advancement in the diagnostic techniques, the prognosis has also improved significantly with an increase in survival rate especially due to early detection [3]. The pioneer in the study of lupus erythematosus in the modern era was Edmund L. Dubois. Then came the first lupus textbook, “Dubois' Lupus Erythematosus” by Dr. Dubois, a graduate of Johns Hopkins University.

Following this, the American College of Rheumatology Research and Education Foundation took up the task of recognizing an outstanding investigator in the field of systemic lupus erythematosus every year awarding them the Edmund L. Dubois Memorial Lectureship award. It was Dr. Dubois who recognised that SLE had no classic pattern and the diagnosis had to be based on an overall view of the entire clinical picture [3]. Being a prototypic autoimmune disease, SLE has a broad spectrum of clinical manifestations in association with autoantibodies to components of the cell nucleus. Its basic pathological features are that of inflammation and blood vessel abnormalities including band or occlusive vasculopathy, vasculitis, and immune complex deposition. “Autoantibody production” is the central immunologic disturbance in SLE. These antibodies interact with a host of self-molecules found in the nucleus, cytoplasm, or surface of cells. In addition the antibodies to soluble molecules such as IgG and coagulation factors are also present in the serum of the patients with SLE.

4. Both inpatients and outpatients were included in the study.

Exclusion criteria

1. All cases that were suspicious of SLE but did not satisfy the revised American College of Rheumatology (ACR) classification criteria [5].

Data collection and analysis: The details of the history of presenting complaints, associated symptoms, past medical history, findings on physical examination, reports of the various investigations done and procedures performed along with the follow up information were obtained from the files of the patients in the medical records department. Hematological and biochemical data was retrieved by using hospital and laboratory information system.

All patients with a diagnosis of SLE were evaluated, along with the details of history, physical examination and clinical examination was performed. All the information were entered in excel sheet and then analyzed using SPSS v 20.

A total number of 127 cases of SLE were included in the present study and their clinical profile were analyzed.

Table-1: Social profile of the study subjects.

The age of the patients in the present study ranged from 6 to 63 years. The mean age of the study subjects in the present study was 29.85±12.61years. Maximum number of patients (68/127 i.e. 54%) fell in the age group of 21-40 years. The male to female ratio was 1:11.7, showing a female predominance (117/127 i.e. 92%).

effusion in 3 cases (12%) without any sign of tamponade and myocarditis in 2 cases (8%) as seen on ECHO. No significant ECG changes were observed.

Pleuropulmonary manifestations: Nineteen of the 127 cases (14.9%) had pleuropulmonary involvement at presentation. Sixteen (12.5%) had pleural effusion. Four cases had pneumonia, while two had pleuritic rub. Other manifestations included breathlessness, cough, bronchiectasis, etc.

Renal manifestations: A total of 58 cases (45%) showed renal involvement at presentation. The patients presented with symptoms such as reduced urinary output and facial puffiness. On investigation, 48 cases (82.7%) had significant protienuria and 14 cases (24%) showed deranged renal function tests (elevated serum urea and creatinine). Four cases showed urinary sediments/ casts and 12 cases (20%) had grade 1 – 2 renal parenchymal changes on ultrasound.

Lupus nephritis: Renal biopsies were done in 38 cases (65.5%) of which 36 cases (94.73%) proved to be lupus nephritis. These cases were then sub-classified into different classes of lupus nephritis and their reports are represented in chart 5. The remaining 2 biopsies were inadequate for opinion.

It was seen that majority of the cases showed the histological pattern of diffuse proliferative glomerulonephritis hence belonged to lupus nephritis – class 4 (18/38 i.e. 47.38%), followed by focal segmental glomerulo nephritis – class 3 (7/38 i.e., 18.42%), mesangio proliferative glomerulo nephritis – class 2 (5/38 i.e. 13.15%), membranous glomerulonephritis – class 5 (4/38 = 10.52%) and minimal change disease – class 1 (2/38 = 5.5%).

Organomegaly & ascitis: Hepatomegaly was a common finding, seen in 42/127(33.1%) cases, followed by splenomegaly which was seen in 21/127 (16.5%). Ascitis was observed in 16 cases (12.6%).

Neuropsychiatric manifestations: Twenty two patients (17.33%) presented with neurological symptoms, which included altered sensorium in 6 cases, loss of power in 5 cases, seizures (new onset) in 3 cases, depression in 2 cases and other manifestations like hemiparesis, neuropathy, involuntary movements and dysarthria (1 case each). Imaging was done in 8 cases which showed hydrocephalus in 2 cases, meningeal enhancement in 2 cases, cerebral atrophy in one case and acute lacunar infarcts in one case.

Of these 13 cases two were diagnosed with type 2 diabetes mellitus and rest 11 cases were steroid induced diabetes. Eleven of the 127 patients were hypertensive, of which 7 cases were old cases and rest 4 were newly diagnosed, all of whom were females. Of the 4 cases that were newly diagnosed, 3 had lupus nephritis.

This was a retrospective-prospective study of the clinical and hematological profile of patients with systemic lupus erythematosus at a tertiary medical centre for a period of 3 years. The clinical manifes-tations of all the cases in comparison with other studies are depicted in the below.

SLE being a disease predominantly effecting the female population, a female preponderance in the patients was an expected result in the study. The male of female ratio was observed to be 1:11.7, and was in concordance with other studies by Mok et al[6], Sasidharan et al [7], Voulgareli et al [8], Fathi et al [9] got a relatively higher M:F of 1:44.

Similarly, the median age of presentation at 29.5 years in the present study, is also expected as SLE is a disease affecting the women of childbearing age group and hence is in concordance with the other studies[10], [11]. However in the study by Voulgareli et al [8], the mean age was 42yrs, which is much higher than the present study.

Unlike the other studies conducted by authors like Mok et al [6](82%), Binoy et al [12] (89.3%), Aleem et al [11] (80.4%) and Paudyal et al[13] (93%) where arthritis was the most common presenting symptom among the patients, followed by fever; the present study reported fever as the most common presenting symptom among the patients occurring in 72.4% of the cases, followed by skin manifestations (62.2%) and arthritis which was seen in 55.1% of the cases.

This was in concordance with a study by Agarwal et al [10] who also reported fever as the most common presenting symptom accounting to 82.8%. The incidence of arthritis in the present study was in concordance with the studies by Voulgareli et al[8] (50%), Fathi et al [9] (60%), Agarwal et al [10] (52.9%) and Kosaraj et al [14](64.58%).

Dermatological manifestations occurred in 62.2% of the cases in the present study and closely matched with the study conducted by Aleem et al [11]

(10.5%) and Swaak et al [17] (46%).

Among the organ involvement, the kidney was the most frequent organ involved inthe patients of the present study, with 45% of the patients presenting with renal manifestations, which is in concordance with other studies by Mok et al [6], Sasidharan et al[7], Voulgareli et al [8], Swaak et al[17], Agarwal et al [10], Aleem et al [11], and Fathi et al [9] who also reported kidney as the most frequent organ involved accounting to about 27%, 23%, 12.5%, 87.03%,33.3%, 56.7%, 26%, 20.83% respectively.

The renal manifestations included protienuria, deranged RFT and urinary casts and sediments which were seen in 37.7%, 88.88% and 9.4% of the cases respectively. This is in concordance with a study by Agarwal et al[10] who reported 7.15% cases of protienuria, 24.1% cases of deranged RFT and urinary casts and sediments in 3.4% cases.

Among the 38 study patients who underwent renal biopsy, 36 were confirmed to be lupus nephritis. Of the 36 cases of lupus nephritis, most of the cases were classified as Class IV (18/38 = 47.38%), and Class III LN (7/38 = 18.42%), followed by Class II (5/38 = 13.15%), and Class V (4/38 = 10.52%). Only 2 cases (5.5%) showed minimal change disease – class 1.

This is to a certain extent is similar to the series by Mok et al[6] and Saigal et al[15], who found the overall frequency of Class II, III, IV and V is 1%, 20%, 65%, 14% and 0%, 5%, 90%, 5% respectively.

Pleuropulmonary manifestations were seen in 14.9% of the cases, which included pleural effusion, pleuritis and pneumonitis, which is in concordance with the study by Saigal et al [15], Fathi et al [9], Aleem et al [11] and Kosaraj et al[14] who reported 11.7%, 15.16% 15.9% and 12.5% of the cases respectively, while other studies by Sasidharan et al [7] and Binoy et al [12] showed a lower frequency of pulmonary manifestations (3% and 8% respectively), and a higher incidence of 20% was seen in the study by Paudyal et al [13].

Among the pleuropulmonary manifestations documented in the present study, pleural effusion was the most common manifestation which in concordance with the other studies as well.

Cardiovascular manifestations were seen in 18.8% of the cases in the present study, which included valvular deformities, pericarditis, myocarditis, and pericardial effusion.

The clinical features of the SLE patients seen in the present study cannot be generalized to the whole community and hence it cannot be taken as a true representative of the clinical profile of SLE Patients. The follow-up of these SLE patients was not available; hence, the change in clinical profile of these patients as the disease progress was not observed.

SLE is a multisystem disorder affecting predominantly young females. Polyarthritis was the most common clinical feature. Incidence of fever was the most common symptoms followed by dermatological manifestation. Kidney was the major system involved among majority of the cases followed by cardiac, Neuropyschiatric and Pulmonary. Hence all the cases of SLE should be evaluated in detail for the involvement of the all the systems and its manifestations in detail to improve the overall condition of the patients.

SLE being a multi-systemic disorder presents with a large spectrum of symptoms, a complete evaluation of the patient including detailed hematological as well as biochemical and immunological studies should be done for every patient to detect early lesions and hence improving the prognosis.

The data collection and analysis was performed by Dr. Akshatha N, The review of articles, introduction, references and Manuscript preparation was done by both Dr. Akshatha N and Dr. Shwetha Patil. The final proof reading was done by Dr. Shwetha Patil.