Pakistan needs more centers for training in Rheumatology-Prof.Khalid Mahmood


Childhood SLE is not uncommon but it is misdiagnosed
Pakistan needs more centers for training
in Rheumatology-Prof.Khalid Mahmood
PSR urged to start short courses in Rheumatology
for Family Physicians

Karachi: Childhood SLE is not uncommon but it is misdiagnosed. For SLE NFC is a good instrument to see the progress of the disease. We have a few rheumatologists in the country who cannot cope with the burden of the disease. Hence, we need to establish more training centers in rheumatology. The Pakistan Society of Rheumatology and medical universities should also start some short courses in Rheumatology for the family physicians so that they can diagnose and manage minor cases, identify and refer the serious cases to qualified rheumatologists. This was stated by Prof.Khalid Mahmood former Prof. of Medicine at Dow University of Health Sciences. He was summing up the presentations in one of the scientific sessions during the 23rd International Rheumatology Conference organized by PSR in Karachi from 14-17 March 2019. He chaired this session alongwith Prof. Rukhsana Zuberi an eminent medical educationist.

Continuing Prof.Khalid Mahmood said that he had interest in Rheumatology from the very beginning and my FCPS dissertation was also on Rheumatoid Arthritis. Many people who were trained with me later adopted Rheumatology as a career and they know how rheumatic disorders used to be discussed in detail during our ward rounds. I used to take synovial biopsies. In rheumatology diagnosis, he said, are criteria dependent and these criteria’s are far from perfect. They have to be revised. He was of the view that we in Pakistan need to develop our own criteria for diagnosis of rheumatic disorders as there are some features which are unknown. We need to include some features which are common in our patients in the criteria. Hence we need our own criteria for our patients rather than depending on criteria’s developed overseas. Some of the investigations for diagnosis of rheumatic diseases are very expensive, hence the treating physicians should always advice relevant investigations. Cost of investigations can also be brought down to one third of the market value, he remarked. He commended all the speakers for making excellent presentations.

 Prof. Sumaira Farman President of Pakistan Society of Rheumatology presenting a
memento to Prof.Khalid Mahmood who was invited by the organizers to chair one
of the sessions during the conference held at Karachi recently.

Earlier Dr. Saira khan spoke about macrophage activation syndrome (MAS). She discussed in detail the cause of LUPUS diagnosed in UK. This, she opined, was not a single disease but a syndrome. Typically it occurs later in life. There is delay in diagnosis which is the main cause of mortality. Different diagnostic criteria’s have been proposed. There are challenges in management as two conditions are confused i.e. JIA and SLE. So far there is no consensus on treatment strategy. Corticosteroids and cyclosporine are used. IVIG is the first biologic used in this disease. Severity of disease and seriousness of infection must be considered while planning treatment strategy. She concluded that MAS is more common and underdiagnosed. Disease specific criteria should help in diagnosis. Treatment should be individualized. HLH protocol 2004 may not be an optimal therapeutic approach.

Dr. Babar Salim from Rawalpindi was the next speaker who spoke on Cutaneous SLE. He presented his view on old and new criteria besides discussing the treatment and stated that 85% of SLE patients have skin lesions. About 25% of these patients are going to dermatologists. He also talked about isolated skin diseases. He further stated that the criteria’s are for research purposes and not diagnosis. He then mentioned about oral ulcers, discoid rash which can be localized or generalized and chronic cutaneous lesions. He then showed some interesting informative slides of lupus panniculitis, non-scaring alopecia, Lupus vascular lesions, lupus related urticaria, angioderma. He was of the view that in case of any doubt, one should do skin biopsy and correlate it with history. He also referred to the association of cutaneous lupus lesions with SLE. For management he suggested sun screen, sun protection and some pharmacological preparations.

Dr. Sumaira Farman from Lahore discussed Juvenile Systemic Lupus Erthyromatosis (SLE). She pointed out that Paediatric rheumatology was under recognized and underrepresented. Most of the Paediatric rheumatologists are in North America while most of the patients are in Asia. Developing countries have more rheumatic disease. We have a great burden of disease and very few rheumatologists. Adult rheumatologists are providing the services to treat Paediatric rheumatology cases. Continuing Dr. Sumaira Farman said that 10-20% of these diseases start at childhood age and more than 5% have the disease onset before ten years of age. Paediatric SLE is more serious in children and they also have more mortality. Use of steroids is also more in children than adults. SDI assessment is the only available disease damage index. Childhood lupus is more aggressive and involve various organs. These patients present with fever, weight loss, fatigue, renal disease and hemolytic anaemia.

She then discussed in detail the clinical manifestations of SLE and pointed out that all children have some sort of hematological involvement. Some patients are diagnosed with lupus with vasculitis and they are misdiagnosed. There is 25-75% pulmonary involvement. GI symptoms are less common. She also talked about NPSLE. Allergy and Eczema, she stated, are treated by dermatologists. These diseases are difficult to diagnose and treat. Almost 75% of these children have kidney involvement. Clinical symptoms always do not correlate with biopsy. She then presented details of a few lupus nephritis cases. For the management of these patients psychosocial support, she stated, is important. One needs to assist with school issues, ensure sun protection and proper growth. Growth failure occurs in 15% of patients due to inflammation and steroids. Puberty may also be delayed. Vaccination may have been done but these patients may need re-vaccination.

Prof. Sumaira Farman Raja

Speaking about the treatment she emphasized the importance of using optimal dose of steroids. Everybody should be on hydrochloroquine <6.5/KBW. Ensure use of sun blocks and antimalarial agents. In severe diseases, use steroids and cyclophosphamide but premature atherosclerosis should always be kept in mind. CSLE has more active disease as compared to adult hence if they are diagnosed and identified early and managed, it will ensure better outcome. More severe disease results in more infections, high rate of damage to kidneys and bone. However, overall prognosis has improved during the last few years. Now five years survival is almost 95% as compared to 30-40% in 1950s. She laid emphasis on good diagnostic plan. Patients should be called for visit and laboratory evaluation every three months. If need be treat with ACEIs and ARBs. Use Hydroxychloroquin. Tobacco industry support in our country is giving use more hypertension and diabetes, she concluded.

Dr.Khalid Ali Khan from UAE was the last speaker in this session whose presentation was on “Nail fold Capillaroscopy”. He described in detail as to which parameters should be assessed. Micro angiopathy, he said, was hall mark of connective tissue diseases. It is important to know whether we can predict the micro vascular changes taking place in the patient. It is noninvasive reliable diagnostic tool procedure. Almost 90% of patients present with RP and with micro vascular abnormalities. There is potential for monitoring disease progression.

He then discussed the treatment response in NFC in rheumatic diseases in future and highlighted scleroderma, devices used to perform this test. He was of the view that nail fold video capillary scope is the best. He also talked about early scleroderma patterns with the help of slides. Almost 33% of organs are involved while in later scleroderma pattern up to 63% organ involvement is seen. It is also important to be aware of differential diagnosis and there is a well-established role for early diagnosis, he added.

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