Paget's disease of the bone: a report of three cases.

Paget's disease is uncommon in Asians. We present 3 patients with varying manifestations. A 50 years old lady was detected to have raised alkaline phosphatase at a routine health check-up. Isoenzyme studies confirmed its bony origin. Bone scan revealed polyostotic disease. A 65 years old male patient with known Paget's disease of 6 years duration presented with pelvic girdle pain, progressive increase in alkaline phosphatase levels and worsening bone scan. A 45 years old male was admitted with chest pain. Cardiac evaluation was normal. Bone scan picked up a cough fracture in the left 7th rib and an incidental Paget's disease of the skull. All three had elevated markers of bone metabolism in the serum and urine. With monthly intravenous pamidronate there was significant improvement in the bone markers and follow-up bone scans.

NSAIDs and kidney.

NSAIDs are commonly used drugs. Even with the advent of selective COX-2 inhibitors, nephrotoxicity still remains a concern. The adverse effects of NSAIDs are mediated via inhibition of prostaglandin synthesis from arachidonic acid by non-specific blocking of the enzyme cyclooxygenase leading to vasoconstriction and reversible mild renal impairment in volume contracted states. When unopposed, this may lead to acute tubular necrosis and acute renal failure. NSAIDs also produce interstitial nephritis with or without nephrotic syndrome secondary to minimal change disease. Although this presents as acute renal failure, it can progress in some cases to chronic renal failure. Papillary necrosis has been incriminated in the development of chronic renal failure secondary to NSAIDs. In patients on long term NSAIDs without acute or chronic renal failure, subclinical renal dysfunction such as reduced creatinine clearance and impaired urine concentrating ability has been shown to be present. Although this sub-clinical dysfunction is reversible on withdrawal of NSAIDs, some reports have suggested a persistent residual dysfunction. Even with a wide range of NSAIDs at our disposal, a renal safe NSAID is yet to be discovered.

Rheumatoid arthritis and the kidney.

It is clear that kidney is involved in rheumatoid arthritis (RA) with both glomerular and tubular damage. Renal disease in RA however is usually asymptomatic and is detected only on laboratory investigations. It is often difficult to differentiate between damage due to disease activity and that due to drugs used to treat RA. Although there are a number of parameters to study renal function, these cannot be applied to day to day practice and still remain research tools. In such a scenario, it is important to periodically monitor serum creatinine and carry out urine examination so as to pick up the earliest signs of renal dysfunction.

Myelodysplasia and acute myeloid leukaemia in a case of rheumatoid arthritis with secondary amyloidosis treated with chlorambucil.

Immunosuppressive therapy related secondary haematologic malignancy is well reported. A 52 years lady with established rheumatoid arthritis developed reactive amyloidosis. This was initially treated with colchicine and cyclophosphamide and later with chlorambucil. Ten months after stopping chlorambucil she developed pancytopenia and vitamin B12 deficient megaloblastic anaemia. The pancytopenia was refractory to vitamin B12 supplements and a repeat bone marrow confirmed myelodysplasia (FABI RAEB-T). Within three weeks of this diagnosis she evolved into acute myeloid leukaemia and expired due to refractory thrombocytopenia and uncontrolled bleeding. This case stresses the need for long term follow up of RA patients treated with alkylating agents.

A longitudinal study of serum creatinine levels in patients of rheumatoid arthritis on long term NSAID therapy.

AIM: Primary: To study the effect of long term NSAID therapy on serum creatinine in patients of rheumatoid arthritis. Secondary: To study the effect of discontinuation, reduction in the dose or continuation of NSAID and of rechallenge. MATERIAL AND METHODS: Case records of RA patients with a minimum two years of follow up were analysed. Age, sex, duration of RA, type, dose and duration of NSAID and DMARD therapy, co-morbid conditions and serial serum creatinine levels were charted. RESULTS: Ninety nine case records were studied. Incidence of abnormal creatinine level (renal insufficiency) defined as rise in creatinine equal to or above the upper limit of normal was 27.7%. This rise was asymptomatic in all patients. No NSAID was particularly associated with an increased risk in renal insufficiency. The rise of serum creatinine was reversible in most patients irrespective of discontinuation or continuation of NSAID but settled at a higher level. Rechallenge resulted in rise of serum creatinine in 50% patients. Hypertension, DM, IHD and diuretics carried a higher but not statistically significant risk of renal insufficiency. CONCLUSION: NSAID-induced asymptomatic rise of creatinine in patients of RA on long term NSAIDs is common. It is mostly reversible. Regular monitoring of serum creatinine is essential.

Postoperative wound infection in patients undergoing coronary artery bypass graft surgery: A prospective study with evaluation of risk factors.

PURPOSE: Wound infection is an important cause of morbidity and occasional mortality after coronary artery bypass graft surgery (CABG). The aim of this study was to report postoperative wound infection in CABG surgery patients. METHODS: Consecutive patients undergoing CABG surgery between January 1998 and October 1999 have been studied. The exclusion criteria included, age less than 30 years, penicillin / cephalosporin allergy and associated other cardiac pathologies. The parameters studied were age, sex, obesity, hypertension, diabetes, myocardial infarction, chronic renal failure, previous surgeries, alcohol consumption, smoking, length of pre and postoperative hospital stay, antibiotic prophylaxis, MRSA screening, and duration of surgery. Wounds were classified as per modified CDC's NNIS criteria. Suspected sites of infection were cultured and antibiotic susceptibility of cultured organisms was tested. Postoperative follow up was for two months. RESULTS: Six hundred and fifteen patients were studied. Of these 116 (18.86%) developed SSI, involving sternum 75%, leg 21.3%, and forearm sites 3.44%. Organisms isolated at sternum site were MSSE, MRSA, and MRSE, at leg site E. coli and MSSE, and at forearm site MSSE and MSSA. Sternal site, obesity, diabetes mellitus and female sex were associated with significantly higher infection rates (p= 0.001). No antibiotic protocol proved more effective. SSI increased the postoperative hospital stay and the total treatment cost. CONCLUSIONS: Post CABG surgery SSI rate is high. Sternum and leg are the common infection sites. Obesity, uncontrolled diabetes mellitus and female sex are associated with higher infection rates. "Higher" antibiotics do not lower postoperative infection rates.

Arthritis in the elderly.

Joint disorders are common in the elderly population. Commonest conditions are rheumatoid arthritis, osteo-arthritis and crystal induced arthritis. Comorbid events in the elderly patients make disease management even more difficult. Some tests are needed to distinguish these conditions from more serious diseases like carcinomatous arthritis or multiple myeloma. Management strategies are usually similar to that of younger onset arthritis with dose adjustment of drugs due to underlying age related hepatic and renal dysfunctions.

Autoimmune hepatitis: a study of 50 patients.

INTRODUCTION: Autoimmune hepatitis (AIH) is a well-defined entity in the West but there are sparse Indian data on this disease. AIM: To study the clinical profile and response to treatment of Indian patients with AIH. METHODS: This is a part retrospective and part prospective study of 50 patients (median age 48 years, range 11-82; 43 women) seen between 1995 to 2001, diagnosed to have AIH as per the revised scoring system. Clinical and laboratory profile, response to treatment, and complications of treatment were analyzed. RESULTS: AIH accounted for 6% of all patients with liver disease seen during the period. The presenting symptoms were gastrointestinal in 43 and non-gastrointestinal in 7, with median symptom duration of 6 months (range 2 weeks to 40 years). Forty patients (80%) had chronic liver disease. Associated illnesses were present in 28 patients. Twenty-six patients were classified as definite and the rest as probable AIH. Forty-nine patients had Type 1 AIH. Five patients had overlap syndrome. Forty-five patients (90%) received immunosuppressive therapy. Twelve of 18 patients receiving only prednisolone and 21 of 27 patients receiving prednisolone and azathioprine combination responded. Thirteen (26%) patients had therapy-related complications (infectious 5, non infectious 8) with two treatment-related deaths. CONCLUSION: Type 1 AIH was the predominant type of AIH. The majority of patients with AIH presented with chronic liver disease. There was good response to immunosuppressive therapy. Therapy-related complications occurred in one-fourth of patients.

Ultrasonography in the diagnosis and follow-up of idiopathic inflammatory myopathies--a preliminary study.

OBJECTIVE: To study the utility of ultrasonography (US) in diagnosis and follow up of patients with idiopathic inflammatory myopathy (IIM). METHODS: High-resolution US of thigh muscles was recorded at baseline and after six months of treatment in eleven patients of early, active, untreated IIM. Parameters studied were muscle power, timed function tests (TFT), muscle enzymes, electromyography, muscle histopathology and US parameters such as echogenicity of muscle and perimysial septa count per 1 cm muscle width. RESULTS: There was a significant increase in muscle echogenicity and septa count of patients as compared to those of controls (p = 0.002 and 0.00003, respectively). These abnormalities resolved on treatment. Muscle echogenicity and perimysial septa count showed mild positive correlation with walking time, 4-step climbing and myopathic pattern on EMG. Perimysial septa count in addition correlated weakly with creatine phosphokinase, lactate dehydrogenase and muscle fibre necrosis. CONCLUSION: US seems to be a useful, non-invasive, cheap modality to monitor the disease course in IIM. Larger studies to confirm these primary findings are warranted.

Treatment of AA amyloidosis in rheumatoid arthritis.

Four patients of rheumatoid arthritis (RA) with biopsy confirmed AA amyloidosis were treated with chlorambucil. All had established but uncontrolled RA with a persistently raised ESR. Moderate (> 1 gm, < 3.5 gm/d) to nephrotic range (> 3.5 gm/d) proteinuria and a relatively well preserved renal function was noted in three patients. One patient had deranged renal function and required dialysis. On chlorambucil, there was complete recovery, partial improvement and no improvement in one patient each. The fourth patient required haemodialysis, did not tolerate chlorambucil and succumbed to the illness. Therapy with chlorambucil can benefit some patients of RA with AA amyloidosis. Leucopenia is the most important dose limiting side effect.