Macrophage activation syndrome: experience from a tertiary referral centre.

Over a period of 3 years, 13 patients with Macrophage Activation Syndrome were seen. Most had underlying connective tissue disease or malignancy. High-grade fever, cytopenia and elevated transaminases were the common presenting manifestations. Elevated LDH and ferritin were characteristic. Due to low index of suspicion the diagnosis was delayed in majority of cases. Five of the 13 expired. Macrophage Activation Syndrome is associated with a high mortality and should be considered in the differential diagnosis of unexplained pancytopenia in-patients with connective tissue disease and malignancy.

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.

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.

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.

Pulmonary nocardial infection and pseudomonas infection of the tongue in a patient with dermatomyositis.

Opportunistic infections in immunocompromised patients are common. We report the case of a 63 year old female patient with dermatomyositis who while on oral steroids developed nocardial infection of the lung and pseudomonas infection of the tongue simultaneously. Nocardial infections are not very commonly seen in patients with dermatomyositis. Pseudomonas infection of the tongue is a rarity. We report this case for its rarity as regards the type and site of infections and review the relevant literature.

Accidental self-injection with Freund's complete adjuvant.

The case of a research worker who accidentally injected herself with Freund's Complete Adjuvant (FCA) in the right forearm is reported. This resulted in a serious granulomatous inflammation of the hand an forearm. She was treated with corticosteroids and chloroquine with good result. The course of this reaction and the hazards of injection with FCA are emphasised and the relevant literature reviewed.

Unilateral inter-nuclear ophthalmoplegia in systemic lupus erythematosus.

We report a case of a 14 year old girl with SLE who developed neurological involvement in the form of posterior internuclear ophthalmoplegia (pINO). An MRI showed lesion involving pons which corroborated with the pINO.

Neuroleptic malignant syndrome occurring in a suspected case of systemic lupus erythematosus.

The case of a 40 year old female patient of SLE who developed fatal neuroleptic malignant syndrome following administration of serenase (haloperidol) is reported. Relevant literature has been reviewed.

Tuberculosis in patients with systemic lupus erythematosus.

In a retrospective analysis of 146 systemic lupus erythematosus (SLE) patients seen over a 5 year period, 17 patients of tuberculosis (TB) were identified yielding a prevalence rate of 11.6%. The median duration of SLE was 12 months (range 2-96 months) and 12/17 patients had disease activity score of more than five. The median duration of steroid treatment was 12 months (range 0-96 months) and the median cummulative dose of steroid was 7.75 gms (range 0-22.1 gms). Pulmonary TB (miliary-5, nonmiliary infiltrates-7 and pleural effusion-2) was the commonest type and there was an average diagnostic delay of approximately 1 month. While, majority of the patients responded adequately to treatment, 1 patient had a relapse and 1 expired due to a combination of active lupus and disseminated TB. Only 1 patient had received prophylactic isoniazid.

Evaluation of the knee pathology by ultrasonography.

Fourteen patients undergoing total knee replacement for the arthritic conditions of the knee were evaluated by ultrasonography (USG) prior to surgery. USG findings (synovium, fluid, femoral cartilage, cyst and loose bodies) were compared with surgical findings. USG and surgical findings matched for synovial pathology in 57%, for presence fluid in 71% and for cartilage damage in 45%. Loose bodies and cyst present in one patient was not picked up by USG. Baker's cyst was detected in one patient.

The spectrum of chronic autoimmune hepatitis.

Autoimmune hepatitis (AIH) is characterised by the presence of periportal hepatitis coupled with the presence of autoantibodies in the serum. We report our experience with 10 cases (females--8, males--2) who presented to the rheumatology clinic with either articular or extra-articular manifestations. Three patients (1 SLE, 1 Sjogren's and 1 RA), satisfied the criteria for an underlying rheumatic disease (secondary AIH) while, others had primary AIH. Median duration of hepatic involvement was 6 months and the varied presentations were noted. Articular disease ranged from arthralgias, palindromic arthritis to persistent non-erosive/non-deforming arthritis (Jaccoud's arthritis). Autoimmune thrombocytopenia was seen in 2 and autoimmune hypo and hyperthyroidism were seen in 3 patients each. Anti-nuclear antibody was positive in 9/10 (6 with speckled pattern and 3 with homogenous pattern) and anti-mitochondrial antibodies were positive in three. Hepatitis C virus (HCV) markers were positive in 1, who probably had viral hepatitis with dominant autoimmune features. All have been started on steroids (5 patients--1 mg/kg dose, 1 patient--0.5 mg/kg dose and 3 patients--0.25 mg/kg dose). The HCV positive patient was on a low dose steroid (0.25 mg/kg) and interferron treatment was contemplated before she was lost to follow up. Four patients are also on azathioprine in the dose of 2 mg/kg/day. Of the 6 patients who are under regular follow up, the liver parameters have normalised in 5 and one showed hypoalbuminaemia with normal enzyme levels at the last follow up.