Clinical Profile of Acute Rheumatic Fever Patients in a Tertiary Care Institute in Present Era.

INTRODUCTION: Rheumatic heart disease (RHD) is still a common cause of morbidity and mortality in India. Rheumatic fever commonly occurs between 5-15 yrs of age. Rheumatic activity is a common cause of deterioration of symptoms in a stable or undiagnosed case of RHD. MATERIAL AND METHODS: 41 consecutive patients of acute rheumatic activity were studied. Presenting symptoms were noted. Physical examination was done for major and minor Jone's criteria. Detailed cardiac examination was done. All underwent echocardiography. RESULTS: Mean age was 14.80 ± 7.01 yrs, 26 were female and 15 were male. Joint pain was the commonest present complaint (29 patients), followed by fever in 17 patients, 32 had carditis, 26 had arthritis, 13 had subcutaneous nodules, 7 had chorea and one had erythema marginatum. In patients with carditis, 30 had mitral regurgitation (MR) only, 15 had MR with aortic regurgitation (AR). Out of 32 patients with carditis, 10 patients did not have any clinical evidence of carditis and were detected by echocardiography only. Six patients were known cases of rheumatic fever but had stopped penicillin prophylaxis. CONCLUSION: Commonest complaint in patients with rheumatic fever was joint pain followed by fever. In patients with carditis, all had MR, with 1/3rd of these patients having associated AR. 1/3rd of patients with carditis were detected by echo only and therefore, echo should be included in diagnostic criteria for acute rheumatic fever. None of the patients who developed rheumatic fever was on penicillin prophylaxis.

[Lung involvements of RA, diagnosis and treatment].

Various pleuro-pulmonary abnormalities and conditions are known to complicate rheumatoid arthritis. Some of them are asymptomatic, tend to ameliorate spontaneously(ex. pleuritis and rheumatoid nodule), others may cause serious conditions and often fatal(ex. interstitial pneumonia and constrictive bronchiolitis). Furthermore therapy-related lung complications and opportunistic infections may develop in RA patients. Clinicians must differentiate these conditions, using serological, physiologic, bacteriological, radiographic, and if necessary, invasive techniques including bronchoscopy and video-assisted thoracoscopic surgery. Therapy may vary according to the nature of these conditions. Appropriate anti-microbial agent must be given for infectious diseases. On the other hands immuno-suppressive therapy should be applied for interstitial pneumonia if the disease progress rapidly and threaten life. Anti-RA drugs should be withdrawn in case of drug-induced lung injury.

Laryngeal involvement in systemic lupus erythematosus.

Laryngeal involvement in systemic lupus erythematosus (SLE) can range from mild ulcerations, vocal cord paralysis, and edema to necrotizing vasculitis with airway obstruction. In this report, four cases showing the range of severity of this disease manifestation are presented, accompanied by a comprehensive review of the literature. The clinical course of 97 patients with laryngeal involvement with SLE are reviewed, of whom 28% had laryngeal edema and 11% had vocal cord paralysis. In the majority of cases, symptoms such as hoarseness, dyspnea, and vocal cord paralysis resolved with corticosteroid therapy. Other, less common causes of this entity included subglottic stenosis, rheumatoid nodules, inflammatory mass lesions, necrotizing vasculitis, and epiglottitis. The clinical presentation of laryngeal involvement in patients with SLE follows a highly variable course, ranging from an asymptomatic state to severe, life-threatening upper airway compromise. With its unpredictable course and multiple causations, this complication remains a diagnostic and therapeutic challenge to physicians involved in the care of patients with SLE.

Immunohistochemical and histochemical profile of Aschoff bodies in rheumatic carditis in excised left atrial appendages: an immunoperoxidase study in fresh and paraffin-embedded tissue.

We have evaluated the nature of Aschoff cells within Aschoff bodies seen in 35 of 100 excised left atrial appendages from cases of rheumatic mitral stenosis who underwent closed mitral valvotomy. These were tested using a panel of monoclonal and polyclonal antisera by the indirect immunoperoxidase staining for leucocyte common antigen, macrophage, desmin, vimentin, alpha-1-antitrypsin, alpha-1-antichymotrypsin, lysozyme, acid phosphatase and nonspecific esterase. The Aschoff cell gave strong reactivity with monoclonal antisera to vimentin, macrophage and variable reaction with polyclonal antisera known to recognise macrophages/histiocytes in tissues, namely alpha-1-antitrypsin, alpha-1-antichymotrypsin and lysozyme. These were also strongly positive for acid phosphatase and nonspecific esterase. The Aschoff cell lacked affinity for desmin and only an occasional cell in 4 out of 20 and 6 out of 35 cases showed a weak reaction with myoglobin and leucocyte common antigen, respectively. Intense consistent reactivity with several histiocytic markers affirms the genesis of these cells from macrophages/histiocytes and not muscle cells; a controversy which must be laid to rest!

Superficial ulcerating rheumatoid necrobiosis: a perforating rheumatoid nodule.

We report a case of superficial ulcerating rheumatoid necrobiosis (SURN), which is a recently described cutaneous manifestation of severe rheumatoid arthritis. As with classic rheumatoid nodule, there is evidence that this lesion may result from a vasculitis. SURN appears to represent a form of "perforating" rheumatoid nodule, and completes a triad of major necrobiotic processes (granuloma annulare, necrobiosis lipoidica, rheumatoid nodule) that have been reported to show epidermal perforation.

Origin of Aschoff nodule. An ultrastructural, light microscopic and histochemical evaluation.

Excised left atrial appendages were subjected to light and electron microscopy and histochemical analysis. Aschoff nodules (AN) showed strong acid phosphatase and nonspecific esterase activity. Ultrastructurally, the Aschoff cells had several features of fibroblasts and epithelioid cells. Rough endoplasmic reticulum (RER) was striking in most cells. Dilated cisterns of RER containing homogeneous material were also frequent. Mitochondria and ribosomes were present. AN thus seem to arise from mesenchymal tissues of the heart. It is likely that cross-reactivity between streptococcal antigen and myocardial connective tissue causes the cardiac histocyte/macrophage to transform to cells having features of epithelioid cells and/or fibroblasts.