Introduction:

Sarcoidosis with a prevalence of 10/100,000, is a multisystem disease of unknown etiology characterized by non-caseating granulomas.i It involves virtually any organ, lungs, thoracic lymph nodes, skin liver, central nervous system, eyes, kidneys and heart, and is more common in Afro-Americans. There is no sex predilection but some manifestations of the disease are more common is females. ² It begins in the third or fourth decade and tends to be rare in children and the elderly.? We are presenting a case of sarcoidosis of the lungs which should be considered in the differential diagnosis of tuberculosis, common in countries like Pakistan.

Discussion:

Considering the common disease first which is tuberculosis, long-standing history of low grade fever, weight loss and persistent cough not responding to anitibiotics along with raised ESR and positive PPD(13 mm) supports tuberculosis put PPD is not reliable in developing countries as a tool for diagnosis.

Diagnostic procedure performed was an endobronchial biopsy. ² Histopathological examination showed multiple granulomas, composed of epitheloid histiocytes, with occasional Langerhans giant cell and no necrosis. According to one study endobronchial biopsy increased the yield of fibreoptic bronchoscopy by 20.6%.4 Fungal stain, AFB stains and cultures, were negative (Table II).

The patient had X-ray findings consistent with stage II disease. 1,5 Unilateral hilar adenopathy is an atypical manifestation of sarcoidosis in initial stage. 6 Her ACE level was raised about three folds, serum calcium was in upper normal range and ESR was persistently raised (Table I) supported by radiological findings, which favour sarcoidosis. PPD is negative in sarcoidosis are anergic to PPD tuberculin but distribution of memory T-cells in the blood and humoral immune response are normal and opportunistic infections are rare.¹

Fifty percent of sarcoid cases have clinically silent involvement of organs, which is one of the hallmarks.¹ 20-40% of symptomatic patients have respiratory symtoms and 10-40% have eye pain, rashes arthralgia and other symptoms while 20-30% with constitutional symptoms like weight loss and fatigue.¹

Sarcoidosis may present atypically as Lofgrens and HeerFordts syndrome.¹ Sarcoidosis in younger patients with spontaneous remission and less than two years duration of symptoms are classified as subacute while chronic form have more than two years duration with insidious onset and presents with constitutional symptoms, pulmonary fibrosis and nephrocalcinosis.¹

In one study in which histopathological diagnosis required presence of non-caseating granuloma, bronchoscopic biopsy yielded diagnosis in 60% of patients with stage I disease, tranbronchial needle aspiration yielded diagnosis in 53% and two procedures in combination yielded 83% of diagnosis. 7,8 In stage II disease probability of obtaining a positive result with a single specimen was 46%, the yielded with more specimens followed a logarithmic curve and increased to 90% with four specimen, at which point the yield approached a plateau for additional specimen. 7,8 The suggests that four biopsy specimens are sufficient to make diagnosis. Biopsy of gastrocnemius muscle is another sensitive and specific tool for diagnosis. 9 If transbronchial biopsies are non-diagnostic, mediastinal lymph node biopsies are diagnostic in 8-90% of case. 10 Finally , the patient was kept on steroids (Deltacortil) 40mg/day in divided doses and He/She responded well with remarkable improvements. 11 His/Her ESR came down to 13mm, chest X-ray showed remarkable improvement as infiltrates and bilateral hilar adenopathy disappeared altogether.

We kept His/Her on the same dose of Deltacortil till 8 weeks, and then it was trapered off to 7.5mg/day without recurrence.