Teaching Case

Patient belonging to a family of millers presenting with “shortness of breath”. Her father and one brother suffered from lung tuberculosis.

The patient consulted for “shortness of breath”. She belonged to a family of millers. Her father and one brother suffered from lung tuberculosis. There was a marked decline of her general condition and a weight loss of 20 kg over 2 years. Because ECG showed signs of ischemia a coronarography was planned but preoperative blood analysis revealed an alkaline phosphatase of 247 U/L (max 190 U/L) and a gamma GT of 60 U/L (max 24 U/L). Angiotensin-converting enzyme was elevated to 144 U/L (max 35 U/L), and there was a lymphocytosis of 35% (nl. 6-17%). Pulmonary function was restricted and diffusion capacity was decreased. Plain film of the chest, abdominal ultrasonography, CT scan of the abdomen and the thorax were performed. Biopsies were taken from lung, liver and bone. Radiological diagnosis

Sarcoidosis is a chronic multisystemic disease of unknown origin with accumulation of T-cell lymphocytes, mononuclear phagocytes and formation of non-caseating epitheloid granulomas. The presentation is most frequently with bilateral hilar lymphadenopathies, pulmonary infiltration and skin or eye lesions. Many subclinical cases are discovered on screening chest films. Virtually any organ can be affected. Clinical course and prognosis depend on which organs are involved. Although many granulomas resolve over time, some undergo fibrosis and pulmonary fibrosis may be the rule in many cases. On plain chest film and CT there are three basic patterns: reticular, acinar and large nodules. Cavitation, atelectasis, pleural effusion and spontaneous pneumothorax are rare manifestations. Bilateral hilar lymph node enlargement occurs in 75 to 85% although lymph nodes can be found anywhere as shown in our case. The majority of patients with sarcoidosis are young or middleaged black adults. The finding of non-necrotizing granulomas is a specific and the diagnosis is made by combining clinical, radiological and biochemical findings. BAL, Gallium scan and pulmonary function tests can give extra information. Activity can be assessed by plain chest film, ACE titer, BAL or Gallium scan. Corticosteroids remain the corner stone in therapy although it is not clear if it changes the course of the disease. When sarcoidosis presents in a disseminated form the differential diagnosis becomes wide and ncludes lymphoma, tuberculosis and extrinsic allergic alveolitis.