Unusual presentation of sarcoidosis

The extrapulmonary presentation of sarcoidosis is a frequent occurrence - over 30% of cases when the observation is prospective - but early diagnosis may still be a challenge. A comparison between two different Italian series - (1) patients visiting the Milan Sarcoidosis Clinic of an Internal Medicine Department and (2) an Italian Register from many pneumology centers (RIPID) - has shown a different approach to the diagnosis because pneumologists are more likely to use transbronchial biopsy, while internists are more likely to use extrapulmonary biopsies. Such a different clinical approach may be due in part to a different case mix of the two series.     

Unusual presentation of lupus nephritis

We report a male patient who presented with pyrexia, generalized lymphadenopathy, hepatosplenomegaly, and pleural effusion with no cutaneous or musculoskeletal symptoms. Despite extensive investigation, no cause was detected. His initial serology was also negative for autoantibodies. The patient was placed on a trial of antitubercular treatment in view of a positive Mantoux test. His disease evolved into the full clinical picture of systemic lupus erythematosus with nephritis (World Health Organisation class IV) and strongly positive antinuclear antibody and dsDNA over a period of months. He was treated successfully with intravenous cyclophosphamide pulses along with oral prednisolone, and the disease was still in remission after 3 years of follow-up.     

Unusual presentation of cardiac sarcoidosis

A 46-year-old white woman presented to the emergency department with hemodynamically stable sustained ventricular tachycardia (VT). She was chemically cardioverted with lidocaine. Her electrocardiogram, showing sinus rhythm, was unremarkable, and serial cardiac enzyme tests excluded myocardial infarction. A signal-averaged electrocardiogram was abnormal, with a filtered QRS duration of 187 milliseconds. Echocardiography showed normal left and right ventricular systolic function but revealed diastolic dysfunction of the left ventricle. Electrophysiologic testing revealed easily inducible sustained VT of 4 distinct morphologies. A diagnosis of possible arrhythmogenic right ventricular dysplasia was made based on the signal-averaged electrocardiographic and electrophysiologic findings. Cardiac magnetic resonance imaging showed no evidence of arrhythmogenic right ventricular dysplasia, however. An endomyocardial biopsy revealed noncaseating granulomas consistent with sarcoidosis. This case illustrates the importance of complete testing, including invasive studies such as endomyocardial biopsy, in patients presenting with life-threatening ventricular arrhythmias to establish a correct diagnosis.     

Unusual presentation of Bouveret's syndrome

Received: September 8, 2000 / Accepted: April 13, 2001     

Unusual presentation of rectal adenocarcinoma

Metastatic tumors in the gastrointestinal tract are rare with an overall prevalence of 1-4 per cent in the postmortem series. Lung cancer, renal cell carcinoma, breast carcinoma and malignant melanoma are considered the most common primary tumors metastatic to the small bowel. Local duodenal metastasis from colonic cancer and cecum have been reported, but metastasis to the duodenum from rectosigmoid adenocarcinoma has not been reported before. We report the first case of metastasis in the duodenum from an adenocarcinoma of the rectum presented as a recurrent acute prerenal azotemia caused by volume depletion which had resulted from duodenal obstruction.     

Unusual presentation of polyarteritis nodosa

We describe an unusual presentation of a localized form of polyarteritis nodosa (PAN) manifested by acute onset of severe calf pain. Biopsies of the gastrocnemius muscle and fascia revealed an acute necrotizing arteritis with fasciitis. The lumens of affected vessels were occluded by thrombi. PAN localized to calf muscles is extremely rare. To our knowledge this is the first report of evidence of fascial involvement believed to contribute to the severity of the clinical features of PAN. The occurrence of multiple intraluminal thrombi in conjunction with anticardiolipin antibodies suggested the possibility of a coexisting coagulopathy, and they were also likely contributors to the severity of the pain.     

Unusual presentation of anaerobic osteomyelitis

Acute hematogenous osteomyelitis was detected in a 23-year-old sickle-cell patient after standard radiographs of the right femur showed gas within the medullary cavity. Three anaerobic organisms were cultured from surgically aspirated materials, but no source of infection was found. After surgical drainage and six weeks of intravenous clindamycin therapy, there was clinical improvement and radiographic resolution of the gas formation.     

Unusual presentation of renal tuberculosis

Urogenital tuberculosis (TB) is a common late manifestation of an earlier symptomatic or asymptomatic pulmonary TB infection. A latency period ranging from 5 to 40 years between the time of the initial infection and the expression of urogenital TB frequently occurs. As one of the most common sites of involvement of extrapulmonary TB, urogenital TB accounts for 15% to 20% of the infections. We present a patient who had culture-negative active tubercular kidney disease due to silent tuberculous infection. Our case demonstrates the limitations of noninvasive testing in establishing the diagnosis of renal tuberculosis.     

Unusual presentation of endomyocardial fibrosis

A case of endomyocardial fibrosis in a Greek patient who had not visited the tropics is described. The patient suffered from an unusual form of the disease in that it was aggressive and recurrent (requiring three operative procedures) and presented with tricuspid stenosis, leading to syncope.     

Unusual presentation of recurrent Wegener's granulomatosis

A 65-year-old woman presented with recurrent Wegener's granulomatosis following two years of immunosuppressive therapy and three years of complete remission. At her initial presentation, she had a characteristic x-ray picture showing multiple nodules with total resolution of these findings at three months. Five years later, at the time of clinical relapse, her chest x-ray film showed bilateral diffuse infiltrative disease. This change in radiologic presentation upon relapse of Wegener's has not previously been reported. Other unusual features include diffuse infiltrates as the pulmonary presentation and the long interval between cessation of therapy and relapse. We review the radiologic manifestations of Wegener's granulomatosis.