Maciej Sebastian

Chest HRCT findings in patients with primary Sjögren’s syndrome

BACKGROUND Pulmonary manifestations (PMs) in primary Sjögrens syndrome (pSS) are among the most frequent extraglandular complications, with reported prevalence varying widely (9-75%), depending on the methods of detection. OBJECTIVES The aim of this study was to assess the incidence of PMs in pSS and to determine the factors predisposing to the occurrence of this complication. MATERIAL AND METHODS The study group consisted of 68 patients with pSS. Among the patients who were possibly affected by PMs, chest High Resolution Computed Tomography (HRCT) was performed. RESULTS In the group of all patients afflicted with pSS, 30 people indicated the need to expand medical imaging via chest HRCT scan. (The most frequent reason, in 80%, was persistent, dry cough periodically waking up patients at night). The chest HRCT scan revealed lung tissue changes in the course of 29% of all examined patients (of 68). No correlation was found between the occurrence of HRCT changes and the age of patients (p = 0.8), increased CRP > 5 mg/1 (p = 0.1) or ESR > 20 mm/h (p = 0.9), focus score (p = 0.8), leucopenia (p = 0.5), RF value (p = 0.3), gamma globulin value (p = 0.5), intensity of eye and oral cavity dryness (p = 0.6; 0.3) and smoking cigarettes. Additionally, no correlation was found between more frequent occurrences of antibodies anti-SSA, anti-SSB or anti-Ro52 and HRCT changes (p = 0.3; 0.07; 0.4). Pertaining to the clinical signs, HRCT changes occurred more often only in patients suffering from peripheral arthritis (p < 0.01). CONCLUSIONS PM is a frequent symptom of pSS. A factor predisposing to the development of changes in the respiratory system was not found. Changes in HRCT occur more frequently in patients with peripheral arthritis.

Pancreatic heterotopia in the gallbladder: an incidental finding after cholecystectomy

Ectopic pancreas in the gallbladder is found very rarely in histological examination after cholecystectomy. The etiology of this entity is not yet clear, but there exist several hypotheses about its origin. Our histological study revealed both exocrine and endocrine components of pancreatic tissue as shown by H&E and immunohistochemical staining of a gallbladder sections of a 55-year old man. Ectopic pancreatic tissue may be an underestimated cause of acute idiopathic pancreatitis thus detailed postoperative histological examination may decrease the number of acute pancreatitis cases without the known cause.

The variety of clinical presentations in IgG4-related disease in Rheumatology

IgG4-related disease (IgG4-RD) belongs to the group of rare diseases in which the identification of the characteristic histology and immunohistochemistry provides with the gold standard in the diagnosis. The variable organ dysfunction reflects the clinical presentation. The examples of different IgG4-RD presentations in the Rheumatology Unit were discussed in this article. The spectrum of IgG4-RD is wide-ranging and manifested in one or more organs synchronously or metachronously. In the presented article, we described five different cases of IgG4-RD. Four cases were reaffirmed in the histopathological assessment. The clinical and laboratory findings were analyzed and the assigned therapy was discussed. According to our experience, the diagnosis of IgG4-RD requires the careful clinicopathological correlation. The diagnosis relies on the coexistence of various clinical, laboratory, radiological, and histopathological findings, although none of them is pathognomonic itself. The time needed for the diagnosis and variety of clinical forms of IgG4-RD shows that there is need of the cooperation among many specialists for the better and earlier recognition of the disease.

The Dufourmentel modification of the limberg flap: Does it fit all?

BACKGROUND Pilonidal disease is a common problem, especially in young adults. Therapy for the acute disease involves abscess incision and drainage. Treatment of the chronic disease is more complicated, due to the variety of methods and relatively high recurrence rate after surgical therapy. OBJECTIVES The aim of this study was to investigate the effects of the Dufourmentel modification of the Limberg flap in the treatment of pilonidal disease. MATERIAL AND METHODS The study involved 37 cases of chronic pilonidal disease that were operated on by the method presented. The group included 34 patients with primary disease and three patients with recurrences after primary excision. RESULTS The complication rate in the study group was 8.1%, but the complications were successfully treated, surgically in one patient (using the same method) and non-surgically in two other patients. There were also two cases of temporary numbness in the operated area. The only disadvantage of the method presented may be the cosmetic results, but they were not the most important factor for the patients in the study. CONCLUSIONS The authors find the Dufourmentel modification of the Limberg flap superior to other methods of treating pilonidal disease due to its flexibility, simplicity of reconstruction, repeatability and good outcomes in terms of the recurrence rate. These factors are extremely important to young and active patients who are diagnosed with pilonidal disease.

Laparoscopic management of abdominal pseudocyst following ventriculoperitoneal shunt implantation in hydrocephalus

Abdominal pseudocyst (AP) is quite a rare complication after ventriculoperitoneal shunting (VPS) for the treatment of hydrocephalus. Laparoscopy is an alternative method to open surgery with well-known advantages. There are not many reports of using laparoscopy to treat this condition and existing algorithms are based on small groups of patients; thus every case would give an additional insight into the treatment of this complication. A 37-year-old male patient with a history of VPS presented with headache, nausea and upper abdominal pain. Computed tomography of the abdominal cavity revealed a cyst in the left upper quadrant. After the ultrasound-guided percutaneous drainage the patient was qualified for laparoscopy. The cyst was removed laparoscopically under guidance of laparoscopic ultrasound. The patient recovered uneventfully with very good general and cosmetic results. In the follow-up period of 3 months we have not observed any abdominal or general health problems.

How to Distinguish Patients with pSS among Individuals with Dryness without Invasive Diagnostic Studies

In the course of pSS, inflammatory cell infiltration consists mainly of lymphocytes infiltrating exocrine glands, which leads to their impaired function. The characteristic feature is generalized dryness. The aim of this study was to attempt to answer the question whether it is possible to distinguish between patients with pSS and individuals with dryness caused by other pathologies without applying invasive studies. The study included 68 patients with pSS and 43 healthy controls with dryness. FS ≥ 1 was observed in 90% of patients with pSS (with or without dryness), and only in 23% of the control group (only with xerostomia). In the pSS group, anaemia (p = 0.0085), lymphocytopenia (p = 0.0006), elevated ERS (p = 0.001), higher RF titer, and ANA antibodies were noted. Configuration of anti-SSA + SSB + Ro52 antibodies was characteristic for the pSS group. Considering the clinical symptoms, statistically significant differences were noted between pSS patients and the control group in frequency (p = 0.02) and severity (p = 0.042) of fatigue, lymphadenopathy, major salivary gland involvement, and photosensitivity to UV light. In conclusion, invasive methods are pivotal in pSS diagnosis in this salivary gland biopsy. Chronic fatigue syndrome is more common in pSS patients and can be subjective distinguishing factor in the group of people with dryness.

Schwannoma in the porta hepatis – laparoscopic excision under laparoscopic ultrasound guidance

Schwannomas are usually benign tumors attached to peripheral nerves and are rarely found in the gastrointestinal tract. Schwannomas in the porta hepatis are extremely rare, with only 15 cases described in the literature to date. A 22-year-old female patient presented with colicky upper abdominal pain lasting 3 months. Magnetic resonance imaging of the abdominal cavity revealed a tumor in the porta hepatis. The patient was qualified for laparoscopy. The tumor was totally excised laparoscopically under guidance of laparoscopic ultrasound without intra- or postoperative complications. Postoperative histopathological examination confirmed the porta hepatic schwannoma. The patient recovered uneventfully with very good cosmetic results. In the follow-up period of 5 months we have not observed any abdominal or general health problems. The present case is the first report in the world of laparoscopic ultrasound guided laparoscopic excision of a porta hepatic schwannoma.

Pregnancy exacerbates complications of acquired hemophilia in a patient with systemic lupus erythematosus

Acquired hemophilia (AH) is a rare disease characterized by the production of antibodies directed against blood coagulation fVIII leading to coagulation disorders due to lowering of its activity. These are mostly the IgG class of antibodies which does not fixate the complement (IgG1 and IgG4 class) against A2 or C2 domains of fVIII which are responsible for its activity. The incidence of AH is 1.3–1.5 cases per 1 000 000 population per year and may affect both women and men [1]. Acquired hemophilia may be a primary disease or occur in the course of malignant diseases (e.g. prostate and lung cancer, lymphoproliferative and myeloproliferative syndromes), autoimmune diseases, during drug therapy (e.g. penicillin, sulfonamides, phenytoin, chloramphenicol, methyldopa, fludarabine and interferon) and pregnancy and puerperium. There were reported cases of AH in the course of systemic lupus erythematosus (SLE), primary Sjogrens syndrome, rheumatoid arthritis, giant cell arteritis and polymyositis. In around 50% of cases the exact cause is unknown [2, 3]. Acquired hemophilia can occur at any age and is characterized by hemorrhagic diathesis caused by bleeding into soft tissues, skin and mucosa. Other symptoms include large areas of ecchymoses, hematomas under the skin, nosebleed, hemoptysis, vaginal, urinal, gastrointestinal and retroperitoneal bleeding. In contrast to congenital hemophilia, hemarthroses are rare [2]. Laboratory tests reveal prolonged activated partial thromboplastin time (APTT), which is not corrected by infusion of fresh frozen plasma (FFP) and prothrombin time (PT) within normal range. Acquired hemophilia is usually characterized by a severe course of the disease and a high mortality rate [4, 5].

Histopathological evaluation of recurrent goiter

The recurrent goiter is the regrowth of thyroid tissue after thyroidectomy. An inadequate surgical removal of the thyroid gland, lack of substitution therapy and pathological stimulation of the thyroid growth can all promote the recurrence. The aim of this study was to find the connection between the histopathological findings during the first and second operation and the recurrence of goiter. The study group consisted of 29 women and 1 man. The mean time to recurrence was 15 years. The most frequent histopathological finding during the first and second operation was struma nodosa. According to our observations different histopathological findings were found in 63.4% cases after primary and secondary thyroidectomy. Some genetic investigations showed that nodules in recurrent goiters did not derive from nodules left during the first operation but from a group of cells which had high growth potential. Thus, not only the operation technique and substitution after operation are key factors of successful therapy of goiter, but also other factors which stimulate the re-growth of thyroid tissue.