Case Report Acta Medica Anatolia Volume 1 Issue 1 2013

The Importance of Microscopic Polyangiitis in Differential Diagnosis of Hemoptysis

Fatih Demircan1, Faruk Kilinc2, Nevzat Gözel3, Cemil Göya4 1 Department of Internal Medicine, Private Cagri Medical Center, Elazig, Turkey 2 Department of Internal Medicine, Dicle University Faculty of Medicine, Diyarbakir, Turkey 3 Department of Internal Medicine, Private Cagrı Dialysis Center, Elazig, Turkey 4 Department of Radiology, Dicle University Faculty of Medicine, Diyarbakir, Turkey

Abstract

Microscopic polyangiitis (MPA) is non-granulomatous necrotizing vasculitis, which affect small vessels of kidney, skin and lung. This disease is characterized by the absence of immune deposits and positive ANCA in biopsy. In this article, we describe a microscopic polyangiitis case that presenting with hemoptysis, dyspnea and progressive cough and emphasized that if a patient with nonspesific pulmonary symptoms, we should consider in the differential diagnosis of vasculitis.

Keywords: Microscopic polyangiitis, hemoptysis, vasculitis

Received: 14.10.2013 Accepted: 10.12.2013

Introduction

Microscopic Polyangiitis (MPA) is a type of systemic MPA has an onset with flu-like non-specific symptoms necrotizing vasculitis manifesting with inflammation and the mean age of onset is 57 with a mildly higher that involves the kidneys, skin and the lungs. This incidence among males compared to females. disease involving the small vessels such as capillaries, Histological investigations reveal leukocytoclastic venules or arterioles is characterized by ANCA positivity vasculitis involving the small veins (1). Absence of the and absence of immune deposits on biopsy (1). granuloma form and a negative immunofluorescent (IF) detected represent significant characteristic findings In 1948, Davson et al first described cases manifesting for the disease (1, 6). In MPA, kidneys are commonly with systemic vasculitis and fast progressing renal involved with the initial sign being microscopic disease. This type of microscopic polyarteritis showing hematuria and proteinuria. At the time of diagnosis, differences as compared to PAN was termed as 80% of the patients have renal dysfunction and 30% Microscopic Polyangiitis (MPA) at the Chapel Hill have oliguria (7). Renal biopsies mostly reveal focal Consensus Conference classification in 1994 and segmental necrotizing glomerulonephritis and described separately from the classic PAN. sometimes its crescent form (7).

Discovery of antineutrophil cytoplasmic antibodies Hemorrhage into the alveolar spaces leads to dyspnea, targeting the proteins in the lysosomal granules of the hemoptysis, anemia and bilateral alveolar consolidation leukocytes in 1980s followed by classification of ANCA on lung graphy. Pulmonary involvement manifesting as as c ANCA (cytoplasmic) and p ANCA (perinuclear) and alveolar hemorrhage occurs in 30-40% of the MPA particularly demonstration of its association with the cases and represents one of the most important causes small vein vasculitis opened up new horizons for of the mortality increase. Approximately 34% of the vasculitis (2, 3). While cytoplasmic ANCA (c ANCA) is patients have recurrence in the first two years. The 5- particularly specific for Wegener Granulomatous, year survival rate is around 74% in MPA (1). perinuclear ANCA (p ANCA) comes to the forefront in microscopic polyangiitis. Its value in the disease In this report we described a patient initially exacerbation is still being investigated (4, 5). presenting with lung-specific symptoms such as hemoptysis and developing life-threatening renal Differentiation of MPA and classic PAN is also failure since immunosuppressive treatment had not important. Classic PAN involves the necrotizing been started, considering the presence of vasculitis in vasculitis of the small-moderate diameter arteries differential diagnosis. without glomerulonephritis, arteriole, venule or capillary vasculitis. Presence of arterial microaneurysm and a negative ANCA in majority of the patients are findings in favor of the presence of PAN (1).

Correspondence: Fatih Demircan MD, Private Cagri Medical Center, Department of Internal Medicine, Elazig, Turkey. 30 [email protected]

Original Article Demircan et al.

Case Discussion

A 65-year-old male patient presented to our Patients with microscopic polyangiitis have rather non- emergency with the complaints of weight loss, fever, specific symptoms (fever, fatigue, sweating, weight cough, bloody sputum and respiratory distress that loss) in the initial period. In this period, mild anemia, have been present for the last 6 months. The patient leukocytosis, thrombocytosis and high sedimentation was hospitalized with the preliminary diagnosis of are observed as laboratory findings (1). Subsequent tuberculosis by the Chest Diseases Department due to alveolar hemorrhage results in hemoptysis and the presence of constitutional symptoms (fever, hypoxemia due to impaired oxygen transport. fatigue, sweating, weight loss, hemoptysis and high sedimentation. PA lung x-ray showed bilateral diffuse In this period, the major symptoms are cough, infiltration. Physical examination detected no dyspnea and hemoptysis. Alveolar hemorrhage is life pathological findings other than diffuse rales. The threatening. In our case, hemoptysis was among the investigations performed in the first weeks showed initial presentation symptoms. mild leukocytosis, high sedimentation and anemia. BUN, creatinine and electrolyte values were normal. Several difficulties are experienced in the pulmonary Non-specific antibiotic treatment was initiated. diagnosis made in a limited time period. If the appropriate diagnosis is made and the Upon experiencing deterioration in the overall status immunosuppressive treatment initiated as soon as and increased dyspnea on the fifth day of possible, renal involvement, a condition that hospitalization, the patient underwent assessment. determines the disease prognosis, would be avoided. The laboratory tests showed microscopic hematuria, The severity of the renal involvement determines the 2.5 grams/day proteinuria, 140 mg/dL urea, 9.2 mg/dL prognosis of the disease. Early diagnosis and creatinine and the lung graphy showed bilateral immediate initiation of steroid and/or cytotoxic agents diffuse interstitial edema, the thorax tomography are required. 34% of the patients experience disease showed bilateral diffuse alveolar hemorrhage (Figure recurrence within the first 2 years. The five year 1). survival rate is 74% (1).

During follow-up, a rapid increase in the urea- Tuberculosis was considered first due to the presence creatinine levels and oliguria developed and thus the of symptoms including hemoptysis, fever, fatigue, patients was started on dialysis treatment. He muscle pain and weight loss. Even if the clinical underwent renal biopsy with the preliminary diagnosis symptoms reflected tuberculosis, the absence of acid- of fast progressing glomerulonephritis. The renal resistant bacteria in the sputum culture and lack of biopsy result was considered consistent with growth caused us to exclude this diagnosis. crescentic necrotizing glomerulonephritis. Immunofluorescent study on the biopsy material Symptoms such as acute dyspnea, hemoptysis, non- yielded a negative result. Perinuclear antineutrophil specific chest pain and fever also suggested the cytoplasmic antibody (p-ANCA) was considered preliminary diagnosis of pulmonary embolus. Absence positive. No pathology was detected in the other of significant pathology in the serum fibrinogen and D- serologic and immunologic tests. dimer results and the pulmonary tomography results caused us to exclude this diagnosis also. The patient was diagnosed with microscopic polyangiitis (MPA) based on the available findings. The Upon recurrence of the disease with renal patient was prescribed pulse steroid treatment (1 involvement following the initial involvement limited gram/day methylprednisolone i.v for 3 days followed to the lungs, the preliminary diagnosis of systemic by 1.5 mg/kg/day oral prednisolone maintenance disease became stronger; absence of asthma and treatment). The treatment resulted in a rapid eosinophilia ruled out the Churg-Strauss syndrome, improvement in patient’s renal functions (urea: 100 the absence of granuloma and a negative c-ANCA mg/dl, creatinine: 2 mg/dl) and pulmonary findings. ruled out Wegener’s syndrome, absence of immune Maintenance treatment was established and the deposits on renal biopsy ruled out Goodpasture’s patient was put under outpatient monitoring. syndrome and the other immundeposit diseases. Based on the positive p-ANCA, the presence of systemic symptoms, the presence of alveolar hemorrhage on thoracic tomography and crescentic necrotizing glomerukonephritis on renal biopsy, the patient was diagnosed with microscopic polyangiitis.

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Original Article Demircan et al.

Figure 1: The thorax tomography showed bilateral diffuse alveolar hemorrhage.

Conclusion

In conclusion, a patient with atypical pulmonary and nonspecific systemic symptoms, a diagnosis of microscopic polyangiitis and other vasculitic syndromes should be considered, differential diagnosis should be made, appropriate treatment should be initiated as soon as possible if it is diagnosed in.

References

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