Microscopic polyangiitis is a systemic small-vessel vasculitis that is associated primarily with necrotizing glomerulonephritis and pulmonary capillaritis. A recurrent and diffuse alveolar hemorrhage due to pulmonary capi llaritis is the main clinical manifestation of lung involvement. Recently, an interstitial lung disease that mimics idiopathic pulmonary fibrosis was reported to be rarely associated with microscopic polyangiitis. Here we report two patients with microscopic polyangiitis who showed a honeycomb lung at the time of the initial diagnosis with a brief review of relevant literature.
Diagnosis ; Glomerulonephritis ; Hemorrhage ; Humans ; Idiopathic Pulmonary Fibrosis ; Lung Diseases, Interstitial ; Lung* ; Microscopic Polyangiitis* ; Vasculitis

OBJECTIVETo analyze the clinical and pathological features of microscopic polyangiitis (MPA) to improve the diagnosis and treatment of the disease.

METHODSTwenty-five cases of MPA were retrospectively analyzed.

RESULTSThe onset symptoms of MPA, often nonspecific, included fever, muscle and joint pain, fatigue, loss of weight, etc, with varying degrees of proteinuria, hematuria and renal insufficiency. The pathological types revealed by renal biopsy were mainly focal segmental necrotizing glomerulonephritis or pauci-immune crescentic glomerulonephritis. Timely immunosuppressive therapy could improve the outcome.

CONCLUSIONThe early symptoms of MPA are often nonspecific to easily result in misdiagnosis. Examination of ANCA titers and timely renal biopsy are helpful to establish an early diagnosis. Immune suppression therapy and plasma exchange when necessary should be initiated after the establishment of the diagnosis. The disease activity and drug toxicity should be carefully monitored to improve the prognosis.

Adult ; Aged ; Female ; Humans ; Male ; Microscopic Polyangiitis ; diagnosis ; pathology ; therapy ; Middle Aged ; Prognosis ; Retrospective Studies

Objective To evaluate the clinical characteristics and prognostic predictors of patients with diffuse alveolar hemorrhage (DAH) and/or interstitial lung disease (ILD) secondary to microscopic polyangiitis (MPA) in a Chinese general hospital. Methods We retrospectively reviewed the medical records of MPA patients admitted to internal medicine departments between the year 2002 and 2012. The patients were divided into the ILD, DAH, DAH combined with ILD (DAHILD), and no pulmonary involvement (NPI) groups according to pulmonary involvement patterns. The clinical characteristics at diagnosis were analyzed. The risk factors associated with short-term death and long-term death were identified with Logistic regression and Cox analysis.Results Of 193 newly diagnosed MPA patients, 181 patients were enrolled in the research, of which 19 had DAH alone, 96 had ILD alone, 18 had DAH and DAH concurrently, and 48 had NPI. The median of serum creatine level in the DAH group was 449 μmol/L, significantly higher than that in the ILD group (123 μmol/L, Nemenyi = -35.215, P = 0.045) and DAHILD group (359 μmol/L, Nemenyi = -43.609, P = 0.007). The median follow-up time was 67 (range: 1-199) months. Patients in the ILD group were older than those in the DAH group (median: 69 years vs. 57 years, Nemenyi = 43.853, P= 0.005). The patients with both DAH and ILD had combined features of the two subtypes, and the highest mortality (72.2% at the end of follow-up). The elevated white blood cell count was a risk factor for short-term death (OR = 1.103, 95%CI: 1.008-1.207, P = 0.032 for one month; OR = 1.103, 95%CI: 1.026-1.186, P = 0.008 for one year). Old age (HR= 1.044, 95%CI: 1.023-1.066, P < 0.001), cardiovascular system involvement (HR = 2.093, 95%CI: 1.195-3.665, P = 0.010), poor renal function (HR = 1.001, 95%CI: 1.000-1.002, P = 0.032) were risk factors for long-term death. Pulmonary infections (38/54) were the leading causes of death, especially for the patients with ILD. Besides, 49 patients suffered from pulmonary infections in the first year after diagnosis. Conclusions MPA patients who presented with different pulmonary involvement patterns have completely different clinical features. These subtypes probably have different pathogenesis and should be studied separately.
Humans ; Microscopic Polyangiitis/diagnosis* ; Retrospective Studies ; Lung Diseases, Interstitial/complications* ; Hemorrhage/complications* ; Prognosis

PURPOSE: Delta neutrophil index (DNI) represents the immature granulocytes count associated with neutrophil-consumption. We investigated whether DNI might be associated with Birmingham vasculitis activity score (BVAS) at diagnosis and could predict relapse during the follow-up in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). MATERIALS AND METHODS: We reviewed the medical records of 97 patients having DNI results. Twenty patients had granulomatosis with polyangiitis (GPA), 58 had microscopic polyangiitis (MPA), and 19 had eosinophilic GPA (EGPA). We collected clinical and laboratory data including BVAS, five factor score (FFS), and DNI. The correlation coefficient and cumulative relapse free survival rate were obtained. The optimal cut-off of DNI was extrapolated by calculating the area under the receiver operator characteristic curve. RESULTS: DNI was significantly related to cross-sectional BVAS. Furthermore, among continuous variables, only DNI could reflect BVAS of GPA and MPA, but not EGPA. Severe AAV was defined as BVAS ≥20 (the highest quartile). At diagnosis, patients having DNI ≥0.65% had a significantly higher risk of severe GPA and MPA than those having not (relative risk 4.255) at diagnosis. During the follow-up, DNI ≥0.65% could predict the higher relapse rate. CONCLUSION: DNI could reflect BVAS at diagnosis and furthermore, DNI ≥0.65% could not only identify severe AAV at diagnosis, but also predict relapse during the follow-up in patients with GPA and MPA.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* ; Cytoplasm ; Diagnosis ; Eosinophils ; Follow-Up Studies ; Granulocytes ; Granulomatosis with Polyangiitis ; Humans ; Medical Records ; Microscopic Polyangiitis ; Neutrophils* ; Recurrence* ; Survival Rate ; Vasculitis*

OBJECTIVE: To investigate the significance of a set of seven disease activities and extension measurements and their correlations between one and another for anti-neutrophil cytoplasmic autoantibody associated vasculitis (AAV). METHODS: A total of 121 patients from Peking University International Hospital and Fouth Medical Center of PLA General Hospital with confirmed diagnoses of AAV clinically were enrolled in the study, including 15 cases of eosinophilic granulomatous with polyangiitis (EGPA), 59 cases of granulomatous with polyangiitis (GPA) and 47 cases of microscopic polyangiitis (MPA). A hundred and twenty-one AAV patients were divided into death group and survival group according to their survival conditions. A set of seven disease assessment scales including Birmingham vasculitis activity score (BVAS)-1994, BVAS-2003, as well as BVAS/GPA, vasculitis damage index (VDI), disease extent index (DEI), five factor score (FFS)-1996, and FFS-2009 were measured and scored one by one, and their relationships which were represented by Spearman correlation coefficient were compared between one and another. RESULTS: BVAS-1994, BVAS-2003, as well as BVAS/GPA, VDI, DEI, and FFS, all of those seven evaluation indexes of the AAV patients in the death group were significantly higher than those in the survival group (P<0.05). Except for BVAS/GPA, all those above indicators in the patients with EGPA were lower than those in the patients with GPA and those in the patients with MPA, and those in all of the AAV patients as a whole group. There were high correlations among BVAS-2003, BVAS-1994 and BVAS/GPA (r values were 0.9 and 0.7, respectively); BVAS-1994 was fairly correlated with BVAS/GPA (r=0.69); FFS-1996 and FFS-2009 were highly correlated (r=0.73) with each other; BVAS-1994, BVAS-2003 and BVAS/GPA were fairly correlated with DEI (with r values of 0.62, 0.65, and 0.62, respectively); VDI was also fairly correlated with BVAS-1994 and with BVAS-2003 (r values were 0.49 and 0.52, respectively). CONCLUSION All of those seven AAV assessment indicators above can be used as indicators of disease activity and prognosis in AAV patients, most of which were relevant within one and another. There were high correlations among BVAS-2003, BVAS-1994 and BVAS/GPA, and besides, there were also high correlations between FFS-1996 and FFS-2009.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis* ; Antibodies, Antineutrophil Cytoplasmic ; Autoantibodies ; Biomarkers/analysis* ; Humans ; Microscopic Polyangiitis ; Neutrophils

Wegener's granulomatosis is a systemic vasculitis of the medium and small arteries, as well as of the venules, arterioles, and occasionally large arteries, and primarily involves the upper and lower respiratory tracts and the kidneys. Renal symptoms of Wegener's granulomatosis are indistinguishable from those of vasculitis such as Henoch-Schonlein purpura and microscopic polyangiitis. This case, though initially diagnosed as Henoch-Schonlein purpura, was confirmed as Wegener's granulomatosis from a lung biopsy fifteen years after the initial diagnosis. We report this case with a review of the literature.
Arteries ; Arterioles ; Biopsy ; Delayed Diagnosis* ; Diagnosis ; Kidney ; Lung ; Microscopic Polyangiitis ; Purpura, Schoenlein-Henoch* ; Respiratory System ; Systemic Vasculitis ; Vasculitis ; Venules ; Wegener Granulomatosis*

Wegener's granulomatosis is a systemic vasculitis of the medium and small arteries, as well as of the venules, arterioles, and occasionally large arteries, and primarily involves the upper and lower respiratory tracts and the kidneys. Renal symptoms of Wegener's granulomatosis are indistinguishable from those of vasculitis such as Henoch-Schonlein purpura and microscopic polyangiitis. This case, though initially diagnosed as Henoch-Schonlein purpura, was confirmed as Wegener's granulomatosis from a lung biopsy fifteen years after the initial diagnosis. We report this case with a review of the literature.
Arteries ; Arterioles ; Biopsy ; Delayed Diagnosis* ; Diagnosis ; Kidney ; Lung ; Microscopic Polyangiitis ; Purpura, Schoenlein-Henoch* ; Respiratory System ; Systemic Vasculitis ; Vasculitis ; Venules ; Wegener Granulomatosis*

BACKGROUND/AIMS: Gastrointestinal involvement in vasculitis may result in life-threatening complications. However, its variable clinical presentations and endoscopic features, and the rarity of the disease, often result in delayed diagnosis. METHODS: Clinical characteristics, endoscopic features, and histopathological findings were reviewed from medical records. RESULTS: Of 6,477 patients with vasculitis, 148 were diagnosed as primary vasculitis with upper gastrointestinal involvement. Of these, 21 cases (14.2%) were classified as large-vessel vasculitis, 17 cases (11.5%) as medium-vessel vasculitis, and 110 cases (74.3%) as small-vessel vasculitis. According to the specific diagnosis, IgA vasculitis (Henoch-Schönlein purpura) was the most common diagnosis (56.8%), followed by Takayasu arteritis (14.1%), microscopic polyangiitis (10.1%), and polyarteritis nodosa (6.8%). Gastrointestinal symptoms were present in 113 subjects (76.4%), with abdominal pain (78.8%) the most common symptom. Erosion and ulcers were striking endoscopic features, and the second portion of the duodenum was the most frequently involved site. Biopsy specimens were obtained from 124 patients, and only eight (5.4%) presented histopathological signs of vasculitis. CONCLUSIONS: Diagnosis of vasculitis involving the upper gastrointestinal tract is difficult. Because of the widespread use of endoscopy, combining clinical features with endoscopic findings may facilitate making appropriate diagnoses; however, the diagnostic yield of endoscopic biopsy is low.
Abdominal Pain ; Biopsy ; Delayed Diagnosis ; Diagnosis ; Duodenum ; Endoscopy ; Gastrointestinal Tract ; Humans ; Immunoglobulin A ; Medical Records ; Microscopic Polyangiitis ; Polyarteritis Nodosa ; Strikes, Employee ; Takayasu Arteritis ; Ulcer ; Upper Gastrointestinal Tract ; Vasculitis*

Microcopic polyangiitis (MPA) is a systemic disorder affecting small vessels. In MPA, the central nervous system involvements have rarely been reported. We experienced a case of MPA with subdural effusion and pachymeningitis in a 50-year-old woman. The diagnosis of MPA was made by the presence of mononeuritis multiplex, hematuria, pachymeningitis, and p-ANCA seropositivity in this patient. Subdural effusion was of exudates and an active suppurative inflammation was observed on the dural biopsy.
Antibodies, Antineutrophil Cytoplasmic ; Biopsy ; Central Nervous System ; Diagnosis ; Exudates and Transudates ; Female ; Hematuria ; Humans ; Inflammation* ; Meningitis ; Microscopic Polyangiitis* ; Middle Aged ; Mononeuropathies ; Subdural Effusion*

Microscopic polyangiitis (MPA) is a systemic small vessel vasculitis with few or no immune deposits and no granulomatous inflammation. Peripheral neuropathy occurs in approximately 20%–30% of patients with MPA. We report a case of a 66-year-old woman who presented with paresthesia and motor weakness of the extremities and rapidly progressive glomerulonephritis. She was later diagnosed with MPA based on the findings of positive perinuclear antineutrophil cytoplasmic antibody along with findings on kidney biopsy. Nerve conduction study showed symmetric sensorimotor polyneuropathy. We followed the patient for 3 years, and she showed good functional outcome after immune-modulating therapy although Five-Factor Score more than 2 at diagnosis.
Aged ; Antibodies, Antineutrophil Cytoplasmic ; Biopsy ; Diagnosis ; Extremities ; Female ; Follow-Up Studies* ; Glomerulonephritis ; Humans ; Inflammation ; Kidney ; Microscopic Polyangiitis* ; Neural Conduction ; Paresthesia ; Peripheral Nervous System Diseases* ; Polyneuropathies ; Vasculitis