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21An uncommon presentation of Granulomatosis with Polyangiitis
Eleftherios Pelechas, Georgios Zouzos, Paraskevi V. Voulgari, Drosos A. Alexandros
Mediterr J Rheumatol 2018; 29(1):49-51
mediterranean journal
of RHEUMATOLOGY
s-ISSN: 2529-198X
E-ISSN: 2529-198X
mediterranean journal of rheumatology March 2018 | Volume 29 | Issue 1
mediterranean journal 29
of RHEUMATOLOGY 2018
© Pelechas E, Zouzos G, Voulgari VP, Drosos AA
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under and Creative Commons /qqj M Attribution-Noncommercial 4.0 International L
CASE REPORT
An uncommon presentation of Granulomatosis with Polyangiitis
Eleftherios Pelechas1, Georgios Zouzos, Paraskevi V. Voulgari2, Alexandros A. Drosos3
Rheumatology Clinic, Department of Internal Medicine, Medical School University of loannina, loannina, Greece
: https://orcid.org/0000-0002-9383-5722 : https://orcid.org/0000-0002-5193-2284 : https://orcid.org/0000-0002-2232-0326
ABSTRACT
In this case, we present a patient with an uncommon presentation of Granulomatosis with Polyangiitis (GPA) with hepatic involvement and the possible association with subclinical coeliac disease. We discuss the differential diagnosis and the relevant therapy.
Mediterr J Rheumatol 2018; 29(1): 49-51 doi10.31138/mjr.29.1.49
Article Submitted: 13/09/2017, Accepted 28/09/2017
Keywords: Granulomatosis with Polyangiitis, subclinical coeliac disease, hepatosplenomegaly.
INTRODUCTION
Granulomatosis with Polyangiitis (GPA) is a systemic necrotizing vasculitis characterised by segmental inflammation and necrosis of blood vessels. It mainly affects the medium and small-calibre arteries, especially of the respiratory tract and kidneys.1 The clinical manifestations of the disease vary considerably, and at times, it is difficult to differentiate between the various types of necrotizing arteritis and in addition, it may present with an overlapping syndrome complicating even more the clinical picture.2 In this direction, we present a patient with an uncommon presentation involving liver and spleen pathology, but also positivity to coeliac disease serologic tests.
Corresponding author:
Alexandros A. Drosos, MD, FACR
Professor of Medicine/Rheumatology
Rheumatology Clinic, Department of Internal Medicine
Medical School, University of loannina
Ioannina 45110, Greece
Tel.: +302651007503
Fax: +302651007054
E-mail: adrosos@cc.uoi.gr
Web: http://www.rheumatology.gr
CASE DESCRIPTION
We present a 35-year-old man with a rather non-significant past medical history apart from an episode of si-alolithiasis in the past year and a recent upper airway infection. On his arrival in the emergency department he complained about fevers (mainly during nighttime - he was using paracetamol and non-steroidal anti-inflammatory drugs), diaphoresis, weight loss, generalised arthral-gias and myalgias for almost one month. At admission, his temperature was 38,20C, pulse was 83 beats/min, respiratory rate was 20 breaths/min, blood pressure was 131/77 mmHg and SpO2 95%. He was not in severe distress. The main findings from the clinical examination were decreased breath sounds of the lung bases, palpable but non-painful lymph nodes in the axillae and cervical regions as well as hepatosplenomegaly. The remainder of the examination was unremarkable. Laboratory studies showed elevated C-reactive protein 79 mg/l (0-6), erythrocyte sedimentation rate 42 mm/1st hour (0-20), elevated aspartate aminotransferase and alanine aminotransferase 123 U/L and 77 U/L respectively (10-35), Y-glutamyl transferase 144 U/L (10-52), alkaline phosphatase 254 U/L (35-125) and aldolase 11,8 U/L (0-7,7). Full blood count and urea and electrolytes were within normal limits. A urine dip test was positive for protein and haemoglobin +2/+2 respectively. Two days after his admission to the hospital he developed abdominal pain, but he denied nausea.
Cite this article as: Pelechas E, Zouzos G, Voulgari VP, Drosos AA. An uncommon presentation of Granulomatosis with Polyangiitis. Mediterr 49 J Rheumatol 2018; 29(1): 49-51.
mediterranean journal
of RHEUMATOLOGY
29 1
2018
Figure 1. Chest x-ray on admission with no apparent findings.
Figure 2. Computed Tomography of the abdomen confirming the hepato- and splenomegaly.
Based on the initial clinical and laboratory findings as well as the symptomatology, infective agents should be excluded. Blood and urine cultures, tuberculin skin test and interferon-Y release assay, B and C hepatitis, HIV I+II test were all negative. Also, serological tests for syphilis, leishmaniasis, brucellosis, Epstein-Barr and cytomegalovirus were also negative. Chest x-ray (CXR) on admission had no apparent findings (Figure 1). An abdominal ultrasonography and later a computed tomography (CT) of the abdomen confirmed the hepatosplenomegaly (Figure 2). Electromyography was normal. A liver biopsy showed non-specific inflammation with no fibrosis. Upper and lower gastrointestinal examination revealed also non-specific findings, but a small intestine biopsy came back with results compatible with coeliac disease. Further serologic tests for coeliac disease (anti-tissue trans-glutaminase, endomysial and deamidated gliadin pep-
Figure 3. Multiple infiltrates on a chest x-ray
tide antibodies) were positive, thus, in lack of symptomatology, a diagnosis of subclinical coeliac disease was made. A 24-hour urine collection was initially 1,6gr but reached up to 5gr/24h. Pulmonary function tests as well as diffusing capacity of the lung for carbon monoxide were within normal limits. Bronchoscopy was arranged but the patient developed dyspnoea and later haemoptysis. A new CXR revealed scattered lung infiltrates (Figure 3) and a lung CT showed multiple nodules and ground glass areas (Figure 4). At the same time, the immuno-logic evaluation came back with positive anti-neutrophil cytoplasmic antibodies (c-ANCA) at a titre of 1/80, pro-teinase-3 (PR-3) positive. Later, the renal biopsy revealed pauci-immune necrotic glomerulonephritis with crescent formation.
The patient was treated with 3 pulses of methylpredniso-lone (1gr/day) followed by prednisolone Img/kg/day and
Figure 4. Computed Tomography of the lungs showing multiple nodules and ground glass areas
AN UNCOMMON PRESENTATION OF GRANULOMATOSIS WITH POLYANG IITIS
prophylaxis for osteoporosis. Also, pulses of cyclophosphamide were initiated at a dosage scheme of 0,5gr/ m2/15 days for three months and steroid tapering with significant improvement.
DISCUSSION
GPA or formerly known Wegener's Granulomatosis was first described by Klinger3 in 1931, but in 1936, Wege-ner incorporated both clinical and histological criteria to describe what he believed represented a unique and distinctive syndrome.4 Virtually any organ can be involved but given the rarity of GPA, and non-specificity of symptoms, the diagnosis is often missed and a judgement can only be made on a step-by-step clinical basis. In older studies, hepatic involvement was not a feature of GPA, however, isolated case reports have been published mentioning of hepatic involvement5-6 in GPA patients. Goritsas et al6 in a case report with a GPA patient favor the hypothesis that hepatic vasculitis may be the cause of acute hepatocellular necrosis, because there was immediate response of liver function tests after the initiation of immunosuppressive therapy with prednisone and cyclophosphamide. Carr et al7 has confirmed cytotoxic T lymphocyte-associated molecule-4 (CTLA4) and protein tyrosine phosphatase, non-receptor type-22 (PTPN22) as susceptibility loci in ANCA-associated vasculitides (AAV). These genes encode two key regulators of the immune response and are associated with many autoimmune diseases, including type-1 diabetes, autoimmune thyroid disease, coeliac disease, rheumatoid arthritis, and AAV. Thus, there may be a common pathogenetic mechanism that strengthens the diagnosis of AAV with subclinical coeliac disease in our patient. Although the aetiology of GPA remains unclear and the course of the disease is not always as it appears in the textbooks, GPA remains a real challenge for the clinician both for the diagnosis and the treatment.
CONFLICT OF INTEREST
The authors declare no conflict of interest.
REFERENCES
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