Patient description: A 57-year-old man was admitted to the clinic because of weight loss and persistent dry cough 4 months ago. Chest computed topography showed bilateral multiple infiltrates in the upper lobes and thickened bronchial walls. There is a documented positive serum MPO-ANCA in his medical record. Transbronchial biopsy revealed necrotic granulomas with multinucleated giant cells and the Wegener's granulomatosis was diagnosed for him. He is treating with corticosteroid and cyclophosphamides since 4 months ago. His Birmingham Vasculitis Activity Score (BVAS) is above 4 since the beginning of his disease. His last physical exam and lab study was performed yesterday and showed the results bellow: A wellbeing, well-nourished man, non-icteric, cooperative and alert Weight: 73 kg Height: 177 BP: 120/80 HR: 90/min RR: 22/min Hgb: 13 g/dl WBC: 8000 /mm3 (Neutrophil: 2700/mm3) Plt: 300000 /ml AST: 40 U/L ALT: 56 U/L Alk P: 147 U/L Bill total: 1.2 mg/dl ESR: 120 mm/hr MPO-ANCA: 153 EU

Clinical trial eligibility criteria: eligible ages (years): 18.0-999.0,  Acinar Cell Carcinoma Adenoid Cystic Carcinoma Adrenal Cortex Carcinoma Adrenal Gland Pheochromocytoma Anal Canal Neuroendocrine Carcinoma Anal Canal Undifferentiated Carcinoma Angiosarcoma Apocrine Neoplasm Appendix Mucinous Adenocarcinoma Bartholin Gland Transitional Cell Carcinoma Basal Cell Carcinoma Bladder Adenocarcinoma Breast Metaplastic Carcinoma Cervical Adenocarcinoma Cervical Clear Cell Adenocarcinoma Cholangiocarcinoma Chordoma Colorectal Squamous Cell Carcinoma Desmoid Fibromatosis Endometrial Transitional Cell Carcinoma Endometrioid Adenocarcinoma Esophageal Neuroendocrine Carcinoma Esophageal Undifferentiated Carcinoma Extrahepatic Bile Duct Carcinoma Extramammary Paget Disease Fallopian Tube Adenocarcinoma Fallopian Tube Transitional Cell Carcinoma Fibromyxoid Tumor Gallbladder Carcinoma Gastric Neuroendocrine Carcinoma Gastric Squamous Cell Carcinoma Gastric Undifferentiated Carcinoma Gastrointestinal Stromal Tumor Gestational Trophoblastic Tumor Giant Cell Carcinoma Intestinal Neuroendocrine Carcinoma Intrahepatic Cholangiocarcinoma Lung Carcinoid Tumor Lung Sarcomatoid Carcinoma Major Salivary Gland Carcinoma Malignant Odontogenic Neoplasm Malignant Peripheral Nerve Sheath Tumor Malignant Solid Neoplasm Malignant Testicular Sex Cord-Stromal Tumor Metastatic Malignant Neoplasm of Unknown Primary Minimally Invasive Lung Adenocarcinoma Mixed Mesodermal (Mullerian) Tumor Mucinous Adenocarcinoma Mucinous Cystadenocarcinoma Nasal Cavity Adenocarcinoma Nasal Cavity Carcinoma Nasopharyngeal Carcinoma Nasopharyngeal Papillary Adenocarcinoma Nasopharyngeal Undifferentiated Carcinoma Oral Cavity Carcinoma Oropharyngeal Undifferentiated Carcinoma Ovarian Adenocarcinoma Ovarian Germ Cell Tumor Ovarian Mucinous Adenocarcinoma Ovarian Squamous Cell Carcinoma Ovarian Transitional Cell Carcinoma Pancreatic Acinar Cell Carcinoma Pancreatic Neuroendocrine Carcinoma Paraganglioma Paranasal Sinus Adenocarcinoma Paranasal Sinus Carcinoma Parathyroid Gland Carcinoma PEComa Peritoneal Mesothelioma Pituitary Gland Carcinoma Placental Choriocarcinoma Primary Peritoneal High Grade Serous Adenocarcinoma Pseudomyxoma Peritonei Rare Disorder Scrotal Squamous Cell Carcinoma Seminal Vesicle Adenocarcinoma Seminoma Serous Cystadenocarcinoma Small Intestinal Adenocarcinoma Small Intestinal Squamous Cell Carcinoma Spindle Cell Neoplasm Squamous Cell Carcinoma of the Penis Teratoma With Somatic-Type Malignancy Testicular Non-Seminomatous Germ Cell Tumor Thyroid Gland Carcinoma Tracheal Carcinoma Transitional Cell Carcinoma Ureter Adenocarcinoma Ureter Squamous Cell Carcinoma Urethral Adenocarcinoma Urethral Squamous Cell Carcinoma Vaginal Adenocarcinoma Vaginal Squamous Cell Carcinoma, Not Otherwise Specified Vulvar Carcinoma Patients are eligible under ONE of the following For all cohorts except the gestational trophoblastic disease (GTD) (Cohort #47), patients must have histologically and/or biochemically confirmed rare cancer and must be able to submit specimens; to be eligible for the GTD cohort: patients must have disease confirmed by quantitative serum beta-human chorionic gonadotropin (hCG) within 28 days prior to registration and must be able to submit blood specimens (tissue submission is not required for patients who will be registered to the GTD cohort [Cohort #47]); NOTE: Subsequent to site's Institutional Review Board (IRB) approval of revision 3, patients are NOT required to participate in EAY131 "National Cancer Institute (NCI)-Molecular Analysis for Therapy Choice (MATCH)" to register to S1609 OR FOR WITH PD-L1 (COHORT #50) ONLY: All solid tumors (excluding lymphoma) are allowed for the PD-L1 amplified cohort if they have PD-L1 amplification; patients may be considered for registration to the PD-L1 amplified cohort (Cohort #50) with the confirmation of at least one of the study chairs; PD-L1 amplification is defined as having deoxyribonucleic acid (DNA) copy number of equal to or greater than six by any of the following Clinical Laboratory Improvement Act (CLIA)-approved lab; (Immunohisochemistry [IHC] and fluorescence in situ hybridization [FISH] are not allowed); the assay must be done at or after the diagnosis of advanced disease, but PRIOR TO NOTE: patients with PD-L1 overexpression by IHC or PD-L1 amplification by FISH do not quality for this cohort; OR FOR IN EAY131 "NCI-MATCH" PRIOR TO EAY131 10 ONLY: Patients must have histologically confirmed rare cancer that did not have a match to a molecularly-guided therapy on EAY131 "NCI-MATCH" protocol or who are off protocol treatment on EAY131, "NCI-MATCH" and have no further molecularly-matched treatment recommendations per EAY131, "NCI-MATCH" or who are otherwise unable to receive EAY131, "NCI-MATCH" therapy Patients who do not qualify for one of the histologic cohorts and are not on the ineligible histology list may be considered for registration in the "Not Otherwise Categorized" Rare Tumors cohort with confirmation of at least one of the study chairs via email NOTE: The "Not Otherwise Categorized" Rare Tumors cohort was permanently closed to accrual on 3/15/2019 Patients who are determined to have a rare cancer with unknown primary site are eligible under cohort #32 (tumor of unknown primary [cancer of unknown primary; CuP]), provided that there is histologic documentation of metastatic malignancy with no discernible primary site identified from histopathologic review, physical exam and associated cross-sectional imaging of the chest, abdomen, and pelvis NOTE: The "Tumor of unknown primary (Cancer of Unknown Primary; CuP" cohort was permanently closed to accrual on 12/22/2017 Patients must also meet one of the following Patients must have progressed following at least one line of standard systemic therapy and there must not be other approved/standard therapy available that has been shown to prolong overall survival (i.e. in a randomized trial against another standard treatment or by comparison to historical controls); patients who cannot receive other standard therapy that has been shown to prolonged survival due to medical issues will be eligible, if other are met; OR  Patients who had prior grade 3 or higher immune-related adverse event (e.g. pneumonitis, hepatitis, colitis, endocrinopathy) with prior immunotherapy (e.g. cancer vaccine, cytokine, etc.) are not eligible Patients are not eligible if they have had or are planned for solid organ transplant Patients must not currently be receiving any other investigational agents or any other systemic anti-cancer therapy (including radiation, excluding RANKL inhibitors and bisphosphonates); in event patient recently received any other systemic anti-cancer therapy, patient must be off therapy at least 7 days prior to registration and any therapy-induced toxicity must have recovered to =< grade 1, except alopecia and =< grade 2 neuropathy which are allowed; any planned radiation therapy must be completed before registration to S1609 Patients must not have prior history of allergy or known hypersensitivity to nivolumab or ipilimumab Patients must not have known active hepatitis B virus (HBV) or hepatitis virus (HCV) infection at time of registration; patients with HBV or HCV that have an undetectable viral load and no residual hepatic impairment are eligible Patients must not have active autoimmune disease that has required systemic treatment in past 2 years (i.e., with use of disease modifying agents, immunosuppressive drugs, or corticosteroids with prednisone dose >= 10 mg); replacement therapy (e.g., thyroxine, insulin, or physiologic corticosteroid replacement therapy for adrenal or pituitary insufficiency, etc.) is not considered a form of systemic treatment; autoimmune diseases but are not limited to autoimmune hepatitis, inflammatory bowel disease (including ulcerative colitis and Crohn's disease), as well as symptomatic disease (e.g.: rheumatoid arthritis, systemic progressive sclerosis [scleroderma], systemic lupus erythematosus, autoimmune vasculitis [e.g., Wegener's granulomatosis]); central nervous system (CNS) or motor neuropathy considered of autoimmune origin (e.g., Guillain-Barre syndrome and Myasthenia gravis, multiple sclerosis or glomerulonephritis); vitiligo, alopecia, hypothyroidism on stable doses of thyroid replacement therapy, psoriasis not requiring systemic therapy within the past 2 years is permitted; short-term steroid premedication for contrast allergy is permitted Patients must not have any uncontrolled intercurrent illness including (not limited to): symptomatic congestive heart failure (CHF) (New York Heart Association [NYHA] III/IV), unstable angina pectoris or coronary angioplasty, or stenting within 24 weeks prior to registration, unstable cardiac arrhythmia (ongoing cardiac dysrhythmias of NCI Common Terminology for Adverse Events [CTCAE] version [v] 4 grade >= 2), known psychiatric illness that would limit study compliance, intra-cardiac defibrillators, known cardiac metastases, or abnormal cardiac valve morphology (>= grade 3) Note: Patients with history of CHF or patients who are deemed at risk because of underlying cardiovascular disease or exposure to cardiotoxic drugs should have an electrocardiogram (EKG) and echocardiogram (ECHO), as clinically indicated, at baseline and at the start of each cycle; patients who have evidence at baseline (or subsequently) of CHF, myocardial infarction (MI), cardiomyopathy, or myositis cardiac evaluation (NYHA I/II) should have additional consult by a cardiologist, including review of EKG, creatine phosphokinase (CPK), troponin, echocardiogram, as clinically indicated Patients must not have symptomatic interstitial lung disease or pneumonitis